BIOLOGY  LIBRARY 


LIBRARY 


UNIVERSITY  OF  CALIFORNIA. 


Class 


THE    PKINCIPLES  OF   PATHOLOGY 

VOLUME  I 

GENERAL  PATHOLOGY 

BY  J.  GEORGE  ADAMI,  M.A.,  M.D.,  LL.D.,  F.R.S. 


VOLUME  II 

SYSTEMIC  PATHOLOGY 

BY  J.  GEORGE  ADAMI,  M.A.,  M.D.,  LL.D.,  F.R.S. 

AND 

ALBERT  G.  NICHOLLS,  M.A.,  M.D.,  D.Sc.,  F.R.S.  (CAN.) 


THE 


PRINCIPLES  OF  PATHOLOGY 


BY 

J.  GEORGE  AD  A  MI,  M.A.,  M.D.,  LL.D.,  F.R.S. 

PROFESSOR  OF  PATHOLOGY  IN  MC  GILL  UNIVERSITY,   AND  PATHOLOGIST  TO  THE   ROYAL  VICTORIA  HOSPITAL, 

MONTREAL;   LATE   FELLOW  OF  JESUS  COLLEGE,   CAMBRIDGE,   ENGLAND 
AND 

ALBERT  G.  NICHOLLS,  M.A.,  M.D.,  D.Sc.,  F.R.S.  (CAN.) 

ASSISTANT     PROFESSOR    OF    PATHOLOGY    AND    LECTURER    IN    CLINICAL    MEDICINE     IN    MC  GILL    UNIVERSITY; 

OUT-PATIENT    PHYSICIAN    TO    THE    MONTREAL    GENERAL    HOSPITAL;    ASSISTANT 

PHYSICIAN   AND    PATHOLOGIST   TO   THE    WESTERN    HOSPITAL 


VOLUME  II 

SYSTEMIC  PATHOLOGY 


WITH     310     ENGRAVINGS     AND     15     PLATES 


•OF    THE 

UNIVERSITY 

OF 


LEA   &  FEBIGER 
PHILADELPHIA  AND  NEW  YORK 


A* 


SIOLOGT 

LIBRARY 


Entered  according  to  Act  of  Congress,  in  the  year  1909,   by 

LEA  &  FEBIGER, 
in  the  Office  of  the  Librarian  of  Congress.     All  rights  reserved. 


TO 


WILLIAM  H.  WELCH 


TO     WHOM     MEDICAL     RESEARCH     IN     NORTH     AMERICA     OWES     ITS     DEEPEST     DEBT 


THEIR   PREDECESSOR 


WILLIAM   OSLER 


WHO  INITIATED  THE  TEACHING  OF  PATHOLOGY  AT  McGILL  UNIVERSITY 


THIS  VOLUME 


IS  AFFECTIONATELY  DEDICATED  BY 


ITS  AUTHORS 


196513 


PREFACE. 


LITTLE  in  the  way  of  preface  is  here  needed  by  those  who  have  made 
themselves  familiar  with  the  first  volume  of  this  work.  In  that  we 
dealt  with  the  causes  of  disease  and  the  morbid  and  reactive  processes ; 
now  we  pass  forward  to  discuss  the  results  of  disease  as  it  affects  the 
different  systems  and,  through  them,  the  body  as  a  whole. 

We  will  not  pretend  that  our  first  volume  was  other  than  bulky;  to 
many  readers  it  may  have  seemed  that  the  treatment  of  the  various 
sections  was  unnecessarily  full.  These  same  readers  may  be  inclined 
to  consider  that,  working  upon  the  same  scale,  the  subject  matter  of 
special  pathology  demands  at  least  twice  the  space  now  afforded,  or, 
put  otherwise,  that  our  treatment  of  systemic  pathology  is  as  condensed 
as  that  of  general  pathology  was  diffuse.  A  little  consideration  will,  we 
trust,  show  that  our  method,  if  unusual,  is,  nevertheless,  rational.  Pro- 
vided that  the  student  has  acquired  a  good  grasp  of  the  principles  of 
general  pathology,  he  has  but  to  apply  those  principles  in  order  to  become 
possessed  of  a  sound  basis  of  special  pathology.  If,  to  cite  examples, 
he  be  well  acquainted  with  the  modifications  of  the  inflammatory 
process  as  it  affects  parenchymatous  and  connective  tissues,  epithelial 
and  serous  membranes,  respectively;  with  the  different  forms  of  tumors 
originating  from  the  different  orders  of  cells;  with  the  particular  orders 
of  degeneration  likely  to  affect  cells  of  one  type  or  another;  then  his 
familiarity  with  the  histology  of  the  different  organs  gives  him  the  key 
to  special  pathological  histology.  Taking  this  knowledge  for  granted, 
it  becomes  unnecessary  to  describe  in  detail  the  different  conditions  of 
inflammation,  tumor  growth,  and  degeneration  that  affect  the  different 
organs.  Where  these  conditions  are  typical  in  their  manifestations 
their  existence  alone  need  be  cited.  It  is  only  when  their  manifestations 
present  peculiarities  that  extended  description  is  demanded;  as  also  are 
the  details  of  the  gross  morbid  anatomy,  or  naked  eye  appearances,  of 
the  various  organs  under  various  conditions  of  disease. 

We  have  proceeded,  therefore,  upon  the  assumption  of  a  knowledge  of 
the  main  data  of  general  pathology  as  afforded  in  our  first  volume,  and 


viii  PREFACE 

this  second  volume  would  have  had  relatively  small  dimensions  had  we 
not,  in  the  first  place,  included  the  pathology  of  the  blood  and  organs  of 
circulation  (usually  and  erroneously  included  under  general  pathology), 
and,  in  the  second  place,  endeavored  to  make  our  treatment  more 
complete  by  dealing  with  the  disturbances  of  function,  as  well  as  those 
of  structure.  We  would  reiterate  that  pathological  anatomy  (and  his- 
tology) is  but  one  division  of  our  subject,  and  that  from  the  pathologist 
of  today  there  is  equally  demanded  an  acquaintance  with  the  effects  of 
disease  upon  the  function  of  organs.  Upon  this  we  would  lay  particular 
stress,  because  it  is  this  department  or  aspect  of  pathology  which,  for  the 
clinician  and  practitioner,  if  not  the  more  important,  is  assuredly  that 
capable  of  the  more  immediate  application.  It  is,  indeed,  interesting  to 
note  the  extent  to  which,  nowadays,  the  advanced  teaching  in  medicine 
is  based  upon  what  we  would  term  functional  pathology,  nay,  has  become 
teaching  in  this  and  little  more;  to  observe,  for  example,  the  prominence 
given  in  the  modern  Systems  of  Medicine  to  the  preliminary  chapters 
upon  the  "physiological  pathology"  of  the  different  organs.  As  a  con- 
necting link,  therefore,  between  theory  and  practice  this  functional 
pathology  is  of  prime  importance. 

Realizing  the  attention  that  this  branch  of  pathology  receives  from 
our  colleagues,  we  have  not  attempted  an  exhaustive  treatment; 
instead,  remembering  that  the  inclusion  is  more  or  less  of  a  novelty, 
we  have  taken  into  consideration  the  functional  pathology  of  the  more 
important  systems  only,  and,  doing  this,  have  but  dealt  with  certain 
aspects — with  those  which,  in  the  light  of  recent  research,  appeared  to 
call  for  special  remark. 

In  short,  neither  the  previous  volume  nor  this  is  to  be  regarded  as 
exhaustive.  We,  the  authors,  realize  most  acutely  the  deficiencies  and 
the  defects  of  both.  The  most  we  can  plead  is  that  we  have  attempted 
to  lay  down  broadly  the  main  outlines  of  general  and  systemic  pathology 
in  what,  we  hold,  is  a  logical  sequence. 

It  would  be  a  false  modesty  that  led  us  to  be  silent  regarding  the 
kindly  welcome  accorded  to  the  first  volume,  despite  its  imperfections, 
and  our  appreciation  of  the  same;  the  very  warmth  of  that  welcome  has 
proved  to  us  that  we  were  not  wholly  wrong  in  the  belief  that  the  time 
was  ripe  for  a  work  of  its  nature;  it  in  no  sense  lessens  our  admiration 
for  the  courage  displayed  by  Messrs.  Lea  &  Febiger  in  publishing  what 
was,  in  so  many  respects,  an  innovation.  The  present  volume  is  less  of 
a  departure.  We  would  express  the  hope  that  the  dual  authorship  has 
been  of  advantage  in  reducing  the  imperfections,  and  that,  by  so  much 


PREFACE  ix 

as  it  has  reduced  the  individuality  of  the  teaching,  it  has  increased  the 
value  of  the  work  as  a  text-book. 

Those  imperfections  of  the  previous  volume,  unavoidable  under  the 
conditions  surrounding  its  publication,  we  have  been  able  to  remedy 
to  a  large  degree  in  this.  With  infrequent  exceptions  the  illustrations 
are  our  own,  or  have  been  taken  from  the  collections  of  our  colleagues 
from  the  Medical  Museum  at  McGill,  the  Montreal  General,  and  the 
Royal  Victoria  Hospitals.  We  are  particularly  indebted  to  Dr.  Colin 
Russel,  Dr.  A.  E.  Vipond,  Dr.  F.  J.  Shepherd,  and  Dr.  Maude  E. 
Abbott.  When  not  from  these  sources,  we  have  made  the  selections 
from  the  collection  of  cuts  in  the  possession  of  our  publishers.  We 
would  most  cordially  express  our  appreciation  to  them  for  this  as  for  so 
many  other  services  in  connection  with  the  production  of  this  volume. 
To  Messrs.  Wood  &  Company  and  the  W.  B.  Saunders  Company 
we  are  indebted  for  the  permission  to  utilize  illustrations  which  have 
appeared  in  works  or  articles  by  one  or  other  of  us  published  by  them. 

J.  G.  A. 

A.  G.  N. 
MONTREAL,  1909 


CONTENTS. 


INTRODUCTORY    17 

SECTION  I. 
THE  CARDIOVASCULAR  SYSTEM. 

CHAPTER  I. 

THE    BLOOD QUANTITATIVE    ALTERATIONS ANEMIA— HYPEREMIA     ....  19 

CHAPTER  II. 

THE  BLOOD THE  EFFECTS  OF  CLOSURE  OF  VESSELS 33 

CHAPTER  III. 

THE  BLOOD HEMORRHAGE 76 

CHAPTER  IV. 

THE  BLOOD QUALITATIVE  CHANGES 83 

CHAPTER  V. 

THE  LYMPHATIC  SYSTEM (EDEMA 103 

CHAPTER  VI. 

THE  CARDIOVASCULAR  SYSTEM 115 

CHAPTER  VII. 

THE  HEART'    PATHOLOGICAL  ANATOMY  AND  HISTOLOGY 135 

CHAPTER  VIII. 

THE  VESSELS VASCULAR  FUNCTION  AND  ITS  DISTURBANCES ----^^  171 

CHAPTER  IX. 

PATHOLOGICAL    ANATOMY    AND    HISTOLOGY    OF    THE    ARTERIES,     VEINS,     AND 

LYMPHATIC  VESSELS 190 

CHAPTER  X. 

THE  BLOOD-FORMING  ORGANS   .  ...       208 


xii  CONTENTS 

SECTION    II. 
THE  RESPIRATORY  SYSTEM. 

CHAPTER  XI. 

THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 237 

CHAPTER  XII. 

THE  RESPIRATORY  PASSAGES 264 

CHAPTER  XIII. 

THE  LUNGS 283 

CHAPTER  XIV. 

THE  PLEURA 324 

CHAPTER  XV. 

THE  MEDIASTINUM    .  333 


SECTION    III. 

THE  ALIMENTARY  SYSTEM. 
CHAPTER  XVI. 

THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 339 

CHAPTER  XVIT. 

THE  MOUTH  AND  ITS  ACCESSORIES 381 

CHAPTER  XVIII. 

THE  OESOPHAGUS 397 

CHAPTER  XIX. 

THE  STOMACH 404 

CHAPTER  XX. 

THE  INTESTINES 422 

CHAPTER  XXI. 

THE  LIVER 455 

CHAPTER  XXII. 

THE  BILIARY  PASSAGES  489 


CONTENTS 
CHAPTER  XXIII. 


xin 


THE  PANCREAS 493 

CHAPTER  XXIV. 

THE  PERITONEUM 504 

SECTION  IV. 
THE  NERVOUS  SYSTEM. 

CHAPTER  XXV. 

THE  STRUCTURE  OF  THE  NERVOUS  SYSTEM  AND  ITS  BEARING  UPON  DISEASE  .       515 

CHAPTER  XXVI. 

THE  BRAIN 523 

CHAPTER  XXVII. 

THE  SPINAL  CORD 569 

CHAPTER  XXVIII. 

THE  PERIPHERAL  NERVES 600 

CHAPTER  XXIX. 

THE  EYE 607 

CHAPTER  XXX. 

THE  EAR 655 

SECTION  V. 

THE  DUCTLESS  GLANDS. 
CHAPTER  XXXI. 

THE  FUNCTIONS  OF  THE  DUCTLESS  GLANDS  AND  THEIR  DISTURBANCES        .        .        .       675 

CHAPTER  XXXII. 

THE  THYROID  AND  THYMUS  GLANDS 687 

CHAPTER  XXXIII. 

THE    SUPRARENAL     GLAND,     PITUITARY,     PINEAL,     AND      CAROTID     BODIES,     AND 

COCCYGEAL  GLAND  700 


xiv  CONTENTS 

SECTION  VI. 
THE  URINARY  SYSTEM. 

CHAPTER  XXXIV. 

THE  RENAL  FUNCTIONS  AND  THEIR  DISTURBANCES 715 

CHAPTER  XXXV 

THE  KIDNEYS  AND  URETERS 728 

CHAPTER  XXXVI. 

THE  BLADDER  AND  URETHRA  .  774 


SECTION  VII. 
THE  REPRODUCTIVE  SYSTEM. 

CHAPTER  XXXVII. 

THE  MALE  SEXUAL  ORGANS 787 

CHAPTER  XXXVIII. 

THE  FEMALE  SEXUAL  ORGANS 810 

CHAPTER  XXXIX. 

THE  PUERPERAL  UTERUS  AND  THE  PRODUCTS  OF  CONCEPTION S69 

CHAPTER  XL. 

THE  MAMMARY  GLAND    .  882 


SECTION  VIII. 
THE  TEGUMENTARY  SYSTEM 

CHAPTER  XLI 

THE  SKIN  AND  ASSOCIATED  STRUCTURES  .  907 


CONTENTS  xv 

SECTION  IX. 
THE  MUSCULAR  SYSTEM. 

CHAPTER  XLII. 

THE  SKELETAL  MUSCLES 979 


SECTION  X. 

THE  OSSEOUS  SYSTEM. 
CHAPTER  XLIII. 

THE  BONES,  JOINTS,  AND  CARTILAGES         .  .     1003 


UNIVERSITY 

UFOR1 


SYSTEMIC   PATHOLOGY. 


INTRODUCTORY. 

IN  the  first  volume  of  this  work,  after  having,  if  we  may  so  express  it, 
excavated  with  the  endeavor  to  lay  bare  so  far  as  possible  the  foun- 
dations of  Cellular  Pathology,  we  proceeded  to  discuss  what  is  commonly 
known  as  General  Pathology,  inquiring  first  into  the  causes  of  disease 
and  next  into  the  general  morbid  and  reactive  processes.  Doing  this, 
we  passed  from  a  study  of  the  cells  to  that  of  the  tissues  which  they 
form,  and  ended  by  considering  the  progressive  and  regressive  changes 
which  may  affect  those  tissues.  Thence  we  pass  naturally  to  a  study 
of  the  yet  larger  aggregates,  namely,  of  the  organs  and  different  systems 
of  the  body,  and  engage  in  the  study  of  what  we  would  term  Systemic 
Pathology.  Moreover,  just  as  in  the  first  volume  we  departed  from 
custom  and,  instead  of  making  our  treatment  mainly  histological,  based 
ourselves  to  a  considerable  extent  upon  the  physiology,  embryology, 
and  chemistry  of  the  cell  and  tissue,  so  here  we  have  endeavored  to 
present  to  the  reader  something  more  than  the  time-honored  Special 
Pathology,  by  which  has  come  to  be  understood  the  study  of  Morbid 
Anatomy  and  Histology,  and  of  that  alone. 

We  would  once  again  emphasize  that  our  subject  has  undergone 
material  development,  and  that  to-day  something  more  than  this  is 
demanded.  Inevitably,  the  study  of  Special  Pathology  forms  the  bulk 
of  our  work,  but  inevitably  also  the  study  of  the  anatomical  changes 
occurring  in  the  various  organs  has  led  to  inquiries  into  the  significance 
of  these  changes  and  the  influence  they  exert  upon  the  function  of  those 
organs,  and,  what  is  more,  upon  the  organism  as  a  whole.  Some 
knowledge  of  Functional  Pathology,  or,  as  some  would  express  it, 
Physiological  Pathology  (although  this  designation  involves  a  contra- 
diction in  terms),  is  now  requisite,  and,  indeed,  forms  a  most  important 
link  between  the  labors  of  the  pathologist  and  the  clinician.  To  indicate 
modern  requirements  we  need  but  mention  the  popularity  of  Professor 
Krehl's  well-known  work,  and  the  many  editions  it  has  undergone, 
despite  the  fact  that  its  teaching  covers  no  particular  university  course. 
We  have  thus  thought  it  wise  to  preface  our  treatment  of  the  individual 
systems  with  a  consideration  of  the  bearings  of  morbid  changes  in  the 
component  parts  of  that  system  upon  function.  The  vast  array  of  data 
that  have  accumulated  in  connection  with  the  Morbid  Anatomy  and 
Histology  of  the  different  organs  make  any  adequate  modern  text-book 
2 


18  INTRODUCTORY 

of  Special  Pathology  necessarily  a  large  volume.  Hence,  in  adding 
these  further  chapters  it  has  been  indispensable  to  deal  with  these 
matters  of  Functional  Pathology  from  a  broad  rather  than  a  detailed 
standpoint.  We  can  only  hope  that  these  additional  chapters  will 
render  the  work  of  additional  service. 

As   regards   the   order   of   procedure  there  are  two  systems  having 

ramifications  which  spread  throughout  the  whole  organism,  namely,  the 

vascular  and  the  nervous;  the  others,  if  perhaps  equally  essential,  are 

structurally  more  restricted  and  localized.     Considering  their  intimate 

relationship  with  every  region  of  the  body,  it  would  be  appropriate 

consider  these  two  universal  systems  first.      But  here  certain  prac- 

l  difficulties   present  themselves.      For  many  reasons   the  nervous 

3tem  should  be  given  pride  of  place;  its  functions  are  so  important, 
so  sharply  defined  from  those  of  all  the  other  systems,  that  it  might 
well  be  treated  first  and,  as  it  were,  dismissed  before  discussing  the 
other  systems  which  functionally  appear  to  be  more  intimately  con- 
nected. But  this  course  is  inadvisable,  and  that  because  the  study  of  its 
pathology,  if  the  most  interesting,  is  also  the  most  abstruse,  and  because 
that  study  is  so  specialized  that  it  throws  little  light  upon  and  does  not 
naturally  lead  up  to  the  other  branches  of  systemic  pathology.  It 
is,  therefore,  most  practical  and  most  wise  to  study  first  the  disturbances 
of  the  vascular  system  and  their  effect  upon  the  body  at  large,  the  blood 
and  lymph  and  the  vessels  which  bear  these;  from  this  almost  inevitably 
we  pass  on  to  consider  the  blood-forming  organs.  And  this  is  addition- 
ally advisable  when  it  is  recalled  that  hitherto  it  has  been  customary  to 
take  up  the  more  important  disturbances  affecting  the  distribution  of 
the  blood  and  its  constituents  (anemia,  plethora,  thrombosis,  embolism, 
hemorrhage,  oedema,  etc.)  as  part  of  the  course  in  General  Pathology. 
By  dealing  with  these  matters  first  we  make  a  compromise  between 
that  more  usual  and  this,  we  believe,  more  logical  method  of  procedure. 
To  repeat,  we  see  no  reason,  save  prescription  and  convenience,  why 
hemal  and  vascular  disturbances — and  not  nervous  and  respiratory  also 
— should  be  treated  as  portions  of  the  course  in  General  Pathology. 
Intimately  associated  with  one  of  the  main  functions  of  the  blood,  the 
respiratory  system  next  demands  attention;  and  following  this  the 
digestive  system,  through  which  the  blood  is  supplied  with  nutrient 
matter.  Following  this  natural  system,  it  becomes  inevitable  that,  great 
as  is  its  importance  in  the  economy,  the  nervous  system  can  only  be 
discussed  late  in  our  course. 

We  begin  then  with  a  study  of  the  pathology  of  the  cardiovascular 
system  and  first  consider  the  pathology  of  the  blood. 


™     0?  THE       w 

UNIVERSITY 

OF 


SECTION    I. 
THE  CARDIOVASCULAR  SYSTEM, 


CHAPTER    I. 

THE    BLOOD— QUANTITATIVE    ALTERATIONS— ANEMIA— HYPEREMIA . 

Ix  discussing  the  circulation,  we  have  to  consider  (1)  the  circulating 
medium,  the  blood,  and  with  it  the  lymph,  for  this,  as  regards  its  fluid 
constituent  at  least,  is  drawn  from  the  blood,  and  like  that,  circulates, 
even  if  slowly  and  imperfectly,  and  (2)  the  circulatory  apparatus,  the 
heart,  bloodvessels,  and  lymphatic  system.  It  is  difficult  often  to  separate 
these,  disturbances  in  the  distribution  of  the  blood,  for  example,  being 
now  dependent  upon  primary  disturbances  in  the  bloodvessels,  and  now 
the  reverse  being  the  case.  Remembering  this,  and  being  prepared  to 
find  that  a  certain  amount  of  overlapping  is  inevitable,  it  is,  on  the  whole, 
conducive  to  greater  clearness  if  first  the  circulating  medium  be  taken 
into  consideration,  and  later  the  circulatory  apparatus. 

That  the  blood  is  the  life  is  an  old  saying;  that  it  ministers  to  the  life 
of  the  constituent  cells  forming  man's  body,  and  is  essential  for  the  con- 
tinuance of  the  same,  more  nearly  states  the  facts  of  the  case  as  we  under- 
stand them  at  the  present  time.  If  this  be  so,  if  through  diffusion, 
osmosis,  and  active  or  selective  assimilation  or  excretion  the  cells  gain 
their  nourishment  from  it,  and  directly  or  indirectly  discharge  into  it 
the  products  of  their  activity,  then  obviously  the  well-being  of  the  organ- 
ism as  a  whole,  as  of  each  constituent  part,  is  liable  to  be  affected  by 
changes  of  two  orders,  namely,  changes  in  quantity  and  changes  in 
quality  of  the  blood,  either  present  in  the  body  as  a  whole,  or  supplied 
to  a  particular  region  or  organ.  Whence  it  follows  that  we  have  to  con- 
sider : 

I.  Quantitative  alterations  in : 

1.  The  amount  of  blood  as  a  whole. 

2.  The  amount  supplied  to  particular  regions. 

Closely  allied  to  these,  the  further  alterations  in  the  amount  and 
distribution  of  the  blood  caused  by: 

3.  Products  of  disorganization  (thrombi  and  thrombosis). 

4.  Presence  of  abnormal  constituents  and  their  effects   (einboli 

and  embolism). 

5.  Escape  of  blood  out  of  the  vessels — hemorrhage. 


20  THE  BLOOD 

II.  Qualitative  alterations  in : 

1.  The  fluid  menstruum  of  the  blood. 

2.  The  corpuscular  elements. 

We  have  written  above  as  though  the  blood  came  into  immediate 
contact  with  the  constituent  cells  of  the  body.  Save  in  the  case  of  the 
endothelial  lining  of  the  vessels,  the  cells  of  the  splenic  sinuses  and 
those  of  the  hemolymph  glands,  and  certain  cells  of  certain  tissues, 
e.  g.,  the  Kupfer  cells  of  the  liver  parenchyma,  this  is  not  the  case. 
It  is  not  the  blood  as  such  that  directly  affords  nourishment  to  the 
vast  majority  of  the  cells  of  the  organism,  nor  do  these  cells,  as  a  rule, 
discharge  their  products  directly  into  the  blood  stream.  It  is  the  lymph 
derived  from  the  blood  that  is  the  essential  medium  of  interchange  for 
most  of  the  cells  of  the  body.  Here  therefore  we  have  to  consider: 

III.  The  lymph,  both  as  regards  quantitative  and  qualitative  changes. 


QUANTITATIVE  ALTERATIONS. 

1.  In  the  Total  Quantity  of  Circulating  Blood. — We  are  apt  to 
accept  too  freely  that  the  total  amount  of  blood  in  the  body  is  about 
one-thirteenth  of  the  body  weight.  This  estimate  we  owe  to  BischorT1 
more  than  a  half  century  ago.  His  method  consisted  in  taking  two 
condemned  criminals,  weighing  them  before  decapitation,  collecting 
the  blood,  washing,  so  far  as  possible,  all  the  remaining  blood  out  of 
the  vessels,  washing  their  chopped-up  organs,  and  finally  deducting  the 
weight  of  the  washed  residuum  from  the  original  weight.  The  method 
was,  to  say  the  least,  somewhat  crude  and  lacking  in  accuracy.  As 
shown  by  Haldane  and  Lorrain  Smith,2  his  results  were  excessive. 
They  found  that  the  amount  varies  between  one-thirtieth  and  one- 
sixteenth  of  the  body  weight,  the  average  given  being  roughly  one- 
twentieth  (-2if.5-)>  or  4.78  grams  per  100  grams  of  body  weight.  The 
method  employed  by  those  observers  was  ingenious,  and  based  upon 
previous  observations  by  Welcker  and  Grehant  and  Quinquaud.3  The 
hemoglobin  of  the  corpuscles  takes  up  carbon  monoxide  (carbonic 
oxide)  with  very  much  greater  avidity  than  it  takes  up  oxygen  (accord- 
ing to  Nasmyth  and  Graham4  the  affinity  is  140  times  as  great),  and  by 
colorimetric  methods  the  proportion  taken  up  by  a  given  blood  can  be 
accurately  determined.  Thus,  if,  for  example,  an  individual  be  made  to 
inhale  during  a  short  period  a  known  amount  of  the  gas,  well  below  the 
amount  necessary  to  saturate  the  blood,  and  then  a  few  drops  of  the  blood 
be  removed,  and  the  percentage  of  CO  present  in  this  sample  be  deter- 
mined, then  it  is  a  simple  matter  to  determine  how  much  has  been  absorbed 
per  cubic  centimeter  and  what  ratio  this  bears  to  the  total  amount  of 

1  Zeitschr.  f.  wiss.  Zool.,  7  :  1855  :  331,  and  9  :  1857  :  65. 

2  Jour,  of  Physiol.,  25:  1900:  331. 

3  Jour,  de  1'Anat.  et  de  la  Physiol.,  1882:  564. 

4  Jour,  of  Physiol.,  35  f  1906:' 32. 


THE  TOTAL  QUANTITY  OF  CIRCULATING  BLOOD  21 

CO  absorbed,  and  from  this  to  estimate  the  amount  of  the  total  circulating 
blood.  The  figures  gained  by  these  two  observers  demonstrated  that 
in  fairly  healthy  individuals  there  may  be  close  upon  twice  as  much  blood 
in  one  as  in  another.  Making  accurate  observations  upon  series  of 
members  of  different  species  of  animals,  Bellinger1  arrived  at  similar 
conclusions.  He,  too,  found  marked  variations  in  the  amount  of  blood 
proportional  to  the  body  weight  where  there  was  no  indication  of  dis- 
ease. These  observations  are  fully  borne  out  by  the  current  observa- 
tions of  any  pathologist  who  performs  a  long  series  of  autopsies.  Often 
in  elderly  people,  as  again  in  those  who  had  suffered  from  progressive 
wasting  disease,  the  tissues  and  vessels  are  characteristically  exsuccous 
(to  employ  old  Sir  Thomas  Browne's  expression);  other  corpses,  on  the 
contrary,  ooze  abundant  blood  at  every  cut;  this  last  is  particularly  notice- 
able, it  has  seemed  to  us,  in  cases  of  obstructive  heart  disease. 

Thus,  it  must  be  kept  in  mind  that  departures  from  the  normal,  not 
only  in  the  specific  gravity  of  a  sample  of  blood  taken  from  the  finger  or 
lobe  of  the  ear,  but  also  in  the  number  of  erythrocytes  per  c.mm.,  do  not 
by  any  means  necessarily  mean  only  qualitative,  but  also  may  indicate 
quantitative  changes.  A  heightened  specific  gravity,  or  an  increase  in 
the  number  of  corpuscles,  may  mean  a  reduction  in  the  fluid  of  the 
blood — a  reduction  in  the  amount  circulating — and  not  an  increased 
production  of  corpuscles;  a  lowered  specific  gravity,  or  decrease  in  the 
number  of  corpuscles,  may  indicate  an  actual  or  relative  increase  in  the 
quantity  of  the  blood  plasma,  and  not  an  increased  destruction  of  the 
erythrocytes.  In  short,  the  mere  study  of  a  blood  film  and  enumeration 
of  corpuscles  is  incapable  of  instructing  us  either  as  to  variations  in 
amount  of  the  circulating  blood,  or  (as  is  too  often  held)  variations  in  the 
production  or  destruction  of  the  erythrocytes.  Hitherto,  we  have  been 
reasoning  on  totally  inadequate  data.  Thus,  as  Lorrain  Smith  has 
pointed  out,  not  a  few  conditions  which  hitherto  have  been  classed  among 
the  anemias  or  conditions  of  lack  of  blood  are  truly  states  of  hydremia, 
of  dilution  and  increase  in  actual  amount  of  blood.  We  admit  freely 
that  the  organism  possesses,  as  shown  by  Sherrington,  Lloyd  Jones,  and 
Cobbett,  a  singularly  delicate  mechanism  to  counteract  any  sudden 
change  in  the  amount  of  circulating  blood,  and  so  to  insure  the  due 
supply  to  all  the  tissues — a  mechanism  so  delicate  that  within  a  very  few 
minutes  after  a  withdrawal  of  blood  the  specific  gravity  of  that  remaining 
within  the  vessels  undergoes  a  decided  fall,  indicating  a  pouring  of 
fluid  from  the  lymph  spaces  and  tissue  cells  in  order  to  restore  the 
quantity  of  the  circulating  medium.  But  this  admission  does  not  oppose 
the  fact  that  different  individuals  need  very  different  amounts  of  blood. 
Your  elderly  maiden  lady  living  sparely,  and  exercising  her  muscles  with 
equal  economy,  has  very  different  circulatory  needs  from  those  of  the 
vigorous  athlete  in  the  prime  of  manhood,  in  whom  the  assimilative 
and  disintegrative  processes  proceed  apace.  And  if  this  be  so  in 
conditions  of  relative  health,  the  variations  in  quantity  of  blood  in  states 

1  Miinch.  med.  Woch.,  1886  :  Nos.  5  and  6. 


22  THE  BLOOD 

of  disease  must  be  even  more  marked.  We  are  bound,  therefore,  to 
recognize  the  existence  of  conditions  of  oligemia,  or  diminution  in  the 
amount  of  circulating  blood,  and  of  plethora,  or  increased  amount. 

Oligemia  (Ischemia,  Anemia). — Where,  as  may  happen  in  secondary 
anemias  and  in  pernicious  anemia,  during  life  the  individual  has  been  so 
bloodless  that  it  has  been  difficult  to  secure  a  drop  of  blood  from  the 
finger,  and  where  at  autopsy  the  amount  of  blood  in  the  heart  and 
vessels  is  noticeably  small  in  addition  to  being  thin  and  of  pale  color, 
there  can  be  no  doubt  that  a  condition  of  oligemia  has  been  present.1 
Similar  reduction  in  quantity  may  follow  extreme  or  repeated  hemor- 
rhage, or  great  loss  of  the  fluid  part  of  the  blood,  as  from  cholera  or 
pernicious  vomiting.  We  are  here  speaking  of  quantitative  changes, 
but  it  must  be  remembered  that  these  conditions  may  produce  profound 
qualitative  changes  also.  Thus,  within  a  very  few  minutes  after  a  con- 
siderable hemorrhage  there  is  a  great  drain  of  fluid  from  the  tissues  into 
the  bloodvessels,  whereby  the  quantity  of  blood  is  brought  toward  the 
normal.  If  the  hemorrhage  be  repeated,  the  tissues  may  no  longer  be 
able  to  afford  more  fluid;  what  blood  there  is  left  in  the  vessels  may  be 
both  thin  and  small  in  amount;  the  corpuscle  count  will  be  greatly 
lowered.  In  cholera,  on  the  other  hand,  there  is  no  loss  of  corpuscles; 
what  blood  there  is  left  is  thick  and  so  concentrated  as  to  be  almost 
tarry. 

Plethora. — For  long  years  the  teaching  of  Cohnheim2  has  influenced 
pathologists  to  disbelieve  in  the  existence  of  plethora.  Cohnheim  showed 
that  if  saline  solutions  were  injected  into  the  vessels,  they  underwent  a 
rapid  excretion  by  the  kidneys  and  removal  from  the  blood  into  the  lymph 
spaces  of  the  body;  the  failure  to  produce  plethora  by  this  method 
led  him  to  realize  the  remarkable  regulative  power  of  the  vessels  and 
tissues  whereby  the  organism  in  health  preserves  a  constant  blood 
ratio.  He  neglected  to  take  into  account  that,  as  with  all  other  mechan- 
isms of  the  organism,  this  also  might  be  thrown  out  of  order  in  disease. 
The  observations  of  Ix>rrain  Smith3  by  his  carbon  monoxide  method 
have  completely  overthrown  this  older  teaching.  It  would  seem,  as 
a  general  rule,  that  ample  nutrition,  coupled  with  active  development 
of  the  muscular  system,  is  associated  with  increase  in  the  amount  of 
blood  above  the  normal.  Take  two  individuals  of  the  same  age  and 
height,  the  one  a  city  clerk,  the  other  a  university  athlete,  and  the  large 
heart  and  full  pulse  of  the  latter  can  only  mean  a  large  amount  of  circu- 
lating blood.  The  rate  of  heart  beat  may  not  be  faster  in  the  muscular 
man,  indeed  it  may  be  slower,  but  one  has  only  to  examine  the  heart 
of  such  a  case  to  determine  the  large  size  of  the  cavities,  which,  with  the 

1  The  term  anemia  has  unfortunately  come  to  mean  not  what  it  should  signify, 
"want  of  blood,"  but  diminution  in  the  hemoglobin  content,  or  in  the  number  of  red 
corpuscles.     It  tends  to  greater  precision  to  refer  to  this  condition  as  oligemia,  or 
small  quantity  of  blood  (6Ap/of,  few). 

2  Lectures  on  General  Pathology,  cap.  7. 

3  For  an  epitome  of  Lorrain  Smith's  work  see  his  Appendix  to  Graham  Steele's 
Diseases  of  the  Heart,  Manchester,  University  Press,  1906:  361 


PLETHORA  23 

associated  greater  size  of  the  aorta,  can  only  indicate  a  larger  volume  of 
blood  to  be  propelled.  There  is  such  a  condition  as  simple  plethora, 
i.  e.,  actual  increase  in  the  amount  of  normal  blood. 

But  as  with  oligemia,  so  here:  conditions  of  abnormal  plethora  are 
more  recognizable,  conditions  in  which  there  is  increase  in  the  fluid  of 
the  blood  in  excess  of  actual  increase  in  the  actual  number  of  corpuscles. 
There  can  be  no  question  regarding  the  existence  of  watery  or  hydremic 
plethora.  Such  occurs,  as  already  noted,  in  many  cases  of  obstructive  heart 
and  liver  disease.  There,  from  the  increased  venosity  of  the  blood,  it  may 
be  mistaken  for  true  pleth6ra,  although  study  of  the  blood  serum  in  these 
cases  has,  in  general,  shown  that  it  is  of  lessened  specific  gravity,  i.  e., 
that  the  serum  is  diluted,  and,  as  Grawitz  has  pointed  out,  a  coincident 
early  sign  of  failure  of  compensation  is  reduction  in  the  number  of 
erythrocytes  per  c.mm.  Lorrain  Smith's  observations1  show  that  in 
these  cases  the  volume  of  blood  becomes  increased  two  and  three  times 
above  the  normal;  they  show  that  at  first,  to  antagonize  its  dilution  and 
the  slowing  of  the  pulmonary  circulation,  the  reduction  in  the  number 
of  red  corpuscles  is  not  proportional  to  the  hydremia,  and  further, 
that  the  color  index  of  the  corpuscles  is  increased  with  indications  of 
a  compensatory  hematopoiesis.  Many  more  studies  are  needed  before 
we  are  fully  conversant  with  the  changes  in  the  blood  accompanying 
heart  disease. 

What  is  the  cause  of  this  form  of  cardiac  plethora  it  is  difficult  to  say, 
whether  it  is  due  to  the  associated  impaired  circulation  through  the 
kidneys,  and  disturbance  of  their  function,  or  to  heightened  venous  and 
capillary  pressure,  dilatation  and  widening  of  the  stream  bed  necessi- 
tating a  larger  amount  of  fluid  in  order  to  keep  the  blood  in  movement. 
A  similar  hydremic  plethora  has  been  noted  in  connection  with  obstruc- 
tive lung  disease.  Closely  allied  is  the  plethora  accompanying  the 
Munich  beer  heart,  ably  studied  by  Bollinger.  The  hypertrophy  and 
dilatation  of  the  heart  that  follows  the  conscientious  daily  consumption 
of  many  liters  of  light  beer  would  indicate  that  with  absorption  of  this 
beer  there  is  a  daily  considerable  increase  in  the  amount  of  the  circulat- 
ing blood. 

Yet  another  form  of  hydremic  plethora  is  associated  with  parenchy- 
matous  nephritis.  It  used  to  be  thought  that  the  accompanying  albumin- 
uria  and  drain  of  serum  albumin  from  the  blood  was  the  cause  of  its  more 
watery  condition.  That,  however,  would  not  account  for  the  observed 
increase  in  amount  in  many  of  the  cases,  and  for  the  absence  of  the 
plethora  in  other  types  of  nephritis.  French  observers  have  of  late  indi- 
cated a  more  probable  cause,  both  of  the  hydremia  and  of  the  plethora. 
They  have  called  attention  to  the  deficient  discharge  of  sodium  chloride 
from  the  kidneys,  and  it  is  now  gaining  increasing  acceptance  that  the 
accumulation  of  the  chlorides  in  the  blood  and  tissues  attracts  an  in- 
creased amount  of  water  leading  to  both  hydremia  and  oedema.  Re- 

1  Trans.  Path.  Soc.  London,  53  : 1900: 136. 


24  THE  BLOOD 

striction  of  chlorides  has  been  found  to  be  followed  by  material  improve- 
ment.1 

2.  Local  Alterations  in  the  Quantity  of  Blood  Supplied  to  a 
Part. — It  is  one  of  the  commonplaces  of  physiological  knowledge 
that  the  blood  supply  of  a  part  varies  according  to  circumstances;  that 
increased  activity  of  an  organ  is  accompanied  by  increased  passage  of 
blood  to  the  same ;  that  such  increase  is  largely  controlled  by  the  central 
nervous  system  through  the  vasodilator  and  vasoconstrictor  nerves, 
although  at  the  same  time  conditions  within  an  organ,  and  again,  the 
composition  of  the  blood — the  presence,  for  example,  of  acids  or  alkalies — 
directly  affect  the  walls  of  the  smaller  arteries  and  capillaries,  causing 
dilatation  or  contraction  of  the  same,  and,  in  consequence,  variation  in 
the  blood  supply  to  the  organ. 

Where  there  is  excess  of  blood  in  a  part  we  speak  of  (local)  hyperemia, 
where  deficiency,  of  (local)  anemia. 

In  the  above  rapid  statement  we  have  by  no  means  exhausted  the  list 
of  conditions  leading  to  a  physiological  alteration  of  the  blood  supply 
of  a  part.  The  tone,  or  state  of  partial  contraction  of  the  vessels,  has 
to  be  kept  in  mind;  the  fact  that  the  amount  of  blood  is  much  below 
the  capacity  of  the  vessels  that  carry  it;  that  hyperemia  and  dilatation 
of  the  vessels  of  one  region  demand  a  compensatory  relative  anemia  of 
the  rest  of  the  body,  or  of  some  region  of  the  same ;  that  there  is  evidence 
of  a  special  nervous  control  whereby,  under  normal  conditions,  the  supply 
of  blood  to  that  region  which  in  the  erect  position  should  be  the  first 
region  to  suffer  from  diversion  of  blood  to  other  parts,  namely,  the 
cranium  and  brain,  becomes  the  last  to  be  seriously  affected.  A  knowl- 
edge of  the  data  upon  which  these  conclusions  are  founded  must  be  taken 
for  granted. 

Local  Hyperemia.— Three  conditions,  it  will  be  seen,  determine  the 
presence  of  an  excessive  amount  of  blood  in  an  organ  or  part,  namely: 
(1)  the  passage  of  an  increased  amount  of  blood  into  it,  the  outflow 
remaining  the  same;  (2)  diminished  outflow,  the  inflow  being  unaltered; 
(3)  no  change  in  the  caliber  of  the  entering  vessels  or  in  the  arterial 
blood  pressure,  and  no  resistance  or  obstruction  to  the  outflow  through 
the  veins,  but  a  widening  of  the  capillary  channels  in  the  organ.  The 
first  of  these  we  term  active  or  arterial  hyperemia,  the  second  passive 
hyperemia  or  venous  congestion;  the  existence  of  the  third  is  largely 
neglected;  we  may  refer  to  it  as  capillary  hyperemia,  and  discuss  it  first. 

Capillary  Hyperemia. — The  clearest  'physiological  and,  we  are  inclined 
to  think,  pathological  examples  of  this  condition  are  afforded  by  an 
organ  which,  it  is  true,  does  not  so  much  possess  capillaries  as  blood 
sinuses.  As  shown  by  Roy  in  his  well-known  oncometric  observations 
upon  this  organ,  the  normal  spleen  exhibits  a  slow  pulsation  of  its 
own,  gradually  expanding  independently  of  any  change  in  the  general 

1  Vaguez  et  Digne,  Etudes  sur  la  Retention  et  1' Elimination  des  Chlorures,  Paris, 
1905;  Ambard,  Les  Retentions  Chlorures,  Paris,  1905.  This  matter,  however,  must 
be  regarded  as  still  sub  judice. 


ACTIVE  HYPEREMIA  25 

blood  pressure,  and  then  gradually  contracting.  This  pulsation  is 
attributed  to  the  expansion  and  contraction  of  the  plain  muscle  fibers 
contained  in  the  capsule.  With  each  act  of  contraction  the  capillaries 
and  blood  spaces  of  the  organ  are  compressed  and  blood  is  driven  out 
of  them;  with  each  expansion  these  become  filled  again.  To  some 
extent  this  must  be  true  of  all  viscera  possessing  muscular  walls; 
expansion  of  that  muscle,  or  loss  of  tone  of  the  same,  must  passively 
permit  a  hyperemia  of  the  capillary  areas  within  the  muscle  layer.  It 
may  well  be  that  the  enlarged  hyperemic  condition  of  the  spleen  in  typhoid 
and  other  infections  is  not  so  much  an  example  of  arterial  or  active 
hyperemia  as  of  the  capillary  hyperemia  due  to  paresis  and  giving 
way  of  the  muscular  elements  of  the  capsule,  although  with  this  another 
probable  factor  is  the  actual  obstruction  to  onflow  of  the  blood  encoun- 
tered in  the  sinuses  themselves,  through  the  arrest  and  accumulation  there 
of  the  red  corpuscles.  But  even  in  this  latter  case  it  will  be  seen  that  the 
hyperemia  is  of  neither  arterial  nor  venous  origin.  It  will  be  recalled 
that  a  similar  capillary  obstruction  has  been  noted,  answerable  for  the 
obstruction  to  the  passage  forward  of  the  blood  and  for  the  hyperemia 
seen  in  acute  inflammation  (vol.  i,  p.  385),  which  thus,  save  in  its  first 
stages,  can  scarce  be  regarded  as  truly  of  the  active  type.  What  may 
be  regarded  as  almost  physiological  examples  of  this  order  are  seen  in 
the  effects  of  cupping  and  hyperemia  induced  by  suction. 

Active  Hyperemia. — This  may  be  either  direct,  due  to  changes 
telling  directly  upon  the  arteries  passing  to  a  part,  and  leading  to  their 
dilatation,  whereby  a  wider  bed  is  afforded,  and  more  blood  enters  the 
part,  or  collateral,  due  to  contraction  of  other  arteries,  whereby,  other 
things  being  equal,  the  blood  pressure  is  raised  and  more  blood  is  poured 
into  the  arteries  under  consideration,  and  so  into  the  region  supplied  by 
them.  Little  need  be  said  regarding  the  latter  condition;  we  rarely 
encounter  well-marked  pathological  examples  save  in  the  development 
of  collateral  circulation  in  a  limb  or  other  region.  The  direct  form 
is  relatively  frequent,  and  may  be  due  either  to  (1)  stimulation  of  the 
vasodilators,  (2)  paralysis,  or  inhibition  of  the  vasoconstrictors,  or  (3) 
direct  local  action  of  physical  or  chemical  agents  (warmth,  temporary 
ligature,  atropine,  croton  oil,  etc.)  upon  the  walls  of  the  arterioles,  leading 
to  a  giving  way  or  expansion  of  the  musculature.  Diminution  of  ex- 
ternal pressure  acting  upon  an  artery  has  a  similar  effect.1 

The  appearances  of  the  affected  part  in  true,  active  hyperemia  are 
characteristic;  it  is  enlarged  and  swollen,  the  color  is  of  a  bright,  arterial 
red;  superficial  parts,  from  the  more  abundant  and  freely  circulating 
blood,  are  warmer  than  the  surrounding  regions,  and  in  not  a  few  cases 
it  has  been  noted  that  the  blood  passing  from  the  veins  of  the  part  is 
arterial  in  character.  In  extreme  cases  there  may  be  a  sensation  of 

1  It  may  be  urged  that  the  example  afforded  by  us  to  illustrate  capillary  hyperemia 
may  be  explained  in  this  way,  and  that  so  this  condition  may  be  placed  among 
the  active  hyperemias.  There  seems,  however,  to  be  a  distinct  difference  between 
widening  the  capillary  bed  by  reduction  of  pressure  or  active  expansion  of  the  organ 
itself,  and  removal  of  pressure  from  the  afferent  vessel. 


26  THE  BLOOD 

throbbing  in  the  affected  part,  together  with  visible  pulsation  of  the 
smaller  arteries,  or  even  of  the  capillaries,  as,  for  example,  at  the  base  of 
the  nail.  The  reddening  may  be  distinguished  from  that  due  to  hemor- 
rhage by  (in  general)  its  brighter  color,  and  more  particularly  by  its 
temporary  disappearance  when  pressure  is  applied. 

Examples  of  these  different  forms  of  active  hyperemia  may  here  be 
rapidly  passed  in  review. 

N europamlytic  hyperemia  due  to  removal  of  vasoconstrictor  influ- 
ences. The  type  example  of  this  form  was  afforded  by  Cl.  Bernard  in 
his  well-known  experiment  of  dividing  the  cervical  sympathetic  nerve 
in  the  rabbit.  In  man  it  has  been  noted  that  similar  section  of  the 
cervical  sympathetic,  or  destruction  of  the  same  by  tumors,  etc.,  leads  to 
hyperemia  of  the  side  of  the  face,  and  dilatation  of  the  retinal  vessels, 
with  heightened  temperature.  A  like  unilateral  reddening  seen  in 
some  forms  of  migraine  or  hemicrania  has  been  attributed  to  inhibitory 
disturbance  of  the  same  nerve.  It  has,  however,  to  be  noted  that  not  all 
the  paralytic  hyperemias  observed  in  the  lower  animals  as  the  result  of 
nerve  section  are  to  be  encountered  in  man. 

Neurotonic  hyperemia,  due  to  stimulation  of  the  vasodilators.  The 
type  example  by  which  this  is  demonstrated  is  the  intense  hyperemia  of 
the  submaxillary  gland  which  ensues  upon  stimulation  of  the  chorda 
tympani,  a  hyperemia  so  intense  that  the  blood  in  the  emergent  veins 
is  of  arterial  character.  To  this  order  probably  belong  many  of  the 
fugitive  erythemas  of  particular  areas  seen  in  neuralgic  and  hysterical 
conditions,  as  again  in  food  and  drug  idiosyncrasies  (vol.  i,  p.  372). 
Here  also  probably  belongs  that  striking  condition,  herpes  zoster,  in 
which  the  cutaneous  distribution  of  one  or  more  nerves  is  sharply  picked 
out  to  be  the  site  of  acute  hyperemia  followed  by  exudation  and  vesicle 
formation.  We  have  already  discussed  the  relationship  of  events  of 
this  order  to  inflammation  (vol.  i,  p.  410).  Of  the  same  order  would 
seem  to  be  erythromelalgia,  a  condition  in  which,  suddenly,  restricted 
areas,  often  symmetrically  situated  on  the  feet  or  hands,  present  a  burning 
pain,  with  pronounced  redness,  heat,  and  pulsation.  Closely  allied 
are  the  reflex  hyperemias,  of  which  blushing  affords  the  familiar  example. 
Characteristic  instances  of  this  order  are  seen  in  certain  cases  of  referred 
hyperemia  or  inflammation,  e.  g.,  in  the  reddening  and  swelling  of  the 
side  of  the  face  which  may  accompany  acute  inflammation  of  a  tooth. 
The  rash  upon  the  cheeks  of  infants  when  teething  (roseola  infantilis)  is 
of  the  same  order. 

Myoparalytic  hyperemia  (Lubarsch)  due  to  influences  acting  directly 
on  the  arterial  wall.  Of  influences  which  directly  induce  arterial  expan- 
sion may  be  mentioned  warmth,  temporary  ligature  or  compression 
(the  active  congestion  which  follows  the  use  of  Esmarch's  bandage  is 
a  well-marked  example),  the  presence  of  acids  in  the  circulating  blood, 
which,  as  Gaskell  has  shown,  causes  dilatation  of  the  cerebral  vessels 
(whereas  alkalies  bring  about  contraction),  atropine,  etc.  In  the  experi- 
mental production  of  inflammation  it  has  been  demonstrated  that  croton 
oil  applied  to  the  rabbit's  ear  acts  so  slowly  that  the  arterial  dilatation 


PASSIVE  CONGESTION,  OR  VENOUS  HYPEREMIA  27 

can  only  be  due  to  direct  action  and  not  reflex.  In  bacterial  inflamma- 
tion the  toxins  must  act  similarly. 

Effects  of  Local  Hyperemia. — Where  this  is  fugitive  no  effects  may  be 
detected;  where  prolonged,  the  increased  blood  flow  is  inevitably  accom- 
panied by  increased  transudation  from  the  distended  capillaries,  with, 
as  a  result,  some  swelling  (oedema)  of  the  part  and  increased  flow  of 
lymph  from  the  same.  Such  increased  flow  signifies  also  increased  nutri- 
tion. We  have  already  discussed  whether  increased  nutrition  in  itself 
leads  to  increased  growth,  concluding  that,  unless  there  be  some  coin- 
cident strain  or  demand  for  increased  work,  cells  subjected  to  increased 
nourishment  do  not  of  necessity  take  on  growth  (vol.  i,  p.  545).  We 
did  not  then  take  into  account  the  undoubted  influence  of  increased 
warmth  in  stimulating  activities  and  growth.  Such  increased  warmth 
is  present  in  active  hyperemia  of  a  superficial  area,  and  we  would 
suggest  that  this  may  help  to  explain  an  apparent  exception  to  the  rule 
that  has  been  noted,  namely,  that  after  section  of  the  cervical  sympathetic 
in  the  rabbit,  the  ear  and  the  hair  on  the  affected  side  grow  more  than 
do  those  on  the  sound  side. 

Extreme  arterial  hyperemia  in  an  organ  provided  with  a  limiting 
capsule  may,  by  compression  of  the  specific  cells,  cause  definite  disturb- 
ance of  function,  as  also  in  loosely  constructed  organs,  by  overfilling, 
it  may  lead  to  capillary  hemorrhages. 

Passive  Congestion,  or  Venous  Hyperemia. — As  already  laid  down, 
passive  congestion  is  brought  about  by  obstruction  or  closure  of  a 
vein,  so  that  the  blood,  propelled  forward  through  the  arteries,  accu- 
mulates behind  the  point  of  arrest.  There  is  this  striking  difference 
between  active  and  passive  hyperemia,  that  the  former  can  only  affect 
a  relatively  restricted  area,  the  latter  may  be  widespread.  A  little  con- 
sideration shows  why  this  is  the  case.  Normally  there  is  considerable 
tone  or  partial  contraction  of  the  arteries.  Removal  of  this  tone  over 
a  wide  area  renders  the  bed  too  big  for  the  available  blood  delivered 
from  the  heart,  and  within  a  very  short  time  there  is  insufficient 
fluid  entering  the  arteries  to  keep  them  distended  and  hyperemic. 
To  preserve  an  overfilling  of  the  arteries  of  one  region,  there  must  be  an 
extensive  contraction  of  the  arteries  of  other  regions.  The  very  con- 
vergence of  the  veins,  so  that  all  from  relatively  large  districts  come 
together  and  pour  their  blood  into  a  common  trunk,  necessitates  that  any 
obstruction  in  this  common  trunk,  or  even  in  the  heart  itself,  results  in 
a  heaping  up  of  blood  behind  the  point  of  obstruction;  the  blood  becomes 
dammed  in  the  main  trunk  or  trunks  and  in  the  organs  from  which 
these  trunks  pass.  In  this  way  obstructive  disease  of  the  left  heart 
leads  to  passive  congestion  of  the  whole  pulmonary  area;  obstructive 
disease  of  the  lungs  or  of  the  right  heart  causes  cyanosis  and  congestion 
of  the  face,  neck,  liver,  and  other  abdominal  organs;  obstructive  disease 
of  the  liver  or  portal  vein  causes  passive  congestion  of  the  intestines, 
spleen,  etc.  Paradoxically  it  may,  and  often  does,  happen  that  even 
complete  blockage  of  a  small  vein  leads  to  little  or  no  passive  congestion, 
and  this  because  in  most  of  the  organs  of  the  body  the  smaller  veins 


28 

present  abundant  anastomoses,  as  a  result  of  which,  if  one  vein  becomes 
overloaded,  the  blood  which  should  pass  along  it  finds  easy  outlet  along 
collateral  channels.  It  is  where  these  anastomoses  do  not  exist,  or  are 
inadequate — in  what  is  known  as  absolute  or  relative  terminal  veins — that 
we  encounter  the  most  extreme  results  of  localized  passive  congestion. 

The  causes  of  arrested  onflow  of  the  blood  in  the  veins  are  manifold. 
In  the  more  widespread  cases  of  passive  congestion  it  is  not  essential 
that  there  should  be  actual  narrowing  of  the  vascular  channel;  mere 
weakening  of  the  heart  muscle,  as  from  fatty  degeneration  without 
valvular  disease,  may  result  in  lack  of  propulsive  power,  whereby  the 
blood  becomes  heaped  up  in  the  venous  system.  Again,  it  has  to  be 
remembered  that  the  normal  advance  of  the  blood  along  the  veins  is  not 
dependent  only  on  the  vis  a  tergo  of  the  ventricular  pump  acting  through 
the  arteries  and  capillaries.  Every  muscular  contraction  presses  on 
certain  veins,  and  through  the  agency  of  the  frequent  valves  present  in 
them  drives  the  blood  forward;  every  beat  of  the  arteries  must  have 
a  like  influence  on  its  venae  committentes.1  The  adjuvant  action  of 
the  negative  pressure  on  the  thorax  during  inspiration  and  the  negative 
pressure  in  the  ventricles  during  diastole  (see  later2)  need  no  comment. 
We  mention  these  things  in  order  to  emphasize  the  fact  that  (1)  cardiac 
weakness,  (2)  hindrances  to  perfect  inspiration  (paralysis  of  the  dia- 
phragm, or  obstruction  to  the  proper  action  of  the  same,  accumulation 
of  fluid,  or  new-growth  in  the  pleural  cavity,  etc.),  (3)  lowered  blood 
pressure  and  weakened  pulsation,  and  (4)  lack  of  muscular  activity, 
all  play  a  part  in  lessening  the  onflow  of  the  venous  blood,  and  all  to 
a  greater  or  less  degree  favor  venous  congestion. 

Such  congestion,  it  may  be  added,  tends  to  show  itself  more  especially 
in  those  regions  in  which  the  veins  receive  least  support  or  compression 
from  the  surrounding  tissues,  and  when  at  the  same  time  these  adjuvant 
factors  are  least  brought  into  play.  Thus,  other  things  being  equal, 
it  is  in  those  taking  little  exercise  that  piles  or  hemorrhoids  are  more 
particularly  apt  to  show  themselves;  in  those  standing  much  on  their 
feet,  rather  than  in  those  indulging  in  much  walking  exercise,  that  vari- 
cose veins  of  the  leg  are  to  be  encountered.  Naturally,  however,  it  is 
where  there  is  narrowing,  blocking,  or  obliteration  of  the  venous  channels 
that  the  passive  hyperemia  below  the  point  of  obstruction  is  most  marked. 
To  cite  all  the  means  whereby  these  conditions  are  brought  about  in  the 
heart  and  veins  would  demand  a  very  lengthy  list;  we  must  content 
ourselves  with  briefly  classifying  the  main  order  of  events  into  (1) 
conditions  acting  from  within  the  blood  channel  (development  of  parietal 
thrombi,  Chapter  II)  of  intravascular  new-growths,  retrograde  emboli 
(Chapter  II);  (2)  conditions  affecting  the  vessel  walls,  leading  to  stenosis 
or  narrowing  (endocarditis  of  heart  valves,  syphilitic,  and  other  forms 
of  phlebitis,  new-growths  involving  the  walls,  etc.);  and  (3)  conditions 
acting  from  without,  compressing  the  vessel  (tumors,  fluid  accumulations, 

1  Sir  Lauder  Brunton,  Therapeutics  of  the  Circulation,  1908:  5. 

2  Chapters  VI  and  XI. 


PASSIVE  CONGESTION,  OR  VENOUS  HYPEREMIA  29 

inflammatory  cicatrices,  granulomas,  pressure  of  enlarged  organs,  e.  g., 
of  the  pregnant  uterus,  etc.).  These  conditions,  to  repeat,  may  affect  the 
heart  or  larger  veins,  or,  again,  individual  veins  within  an  organ  or  tissue. 

Results. — An  organ  or  part  which  is  the  seat  of  passive  hyperemia 
(1)  is  enlarged,  primarily  in  consequence  of  the  increased  amount  of 
contained  blood,  secondarily  as  a  result  of  increased  transudation 
from  the  distended  capillaries;  (2)  is  of  a  dark,  purplish  color,  owing  to 
the  distension  of  its  vessels  with  blood,  which,  owing  to  long  continuance 
in  the  vessels,  has  become  intensely  venous;  (3)  is  (when  superficial) 
cooler  than  the  surrounding  parts,  owing  to  the  slowed  circulation. 

A  few  words  are  necessary  regarding  the  significance  of  these  different 
changes  upon  the  blood,  the  vessels,  and  the  tissues. 

The  Blood. — Relative  or  active  arrest  of  the  blood  within  the  capillaries 
leads  to  greater  giving  up  of  oxygen  and  increased  diffusion  into  it  of 
carbon  dioxide.  It  thus  becomes  intensely  venous.  Thus,  Lepine  found 
as  much  as  64  per  cent,  of  CO2  in  the  venous  blood  of  a  case  of  obstructive 
heart  disease.  It  is  this  that  explains  the  cyanosis  (or  "blue"  state) 
of  sufferers  from  passive  congestion.  One  exception  has  to  be  noted: 
the  brain  and  spinal  cord,  when  congested,  do  not  exhibit  cyanosis; 
at  most,  multiple  blood  points  (distended  capillaries)  stand  out  against 
the  pale  background  of  the  cut  surface  of  the  white  matter.  Complete 
stasis  or  arrest  may  be  followed  by  coagulation  and  thrombosis. 

The  Vessel  Walls. — Continued  passive  congestion  is  constantly  followed 
by  indications  of  injury  to  the  venous  and  capillary  endothelium;  in  the 
first  place,  it  is  abnormally  stretched  and  thinned;  in  the  second,  it 
exhibits  fatty  degeneration,  presumably  as  a  result  of  carbon  dioxide 
intoxication. 

The  Tissues. — As  a  result  there  is  increased  transudation,  and,  it 
may  be,  modified  (exudation),  with  some  heaping  of  fluid  in  the  inter- 
stitial tissue  (cedema).  This  may  not  be  marked  in  individual  organs 
provided  with  a  limiting  capsule,  but  where  there  is  general  venous 
congestion  of  large  areas,  as  from  heart  disease,  it  is  one  of  the  most 
striking  features,  with  great  accumulation  of  fluid  in  the  body  cavities 
(ascites,  hydro  thorax)  and  in  the  subcutaneous  tissues  (anasarca). 
The  most  rapid  accumulation  of  fluid  occurs  in  cases  of  sudden  blockage 
or  obliteration  of  the  portal  vein;  here,  apparently,  in  consequence  of 
the  portal  blood  with  its  material  absorbed  from  the  intestines  being 
more  toxic  than  systemic  blood  in  general.  Where  the  congestion  is 
extreme  and  the  capillaries  ill-supported,  there  may  be  in  addition 
repeated  and  multiple  small  capillary  hemorrhages  (Chapter  III),  and  as 
a  result  of  the  breaking  down  of  the  hemoglobin  the  tissue  may  assume 
a  brownish  color.  In  addition,  through  malnutrition  and  through 
pressure,  the  specific  cells  of  a  tissue  are  apt  to  exhibit  degeneration  and 
atrophy.  This  is  particularly  well  seen  in  the  liver,  where,  through 
passive  congestion,  the  cells  of  the  centre  of  the  lobule  first  undergo 
diminution  in  size  with  pigmentation,  and  eventually  become  completely 
atrophied  and  disappear,  their  place  being  taken  by  greatly  dilated 
capillaries  (Chapter  XXI). 


30  THE  BLOOD 

There  is  still  some  debate  as  to  whether  passive  congestion  of  any 
order  can  lead  to  increased  tissue  growth.  This  we  have  already  dis- 
cussed (vol.  i,  p.  414).  We  would  repeat  that  just  as  the  Polish  Jew 
or  Chinaman  can  live  and  even  thrive  under  conditions  in  which  the 
Anglo-Saxon  would  starve,  so  when  the  congestion  is  of  a  moderate 
grade,  the  simpler  and  hardier  white  and  yellow  connective  tissues  may 
exhibit  growth  and  proliferation  at  the  same  time  that  the  more  highly 
organized  specific  cells  of  a  tissue  manifest  progressive  atrophy.  In 
addition  to  relative  fibrosis  due  to  this  atrophy  of  the  nobler  elements,  in 
the  liver,  for  example,  we  have  to  recognize  definite  cases  of  productive 
fibrosis,  not,  we  would  emphasize,  when  the  congestion  is  extreme, 
but  when  it  has  been  of  a  moderate  grade  and  long  continued.  Thus 
there  is  developed  a  true  cyanotic  fibrosis  or  induration.  Where  the 
congestion  is  extreme,  where,  that  is,  there  is  complete  obliteration  of 
the  vein  or  veins  of  a  part,  with  inadequate  means  of  developing  a 
collateral  circulation,  there  the  stasis  of  the  blood  is  inevitably  followed 
by  necrosis  or  death  of  the  whole  area,  resulting  in  the  production  of 
gangrene  if  the  area  of  distribution  be  large,  e.  g.,  the  foot  or  leg  (vol.  i, 
p.  908),  or  of  hemorrhagic  infarct  if  the  terminal  vein  of  one  portion  only 
of  an  organ  be  involved. 

Local  Anemia. — Local  anemia  may  be  but  part  of  a  general  blood- 
lessness — as  after  a  profound  hemorrhage;  it  may  also  be  collateral, 
due  to  determination  or  drainage  of  blood  to  other  regions,  as  in  the 
anemia  of  the  brain  in  syncopal  attacks  following  upon  dilatation  of 
the  splanchnic  vessels  (vol.  i,  p.  529).  More  often,  however,  we  have 
to  take  into  account  the  results  of  local  disturbances  of  the  blood  supply 
through  particular  arteries,  leading  to  deficient  circulation  in  the  areas 
served  by  those  arteries.  The  causes  of  such  local  anemia  are  of  the 
same  order  as  are  those  of  local  passive  hyperemia,  though  here  it  is 
the  artery  passing  to  a  part  that  is  affected,  instead  of  the  vein  passing 
from  it,  and  the  results  are  widely  contrasted.  Disturbances  may  be 
brought  about  (1)  by  nervous  influences  acting  upon  the  arteries,  (2) 
by  pressure  upon  it  from  without,  (3)  by  disease  affecting  its  walls,  and 
(4)  by  obstruction  or  plugging  of  the  arterial  lumen  by  foreign  or  abnormal 
matter. 

1.  Neurotic  Anemia. — With  Lubarsch  we  can  (perhaps  somewhat 
insecurely)  divide  the  cases  in  which  individual  arteries  become  con- 
tracted through  nervous  influences  into  the  direct  and  the  reflex.  Thus, 
foremost  among  the  direct  he  places  the  local  anemias  due  to  the  action 
of  cold.  We  admit  freely  with  him  that  it  is  in  females  and  those  of 
a  high-strung  nervous  temperament  that  such  local  contractions  of  the 
arteries  most  easily  and  most  frequently  show  themselves.  But  in  the 
first  place,  we  are  inclined  to  believe  that  cold,  like  heat,  acts  directly 
on  the  muscles  of  the  arteries,  and  in  the  second,  to  doubt  whether  here, 
as  indicated  by  the  greater  sensitiveness  to,  and  perception  of,  cold  by 
the  affected  individuals,  there  is  not  also  a  reflex  element  present.  The 
case  does  not  seem  to  be  a  pure  one.  More  probably  Raynaud's  disease 
or  symmetrical  local  asphyxia  comes  into  this  category.  Here  we  deal 


HEMATOGENOUS  ANEMIA  31 

with  a  remarkable  condition,  at  first  spasmodic,  later  persistent,  of  con- 
traction of  certain  arteries,  usually  symmetrical,  most  commonly  of 
certain  fingers  and  toes,  although  the  whole  hand  or  the  pinnae  of  the 
ears  may  be  affected.  The  affected  parts  become  first  anemic  and  feel 
dead  or  tingle  ("pins  and  needles");  if  the  condition  continues  for  some 
hours,  they  gradually  become  blue  (through  lack  of  propulsion  of  the 
blood  which  slowly  accumulates  in  the  capillaries).  After  some  time 
the  condition  may  pass  off  and  the  affected  area  resume  its  normal 
appearance  and  warmth.  The  tendency  is  for  the  condition  to  last 
longer  and  longer  until  the  prolonged  anemia  leads  to  death  of  the  part 
and  gangrene.  Recent  observations  favor  the  view  that  in  these  cases 
we  deal,  if  not  at  first,  certainly  later,  with  definite  sclerotic  changes  in 
the  arteries.  It  is,  indeed,  difficult  to  grasp  the  condition  of  prolonged 
tonic  spasm  of  the  arterial  muscle.  The  cases  thus  may  come  into  line 
with  those  of  senescent  or  so-called  spontaneous  gangrene. 

The  type  reflex  anemia  is  seen  in  the  pallor  of  fright  or  strong  emotion ; 
it  is  noteworthy  that  an  emotion  which  in  the  one  individual  will  stimu- 
late the  vasodilators  and  produce  blushing,  in  another  stimulates  the 
vasoconstrictors  and  produces  pallor. 

2.  Exogenous  or  Pressure  Anemia. — Exogenous  or  pressure  anemia 
may  be  produced  by  the  compression  exerted  upon  the  artery  running 
to  an  organ,  by  tumors,  granulomas,  cicatricial  tissue,  parasitic  and  other 
cysts,  ligatures,  etc.     It  deserves  note  that  in  many  instances  this  form 
is  preceded  by  passive  congestion;  when  the  pressure  is  of  gradual 
development  there  is  a  first  stage  in  which  the  accompanying  vein  with 
its  lower  blood  pressure  becomes  first  obstructed  and  then  occluded, 
with  the  result  that  blood  continues  to  be  pumped  into  the  organ  or 
part  through  the  still  functional  artery,  and  escapes  with  difficulty;  only 
with  increased  pressure  does  the  artery  undergo  obliteration. 

3.  Endogenous  Local  Anemia. — Endogenous  local  anemia  is  induced 
through  changes   in    the   arterial    wall    itself,  e.  g.,  sarcomatous   and 
other  growths,  or,  the  most  frequent  cause,  endarteritis  obliterans  (see 
Chapter  VIII). 

4.  Hematogenous  Anemia. — Hematogenous  anemia  is  brought  about 
by  coagulation  of  blood  within  an  artery — thrombosis  (Chapter  II),  or 
by  obstruction  of  the  artery  by  foreign  matter  borne  along  the  blood 
stream— embolism.     The  various  substances  which  can  act  as  emboli 
will  be  discussed  more  fully  later  (Chapter  II). 

The  results  of  defective  blood  supply  to  a  part,  and  local  anemia,  are 
primarily:  (1)  Pallor;  (2)  some  reduction  in  size  owing  to  lessened  filling 
of  the  vessels;  (3)  firmer  consistency;  (4)  lowered  temperature  of  the  part; 
(5)  arrested  function.  With  these  there  may  or  may  not  be  secondary 
disturbances,  either  a  dead  feeling,  or  sometimes  intense  pain,  e.  g., 
intense  cardiac  pain  when  a  branch  of  the  coronary  artery  becomes 
blocked,  or  agonizing  cramps  when  there  is  anemia  of  the  muscles  of  a 
lower  limb. 

The  ultimate  effects  may  or  may  not  be  of  a  more  serious  type,  all 
depending  upon  the  distribution  of  the  vessels  in  the  affected  part: 


32  THE  BLOOD 

(a)  When  through  abundant  arterial  anastomoses  a  collateral  circulation 
is  rapidly  developed,  the  disturbance  may  be  but  transient.  (6)  Where 
arterial  anastomoses  are  present,  but  the  area  supplied  by  the  blocked 
artery  is  large  relative  to  the  size  of  the  collateral  vessels,  it  is  obvious 
that,  at  first,  the  region  thus  deprived  of  its  normal  blood  supply  obtains 
inadequate  nourishment,  and  its  functional  activity  is  gravely  depressed. 
Such  conditions  we  find  obtaining  characteristically  in  the  lungs,  in  which 
every  region  has  a  double  arterial  supply,  in  the  main  from  the  branches 
of  the  pulmonary  artery,  but  in  addition  from  the  (smaller)  branches  of 
the  bronchial  arteries,  (c)  W7hen  the  artery  is  truly  terminal.  We  shall 
discuss  the  results  in  the  following  chapter. 


CHAPTEE    II. 

THE  BLOOD— THE  EFFECTS  OF  CLOSURE  OF  VESSELS. 

IT  has  been  customary  to  discuss  the  effects  of  arrest  of  circulation 
through  a  vessel  in  connection  with  the  treatment  of  ligation  (in  surgical 
works),  or  of  embolism  and  thrombosis.  The  result  has  been  not  a 
little  repetition,  and  a  tendency  to  regard  the  results  as  due  to  one  or 
other  particular  cause,  and  not  as  the  natural  outcome  of  the  simple 
process  of  occlusion.  It  seems  to  us  better  to  take  up  the  broader 
subject  of  occlusion  first,  and  later  to  deal  with  particular  modes. 

It  is  obvious,  in  the  first  place,  that  arrest  of  the  blood  flow  through  a 
particular  vessel,  the  general  circulation  continuing,  may  be  brought 
about  in  three  main  ways:  (1)  By  pressure  acting  from  without;  (2) 
by  overgrowth  of,  or  growth  within,  the  vessel  walls,  either  concentric 
and  diffuse,  or  localized,  so  that  the  lumen  becomes  more  and  more 
reduced  until  it  is  wholly  obliterated;  or  (3)  by  blockage  from  within. 
The  pressure  from  without  may  be  very  variously  produced — mechan- 
ically, as  by  ligature,  or  from  general  compression  exerted  over  a  part  or 
over  a  particular  vessel  (e.  g.,  by  a  tourniquet),  by  the  growth  of  a  tumor 
or  cyst,  enlarged  glands,  cicatricial  bands,  etc.  The  great  cause  of 
diminution  of  the  vascular  lumen  by  overgrowth  of  the  vessel  wall  is 
arteriosclerosis  (endarteritis  obliterans).  The  corresponding  condition, 
phlebosclerosis,  does  occur,  but  rarely  proceeds  so  far  as  to  obliterate 
the  lumen  of  veins.  The  infiltration  of  the  intima  by  new-growth  may 
lead  to  like  results.  Blockage  from  within  is  due  to  either  one  of  two 
causes,  or  a  combination  of  the  two,  namely  thrombosis,  the  intravital 
and  intra vascular  "  clotting"  of  the  blood,  and  embolism,  the  arrest  of 
matter  foreign  to  the  normal  blood  at  some  point  where  the  size  and 
configuration  of  the  vessel  prevents  further  progress  along  the  lumen. 
Such  foreign  matter,  if  of  irregular  shape,  may  not  at  first  cause  complete 
occlusion,  but  upon  it  there  may  be  deposit  of  platelets  and  clotting  of 
the  blood,  so  that  the  blockage  tends  to  become  complete. 

The  results  depend  upon  whether  we  deal  with  occlusion  of  an  artery 
or  of  a  vein. 

ARTERIAL  OCCLUSION. 

Many  factors  determine  the  results,  namely,  (1)  the  rate  of  narrowing 
or  occlusion  of  the  vessel,  (2)  the  presence  or  absence  of  anastomosing 
arteries,  (3)  the  size  of  the  artery  as  compared  with  the  collateral  vessels, 
(4)  the  extent  of  the  area  supplied  by  the  artery,  (5)  the  condition  of 
the  heart  and  arterial  blood  pressure,  (6)  the  venous  blood  pressure, 
3 


34 


ARTERIAL  OCCLUSION 


(7)  the  rate  of  flow,  or  perhaps  more  accurately,  the  difference  between  the 
arterial  and  venous  pressure  of  a  part. 

We  have  placed  in  the  first  place  what  at  first  thought  would  seem 
to  be  a  minor  factor;  further  consideration  shows  that  there  is  no  factor 
that  so  materially  modifies  the  result.  The  slow  occlusion  of  an  artery 
extending  over  days  before  it  is  complete  permits  the  development  of 
an  adequate  collateral  circulation,  so  that  when  occlusion  becomes  com- 
plete the  region  supplied  by  the  blocked  artery  may  exhibit  not  a  sign 
of  disturbed  nutrition;  the  sudden  occlusion  of  an  artery,  as  by  an  em- 
bolus,  may  be  productive  of  grave  disturbances,  even  of  necrosis  in  the 
area  of  supply,  and  this  notwithstanding  the  presence  of  collateral  anas- 
tomosing vessels.  Before  these  vessels  become  sufficiently  dilated  to 
afford  an  adequate  blood  supply,  the  imperfect  aeration  may  have  led 
to  tissue  death. 

The  presence  or  absence  of  anastomosing  arteries  is  a  very  important 
factor.  •  As  Cohnheim  pointed  out,  the  arteries  of  the  body  are  of  two 
types,  the  anastomosing  and  the  terminal,  as  indicated  in  the  accompany- 
ing diagrams. 

FIG.  1 


Schema  of  an  anastomosing  circulation.  If  a  branch  be  ligatured  or  blocked  as  at  a,  the 
region  supplied  by  that  branch  receives  abundant  blood  through  the  anastomoses  between  it 
and  other  arteries,  b  and  c.  At  most  there  is  an  arrested  circulation  in  the  artery  itself  as  far 
as  the  nearest  points  of  branching  or  anastomosis  above  and  below. 

ANASTOMOSING  ARTERIES — COLLATERAL  CIRCULATION. — It  is  obvious 
that  in  those  of  the  former  order,  ligature  or  blockage,  say  at  a,  will  have 
very  little  effect  upon  the  circulation  of  the  area  beyond;  blood  can  so 
easily  pass  into  the  area  from  the  arteries  b  and  c.  The  case  presents  no 
difficulties  under  the  simple  conditions  exhibited  in  the  diagram.  The 
conditions,  however,  are  not  always  so  simple.  A  large  and  important 
artery,  such  as  one  of  the  iliacs,  may  become  obliterated.  The  dorsal 
aorta,  even,  through  imperfect  development,  as  in  the  condition  known 
as  coarctation,  may  be  either  excessively  narrowed  or  completely  blocked 
just  above  the  region  of  entrance  of  the  ductus  Botalli.  And  notwith- 
standing the  arrest  of  blood  flow  through  such  important  channels, 


ANASTOMOSING  ARTERIES— COLLATERAL  CIRCULATION       35 

instead  of  the  parts  supplied  becoming  necrosed,  we  find  that  they 
receive  sufficient  nutrition  to  remain  alive,  nay,  more,  after  a  time  the 
circulation  through  them  may  become  wholly  sufficient  for  the  demands 
made  upon  the  region  in  the  course  of  every-day  existence.  In  these 
cases  we  deal,  it  is  true,  with  the  same  phenomenon,  but  with  this  differ- 
ence, that  the  anastomosing  or  collateral  vessels  are  often  vessels  which 
normally  are  inconspicuous,  that  it  is  not  merely  the  branches  im- 
mediately above  and  below  the  obliteration  that  carry  the  necessary 
blood,  that  vessels  over  a  singularly  wide  area  may  be  involved,  and 
the  collateral  channels  may  be  curiously  round  about.  An  extraordinary 
and  widespread  series  of  arterial  anastomoses  and  enlarged  collateral 
channels  is  to  be  made  out  in  the  cases  of  congenital  coarctation  of  the 
aorta.  Apart  from  persistence  of  the  ductus  arteriosus,  what  are 


The  main  trunks  of  a  collateral  circulation  established  by  means  of  the  intercostals  and  neck 
vessels  between  the  arch  of  the  aorta  and  the  dorsal  aorta  in  a  case  of  coarctation  or  congenital 
obstruction  of  the  aorta  in  the  region  of  the  ductus  Botalli.  1,  region  of  coarctation;  2,  anas- 
tomotic  branches  between  the  intercostal  arteries;  3,  subclavian  arteries.  From  a  man,  aged 
thirty-five  years.  (After  J.  F.  Meckel  and  Thoma.) 

ordinarily  unrecognizable  anastomoses  between  the  intercostal  arteries 
become  developed  into  arteries  of  the  first  rank,  and  a  series  of  large 
anastomoses  is  to  be  made  out  between  the  arteries  of  the  root  of  the 
neck  and  the  upper  intercostals,  through  which  blood  is  conveyed  into 
the  aorta  below  the  obstruction. 

What,  it  may  be  asked,  is  the  process  whereby  these  small  anas- 
tomosing vessels  become  enlarged,  with  hypertrophy  of  their  walls, 
so  as  to  function  as  main  channels?  It  cannot  be  said  that  we 
wholly  understand  the  process.  Thoma,  who  has  especially  studied 
the  subject,  has  laid  down  certain  principles,  but  these  do  not  explain ; 
they  only  state  the  facts  as  we  find  them.  With  Thoma,  we  are  forced 


36  ARTERIAL  OCCLUSION 

to  see  that  it  is  not  primarily  an  active  distension  of  the  collateral 
vessels  through  increased  pressure.  It  is  true  that  where  a  large  vessel 
becomes  suddenly  occluded,  that  occlusion  induces  increased  pressure 
in  the  vessel  (and  its  branches)  behind  the  region  of  obliteration, 
but  this  is  only  a  temporary  state;  within  a  very  short  time  the  blood 
becomes  redistributed  and  the  pressure  falls  to  the  normal.  As  von 
Recklinghausen  pointed  out,  the  important  factor  is  the  rate  of  blood 
flow.  And  here  it  must  be  noted  that  that  rate  is  determined  primarily 
by  relative  pressure,  i.  e.,  by  the  difference  in  pressure  in  the  vessels 
above  the  obstruction  and  those  in  the  area  whose  supply  has  been  cut 
off;  the  greater  the  difference  between  these  two  pressures,  the  more  rapid 
the  flow  of  blood  into  the  part  through  vessels  that  are  still  open.  Thoma 
lays  down  as  his  first  principle  that  the  distension  of  the  vessels  is 
dependent  upon  the  rate  of  flow  through  them.  This,  however,  docs 
not  carry  us  very  much  farther.  Immediately  after  ligature  or  other 
obstruction,  the  pressure  in  the  artery  beyond  the  obstruction  sinks  to 
zero.  Blood  then  pours  into  the  artery  from  anastomosing  vessels;  the 
greater  the  pressure  in  the  artery  above  the  obstruction,  the  greater 
the  rate  of  the  flow  through  those  vessels.  But  circulation  cannot  be 
reestablished  through  the  capillaries  of  the  area  supplied  by  the 
blocked  artery  until  the  pressure  in  those  capillaries  becomes  higher 
than  that  in  the  veins.  Or  otherwise  the  time  must  come — and  that 
relatively  soon,  unless  the  area  of  supply  is  to  die  from  stagnation  of 
the  contained  blood — when  in  the  vessels  forming  the  anastomoses 
there  is  no  marked  departure  from  the  normal  pressure  difference 
between  artery  and  vein,  and  when,  therefore,  the  rate  of  flow  must 
tend  toward  the  normal  and  the  dilatation  of  these  collateral  vessels, 
according  to  Thoma's  principle,  must  be  brought  to  an  end  and  should 
give  place  to  contraction. 

We  would  suggest  that  another  principle  has  to  be  taken  into  account, 
not  considered  by  Thoma — the  principle  akin  to  that  of  Harris' 
"  functional  inertia,"  noted  in  our  first  volume  (p.  89),  the  principle 
of  "overadaptation,"  which  time  and  again  we  observe  in  vital  phe- 
nomena. We  would  thus  suggest  that  when  collateral  vessels  expand, 
owing  to  increased  rate  of  blood  flow  through  them,  the  expansion  is  in 
greater  ratio  than  the  increase  in  rate  of  flow,  with  the  result  that  the 
capillary  pressure  in  the  area  of  supply,  instead  of  being  lower,  tends  to 
become  even  higher  than  that  in  the  surrounding  tissues,  whereby,  in 
place  of  stagnation  of  the  blood,  an  active  circulation  is  favored,  i.  e., 
a  greater  "fall"  into  the  efferent  veins.  We  fully  accept  Thoma's 
second  mechanical  principle,  that  with  dilatation  of  a  vessel  the  added 
strain  of  the  larger  stream  of  blood  passing  through  it  leads  to  growth 
of  the  walls — provided  that  the  strain  be  not  excessive  (see  vol.  i,  p.  541). 
Thus  it  is  that  what  had  been  little  more  than  arterioles  may  become 
developed  into  relatively  large,  thick-walled  arteries. 

In  other  words,  a  collateral  circulation  can  be  maintained  only  in 
those  cases  in  which  the  anastomosing  vessels  are  sufficiently  large 
or  sufficiently  numerous  to  preserve,  when  dilated,  the  circulation  in 


ANASTOMOSING  VEINS  37 

the  area  beyond  the  obliteration.  Where  they  are  inadequate,  there  they 
pour  blood  into  the  area,  and  in  the  absence  of  a  sufficient  vis  a  tergo, 
the  blood  stagnates  in  the  part,  and  by  its  stagnation  the  flow  through 
the  collateral  vessels,  instead  of  continuing,  becomes  arrested,  and  infarct 
formation  or  gangrene  is  the  necessary  consequence. 

ANASTOMOSING  VEINS. — In  the  case  of  the  veins,  we  have,  it  is  true,  a 
somewhat  different  condition  of  affairs;  the  blood  passing  through  the 
collateral  vessels  has  not  to  supply  later  a  capillary  area;  it  has  merely  to 
find  its  way  into  another  vein  nearer  the  heart.  The  danger  here  is 
not  one  of  stagnation  in  front,  but  of  stagnation  in  the  capillary  area 
behind. 

Venous  anastomoses  are  freer  and  more  widespread  than  are  arterial, 
and,  as  a  result,  the  extent  of  the  collateral  circulation  set  up  is  at  times 
very  extraordinary.  Thus,  when  the  portal  vein  becomes  seriously 
obstructed,  the  blood  from  the  portal  area  may  find  its  way  through 
a  very  wide  series  of  tortuous  collateral  channels — through  the  coronary 
veins  of  the  stomach  into  the  cesophageal  veins,  through  those  of  the 
gastro-epiploic  omentum  to  the  diaphragm  and  so  into  the  vena  azygos, 
through  anastomoses  between  the  inferior  mesenteric  and  the  hemor- 
rhoidal  veins,  and  through  the  retroperitoneal  veins  of  Retzius  joining 
the  radicles  of  the  portal  veins  in  the  mesenteries  with  branches  of  the 
inferior  vena  cava,  as  also  through  the  veins  of  the  round  and  suspensory 
ligaments  of  the  liver  to  the  epigastric  and  mammary  veins.  When  the 
common  iliac  veins  are  occluded,  or  the  lower  end  of  the  inferior  vena 
cava,  we  similarly  find  the  blood  from  the  lower  extremities  conveyed 
through  the  epigastrics  and  veins  of  the  anterior  abdominal  wall  up 
to  the  mammary  veins,  with  the  formation  of  a  pronounced  caput 
medusae,  or  congeries  of  tortuous  distended  veins  in  the  region  of  the 
navel. 

The  obliteration  of  a  large  vein  coming  from  a  part  must  undoubtedly 
be  followed  by  a  marked  rise  of  pressure  behind  the  obstruction,  and 
that  rise  and  the  accompanying  dilatation  of  the  capillaries  of  the  area 
is  frequently  accompanied  by  oedema  and  accumulation  of  fluid  in  the 
tissues  and  spaces.  Where  it  occurs,  this  indicates  that  adequate  col- 
lateral circulation  is  not  immediately  developed,  and  that  for  some  period, 
at  least,  the  circulation  through  the  affected  part  is  impeded.  The  rise 
of  pressure  must  materially  increase  the  rate  of  flow  through  the  col- 
lateral veins.  Certainly  the  impression  given  is  that  the  combined  cross- 
section  of  these  collaterals  comes  in  many  cases  to  exceed  that  of  the 
obliterated  vessel,  and  that  the  occlusion  gains  more  than  compensation. 
On  the  other  hand,  the  great  tortuosity  of  these  vessels  and  their  long 
and  often  roundabout  course  has  to  be  taken  into  consideration,  as 
counteracting  the  free  discharge  of  blood  from  the  affected  area.  On  the 
whole,  we  see  no  reason  to  imagine  that  the  same  principles  are  not  at 
work  in  the  case  of  the  development  of  a  venous  collateral  circulation 
as  in  that  of  an  arterial,  and  we  think  that  for  there  to  be  free  drainage, 
the  dilatation  of  the  anastomosing  vessels  must  be  more  than  proportional 
to  the  increased  rate  of  flow  through  them. 


38 


ARTERIAL  OCCLUSION 


TERMINAL  ARTERIES. — The  case  is  very  different  in  cases  of  the  latter 
order.  Here  ligature  at  a  wholly  cuts  off  the  arterial  supply  to  the  region 
beyond.  The  only  supply  that  can  reach  it  is  either  by  back  flow  through 
the  vein  of  the  part,  or  through  the  abundant  capillary  anastomoses  at  the 
periphery  of  the  area.  Not  to  enter  into  what  has  been  quite  an  active 
discussion,  we  may  say  that  the  first  of  these  methods  is  now  generally 
discredited.  Such  back  flow  would  not  create  a  circulation,  whereas 
microscopic  examination  of  the  peripheral  capillaries  shows  that  they 
become  greatly  dilated  and  distended  with  blood.  Whatever  circulation 
or  entrance  of  blood  occurs  into  the  part  we  now  attribute  to  those 
capillaries.  But  these  may  be  inadequate  to  nourish  the  part,  save, 
perhaps,  at  the  very  edge  of  the  area  of  arterial  supply,  and  as  a  result, 
the  main  bulk  of  the  tissue  undergoes  a  rapidly  developing  necrosis,  and 
an  infarct  is  produced. 


FIG.  3 


L 


Schema  of  a  terminal  arterial  system  in  which  the  anastomoses  are  only  between  the  capillary 
loops.  It  will  be  seen  that  a  ligature  or  obstruction  at  the  point  a  may  cut  off  the  whole  blood 
supply  of  the  region  supplied  by  the  obstructed  artery  and  its  branches,  unless  the  capillary 
circulation  provided  by  neighboring  arteries  be  so  abundant  as  to  afford  nourishment  to  the 
blocked  area. 


The  terminal  arteries  of  the  body  as  usually  given  are:  those  of  the 
kidney,  brain  and  spinal  cord,  spleen,  the  branches  of  the  pulmonary 
arteries,  the  coronary  arteries,  arteria  centralis  retinae,  and  superior 
mesenteric  artery.  More  accurately,  these  are  the  arteries  in  connection 
with  which  infarct  formation  is  encountered.  Further  investigation 
shows  that  these  are  not  necessarily  terminal  arteries  in  the  strict  sense, 
so  that  Cohnheim  himself  was  forced  to  modify  his  views,  and  speak 
of  functional  end  arteries.  The  branches  of  the  superior  mesenteric 
notoriously  have  free  arterial  communication  with  those  above  and 
below,  and  yet  true  and  often  very  extensive  infarction  of  the  small 
intestine  is  encountered.  The  same  is  true  of  the  coronary  arteries; 
these  are  not  devoid  of  definite,  if  small,  anastomoses,  and  the  same 
may  be  said  of  the  brain,  and  even  of  the  kidney.  In  the  lung,  as  again 


INFARCT  FORMATION  39 

in  the  liver,  while  the  pulmonary  and  hepatic  arteries  are  of  the  terminal 
type,  they  do  not  afford  the  sole  blood  supply  to  individual  areas;  in  the 
one  case  branches  of  the  bronchial  artery  open  into  the  same  capillary 
network,  in  the  other  the  portal  vein  supplies  abundant  blood.  We 
come  back  then  to  this :  that  it  is  not  the  absence  of  anastomoses  or  of 
other  arteries  supplying  the  same  area  that  is  essential  to  infarct  formation, 
but  the  absence  of  arterial  anastomoses  sufficiently  large  to  insure  the 
proper  nutrition  of  a  part  once  the  main  nutrient  vessel  to  that  part  becomes 
suddenly  occluded. 

Infarct  Formation. — What  then  is  the  nature  of  infarct  formation? 
When  such  a  main  nutrient  artery  becomes  occluded,  collateral  arterial 
supply  is  inadequate  to  cause  a  sufficient  circulation  through  the  asso- 

FIG.  4 


White  infarct  of  the  spleen.     Section  was  made  through  one  infarct  at  a  and  another  at  6,  the 
organ  being  thereby  laid  open.     (From  the  Pathological  Museum  of  McGill  University.) 

ciated  capillary  area,  and,  owing  to  the  cutting  off  of  the  main  vis  a 
tergo,  the  blood  pressure  in  the  area  sinks  either  to  nil  or,  when 
there  exist  small  collateral  arterial  supplies  to  the  area,  to  a  point  which 
is  below  even  the  venous  pressure  of  the  surrounding  tissue.  As  a 
consequence,  the  specific  tissue  cells  of  the  part  no  longer  gain  sufficient 
oxygen;  anabolism  is  arrested,  although  catabolism  and  disintegration  of 
these  substances  may  continue  and  result  in  the  diffusion  of  carbon  dioxide, 
and  other  end  products  into  the  lymph  and  blood.  We  may  compare  the 
process  with  the  continued  discharge  of  carbon  dioxide  by  the  frog  placed 
in  an  oxygen-free  atmosphere,  or  the  liberation  of  carbon  dioxide  from  the 
removed  kidney  through  which  is  perfused  an  oxygen-free  salt  solution. 


40  ARTERIAL  OCCLUSION 

The  result  would  seem  to  be  a  poisoning  of  the  tissue  with  the  products  of 
its  own  disintegration.  While  this  is  proceeding,  blood  tends  to  pour 
into  the  capillaries  of  the  part  from  the  surrounding  capillaries  with  their 
higher  blood  pressure.  The  extent  of  this  passage  we  shall  discuss  later, 
but  with  it,  following  Weigert's  conception  of  the  process,  there  would 
appear  to  be  some  increased  transudation  of  fluid  from  the  capillaries, 
and  absorption  of  the  same  by  the  tissue  cells.  The  nuclei  of  those 
cells  lose  their  chroma  tin.  Whether  this  diffuses  out,  or  is  converted 
into  non-stainable  matter,  must  be  left  an  open  question.  And  now, 
through  the  formation  or  liberation  of  a  thrombin  or  fibrin  ferment, 
the  whole  area,  cells,  lymph,  and  blood,  undergoes  a  process  of  coagula- 

FIG.  5 


Anemic  infarct  of  cortex  of  kidney  to  show  coagulation  necrosis,  with  surrounding  zone 
of  congestion;  o,  artery.     (Orth.) 

tion;  they  pass  into  a  condition  of  coagulation  necrosis.  The  whole 
affected  area — usually  wedge-shaped  in  consequence  of  the  fan-like 
distribution  of  the  branches  of  the  affected  artery — assumes  a  more 
solid  consistence  than  the  surrounding  tissue,  is  swollen,  so  that  if  it 
abuts  upon  the  surface  of  an  organ  it  projects  somewhat,  and  on  section 
it  cuts  firmly,  and  is  sharply  defined.  It  may  be  either  of  a  pale,  grayish 
yellow  color — white  or  anemic  infarct,  or  of  a  deep  blood-red — red  or 
hemorrhagic  infarct. 

White  Infarct. — The  white  infarct  is  encountered  almost  constantly 
in  the  kidney,  frequently  in  the  brain,  heart,  and  liver,  less  frequently 


RED  INFARCT  41 

in  the  spleen,  rarely  in  the  skin  and  intestine,  practically  never  in  the 
lung.1  Where  recent,  the  finer  histology  manifests  a  peripheral  greatly 
congested  zone,  in  which  the  capillaries  are  greatly  distended  with  blood, 
and  within  this,  an  almost  hyaline  mass,  in  which  the  outlines  of  the 
constituent  tissues  can  be  faintly  distinguished,  the  constituent  cells 
appearing  to  run  into  each  other;  the  nuclei  are  invisible,  and  the 
whole  area  has  a  homogeneous,  unstained  appearance. 

Red  Infarct. — This,  as  regards  incidence,  is  the  converse  of  the  white; 
is  practically  always  the  form  present  in  the  lung,  is  the  commonest 
form  met  with  in  the  intestine,  is  rarely  present  in  the  kidney,  etc.  We 
would  add  that  the  so-called  red  infarcts  of  the  liver,  due  to  obstruction 
of  branches  of  the  portal  vein,  cannot  be  considered  as  genuine  infarcts; 
they  exhibit,  it  is  true,  enormous  congestion  of  the  capillaries,  but  the 
liver  cells  and  vessel  walls  are  not  necrotic.  There  is  still  in  them  a 
collateral  circulation  sufficient  to  prevent  cell  death. 

WThere  recent,  microscopic  examination  shows  that  not  only  are  the 
capillaries  throughout  the  red  infarct  greatly  distended  with  coagulated 
blood,  but  in  addition  there  is  abundant  evidence  of  hemorrhage;  the  tissue 
spaces  are  filled  with  extra vasated  blood  cells.  There  is  no  evidence  of 
actual  rupture  of  the  capillaries,  the  hemorrhage  has  been  per  diapedesin. 
The  tissue  cells  of  the  affected  area  show  necrosis  and  absence  of  staining 
of  the  same  order  as  that  seen  in  the  white  infarct.  Here  one  partial 
exception  must  be  made :  in  most  red  infarcts  of  the  lung  we  have  been 
able  to  recognize  still  the  nuclei  of  the  capillary  endothelium,  staining, 
it  is  true,  more  feebly  than  normal,  but  standing  out  with  some  promi- 
nence in  contrast  with  the  surrounding  non-staining  tissues.  This  is, 
we  hold,  correlated  with  the  existence  of  a  second  arterial  blood  supply 
to  the  part  through  the  bronchial  arteries.  Such  tissue  is  not  absolutely 
necrotic,  and  through  it  the  circulation  can  eventually  become  restored; 
considerations  which  explain  the  singular  rareness  of  indications  of  old 
cicatrized  infarcts  in  the  lungs.  It  is,  indeed,  singular  how  rare  it  is  to 
encounter  anything  that  may  be  regarded  as  the  cicatrix  of  a  previous 
infarct  in  the  lung,  and  yet  infarction  at  a  previous  period  must  be 
common,  especially  in  cases  of  long-standing  cardiac  disease.  Where, 
as  in  the  lung,  there  is  this  double  circulation,  we  must  conclude  that  in 
a  certain,  it  may  be  a  large,  proportion  of  cases  the  vitality  of  the  tissues 
is  not  entirely  lost,  and  that  subsequent  resolution  and  regeneration  of  the 
alveolar  epithelium,  etc.,  is  possible,  the  part  returning  to  the  status 
quo  ante.  We  thus  recognize  a  series  of  transitional  cases,  through  the 
"red  infarct"  of  the  liver,  with  little  or  no  necrosis,  these  red  infarcts  of 
the  lung,  with  necrosis  of  some,  but  not  of  all  the  component  tissues, 
up  to  the  infarct  proper,  with  complete  coagulation  necrosis. 

Here  also,  in  passing,  it  must  be  emphasized  that  the  red  infarct  is 

1  We  have  on  two  occasions  encountered  soft,  gray  infarcts  in  the  lung,  but  these 
were  obviously  late  stages  in  the  resolution  of  a  red  infarct,  with  breaking  down  of 
the  erythrocytes,  and  diffusion  out  of  the  hemoglobin,  and  in  one  of  the  two  cases, 
beginning  organization. 


42  ARTERIAL  OCCLUSION 

not  only  due  to  arterial  obliteration,  but  may  equally  be  produced  by 
blocking  of  the  efferent  vein  of  an  organ  or  part  of  an  organ.  Pro- 
vided that  there  be  no  adequate  venous  anastomoses,  the  continued 
pouring  of  blood  into  the  part  by  the  artery  leads  to  overdistension 
of  the  capillaries,  stagnation  of  the  blood,  tissue  necrosis,  and  hemor- 
rhage. The  picture  is  to  all  intents  and  purposes  identical  with 
that  of  arterial  red  infarct,  save  that  the  congestion  and  hemorrhage 
tend  to  be  extreme.  What  is  perhaps  the  best  example  of  this  form 
is  occasionally  encountered  in  infants  in  the  hemorrhagic  infarct  of 
the  adrenal,  a  rare  cause  of  relatively  sudden  death.  The  primary 
disturbance  would  seem  to  be  thrombosis  of  the  adrenal  vein.  The 
organ  is  converted  into  a  firm,  swollen,  intensely  hemorrhagic  mass  of 
dead  tissue.  It  is  still  a  matter  of  controversy  whether  some,  at  least, 
of  the  red  infarcts  of  the  lung  are  not  similarly  due,  not  to  embolism  of 
branches  of  the  pulmonary  artery,  but  to  thrombosis  of  branches  of 
the  pulmonary  vein.  It  is  quite  possible  that  this  may  be  the  case;  but, 
on  the  other  hand,  the  more  carefully  these  pulmonary  infarcts  are  studied, 
the  greater  is  the  number  in  which  arterial  embolism  is  encountered. 
We  must  thus  conclude  that  pulmonary  infarct  of  venous  origin  is  the 
exception  and  not  the  rule. 

The  Mode  of  Production  of  Red  and  White  Infarcts. — Why  under  certain 
circumstances  and  in  certain  organs  we  obtain  white  infarcts,  and  in 
others  red,  has  been  angrily  debated  for  now  more  .than  a  generation. 
Intimately  associated  with  this  has  been  the  whole  study  of  the  mechan- 
ism of  infarct  formation,  and  many  of  the  positions  taken  have  truly 
had  more  bearing  upon  this  latter  problem  than  upon  the  former.  It 
is  generally  accepted  that  when  the  arterial  supply  to  a  part — or  the 
venous  discharge — is  slowly  obliterated,  infarct  formation  does  not  occur. 
Time  is  then  given  for  the  establishment  of  collateral  circulation.  For 
infarct  formation  there  must  be  relatively  sudden  arrest  of  the  circula- 
tion in  a  part.  It  is  accepted  also  that  infarct  formation  may  occur 
even  when — as  in  the  lungs,  for  example,  and  the  intestines — a  collateral 
arterial  supply  is  present,  but  is  inadequate  to  provide,  of  a  sudden,  suffi- 
cient nourishment  to  the  area.  Again,  it  is  generally  admitted  that  the 
blood  that  pours  into  an  infarctous  area  does  not  enter  by  means  of 
back  pressure  through  the  veins,  but  through  the  capillary  anastomoses; 
there  is  a  higher  blood  pressure  in  the  surrounding  capillaries  than  in 
the  veins.  But  reviewing  the  ancient  controversy,  it  is  abundantly 
evident  that  considerations  of  general  blood  pressure,  or  capillary  and 
relative  arterial  and  venous  blood  pressure  give  no  satisfying  explanation 
why,  in  some  cases,  the  area  becomes  overfilled  with  capillary  blood  until 
hemorrhage  occurs,  and  in  others  the  coagulation  necrosis  occurs  without 
those  phenomena  showing  themselves.  We  have  passed  over  the  con- 
troversy very  rapidly  because  we  believe  that  these  discussions  upon  rela- 
tive blood  supply,  and  the  conditions  affecting  it,  are,  at  the  most,  of  but 
secondary  importance.  The  explanation  is  to  be  sought  along  wholly 
different  lines,  and  has  been  afforded  by  the  more  recent  studies  upon 
autolysis  and  tissue  survival.  It  is,  we  hold,  a  fact  of  high  significance 


THE  MODE  OF  PRODUCTION  OF  RED  AND  WHITE  INFARCTS     43 

that  white  infarcts  involve  just  those  tissues  which  either  have  been 
recognized  as  most  rapidly  succumbing  to  circulatory  arrest,  or,  on  the 
other  hand,  are  those  which  after  death  afford  the  most  abundant  pro- 
teolytic  and  other  ferments,  and  undergo  autolysis  most  rapidly.  Thus, 
for  example,  it  is  the  heart  muscle  that  of  all  the  muscles  in  the  body  first 
shows  rigor  mortis,  and  first,  through  autolysis,  passes  out  of  that  state; 
the  nervous  tissue  that  first  shows  lack  of  response  (i.  e.,  dies)  when  its 
blood  supply  is  cut  off;  the  cortical  tissue  of  the  kidney  that  has  empiric- 
ally been  selected  to  demonstrate  the  necrotic  effects  of  temporary  arterial 
ligature  (Litten's  experiment,  vol.  i,  p.  855);  the  liver,  kidney,  spleen, 
and  heart  muscle  that  most  rapidly  exhibit  autolysis.  Or,  otherwise, 
it  is  just  those  organs  that  either  are  most  susceptible  to  arrest  of  blood 
supply,  and  whose  cells  die  with  relative  ease,  or  again,  those  which, 
dying,  discharge  abundant  autolytic  enzymes,  that  may  exhibit  white 
infarct  formation.  Thus,  we  must  assume  that  the  essential  cause  of 
the  white  infarct  is  the  relatively  rapid  death  of  the  constituent  cells 
of  the  affected  area,  with  liberation  of  thrombin — or  prothrombin — and 
that  coagulation  ensues,  or  rigor  (for  the  two  processes  would  seem 
to  be  of  the  same  order),  before  the  capillary  anastomoses  have  widened 
sufficiently  to  induce  hemorrhage.  That  in  some  organs  we  encounter 
now  white,  now  red,  infarcts  would  seem  to  gain  its  simplest  explanation, 
not  primarily  in  variations  in  blood  pressure  and  in  the  rapidity  with 
which  blood  finds  its  way  into  the  vessels  of  the  infarctous  area,  but, 
as  Lubarsch  points  out,  in  the  state  of  the  cells  at  the  moment  when 
the  circulation  is  cut  off.  He  gives  an  excellent  example  in  proof.  In 
the  healthy  rabbit  it  is  only  after  ligature  of  the  renal  artery  for  about 
an  hour  and  a  half  that  necrobiosis  of  the  kidney  is  induced.  If,  how- 
ever, the  animal  be  inoculated  with  diphtheria  toxins,  or  better,  if  acute 
nephritis  be  set  up  by  intravenous  injections  of  ammonium  chromate, 
and  in  the  course  of  the  next  day  the  renal  artery  be  ligated,  now  closure 
for  only  three-quarters  to  an  hour  is  needed  to  induce  complete  infarction 
of  the  whole  kidney,  recognizable,  not  immediately,  but  eight  hours  or 
so  after  the  temporary  ligature.  He  points  out  that  by  repeated  closure 
of  the  artery  for  half-hour  periods,  eventually  the  kidney  cells  become 
so  susceptible  that  half-hour  closure  is  followed  by  infarction.  We  may 
recall  the  parallel  observations  upon  the  great  variation  in  the  onset  of 
rigor  in  muscles.  Where  the  muscle  has  been  overexercised,  as  in  the 
hunted  animal,  this  may  follow  immediately  upon  death;  in  other  cases 
putrefaction  may  ensue  before  it  can  show  itself.  And  so  it  has  to  be  kept 
in  mind  that  infarct  formation  does  not  necessarily  follow  upon  closure 
of  a  terminal  or  functionally  terminal  artery.  The  state  of  the  tissues 
determines  whether  coagulation  necrosis  does  or  does  not  become  de- 
veloped. This  is  particularly  noticeable  in  the  lung.  We  have  already 
pointed  out  that  here  infarction  is  often  incomplete,  but  in  addition  it 
has  to  be  noted  that  a  large  proportion  of  cases  of  embolism  of  branches 
of  the  pulmonary  artery  fail  to  show  any  sign  of  infarct  formation, 
This  is  more  especially  the  case  where  large  branches,  such  as  those 
supplying  a  whole  lobe,  become  blocked.  In  these  the  most  we  obtain 


44  ARTERIAL  OCCLUSION 

is  congestion.  It  is  quite  possible  that  another  factor  comes  into  opera- 
tion here.  Weigert's  postulate  has  to  be  kept  in  mind,  namely,  that  for 
coagulation  necrosis  to  ensue,  there  must  not  only  be,  as  we  may  express 
it,  the  liberation  of  a  prothrombin  from  the  dying  tissues,  but  a  some- 
thing supplied  to  the  affected  tissues  by  serous  infiltration  from  the  blood- 
vessels before  coagulation  can  ensue.  Where  a  large  tissue  area  has  its 
blood  supply  suddenly  cut  off,  the  pressure  in  the  capillaries  of  that  area 
may  never  reach  a  sufficient  height  to  permit  this  exudation,  or  only 
permits  it  at  a  period  when  further  autolytic  changes  have  already 
manifested  themselves,  and  coagulation  is  impossible.  Here  may  be 
the  explanation  why  infarct  formation  affects  only  relatively  small 
areas,  and  why,  when  the  blood  supply  is  cut  off  from  larger  areas — a 
limb,  for  example — then,  in  place  of  infarct  formation,  we  obtain  a  lique- 
f active  necrosis  or  gangrene. 

Here,  further,  it  must  be  pointed  out,  as  shown  by  the  researches  of 
Greenfield  and  Beattie,1  that  it  is  not  the  mere  presence  or  absence  of 
blood  in  the  vessels  of  the  obstructed  area  that  determines  the  existence 
of  red  or  white  infarct  respectively.  These  observers  state  that  in 
experimental  infarct  formation  in  all  cases  examination  during  the  first 
few  hours  shows  the  distension  of  the  capillaries  with  blood.  It  is  evident 
that  what  determines  whether  the  red  or  white  infarct  becomes  developed 
is  the  rate  of  onset  of  the  coagulation  necrosis — whether  this  occurs  after 
or  before  the  distension  has  led  to  hemorrhage. 

The  Results  of  Infarction. — 1.  Complete  Resolution. — This,  as  already 
indicated,  would  seem  to  occur  where  the  infarction  has  been  imperfect, 
as  in  the  lung.  We  have  evidence  of  the  existence  of  fibrinolytic  ferments 
derived  from  both  polynuclear  and  mononuclear  leukocytes,  as  again 
of  proteolytic  ferments,  broadly  of  a  tryptic  nature,  liberated  in  auto- 
lysis.  It  is  possible  thus  that  capillary  thrombi  undergo  complete  ab- 
sorption— that  the  hemorrhagic  products  in  the  lung  alveoli  become 
likewise  absorbed,  that  with  the  reestablishment  of  an  adequate  circu- 
lation the  vascular  endothelium  regains  complete  vitality,  as  again,  that 
the  alveolar  epithelium  becomes  regenerated  from  the  periphery. 

2.  Organization  and  Cicatrization. — This  is  the  more  typical  event. 
From  the  peripheral  zone  of  congested  capillaries  abundant  leukocytes 
pass  into  the  necrosed  area.     Partly  through  the  heterolytic  enzymes, 
partly,  it  may  be,  through  the  autolytic,  the  necrosed  area  undergoes  a 
process  of  solution,  and  as  this  proceeds,  new  capillary  loops  pass  in 
from  the  periphery,  and  we  have  the  familiar  picture  of  the  development 
of  granulation  tissue  and  of  subsequent  cicatrization  of  the  same.     It  is 
a  frequent  experience  to  find  the  depressed  cicatrice  of  old  infarcts  in 
the  kidney  and  in  the  spleen. 

3.  Cyst  Formation. — In    the   brain   more   particularly,   in   place   of 
organization,  cyst  formation  may  show  itself  (see  vol.  i,  p.  795). 

4.  Putrefaction. — Where  pyogenic  organisms  are  in  the  affected  area, 
or  where  the  embolus  causing  the  infarct  is  infective,  there,  according 

1  Beattie  and  Dixon,  A  Text-book  of  Pathology,"  Lippincott,  1908. 


MORTIFICATION  45 

to  the  size  of  the  infarct,  we  may  encounter  either  a  putrefactive  softening 
of  the  infarct,  with  liquefaction  of  necrotic  area  through  the  proteolytic 
bacterial  enzymes,  and  without  the  formation  of  true  pus,  or — 

5.  Suppuration. — In    small    infarcts,    true    abscess    formation    with 
abundant   leukocytes   attracted   into    the   area   from   the    surrounding 
capillaries  may  be  developed. 

6.  Calcification. — The  conditions  favoring  calcification  of  a  necrosed 
area  have  been  discussed  (vol.  i,  p.  852). 

Mortification. — To  recapitulate:  where  (1)  there  is  inadequate  anas- 
tomosis, and  where  (2)  the  area  whose  blood  supply  is  cut  off  is 
extensive,  there,  in  place  of  coagulation  and  infarct  formation,  we 
encounter  mortification.  We  employ  this  term  rather  than  what  is 
usually  regarded  as  its  synonym,  gangrene,  because,  to  most  minds,  the 
latter  carries  with  it  the  implied  conception  of  putrefaction  and  decom- 
position of  bacterial  origin.  More  recent  research  shows  that,  at  least 
in  the  first  stages  of  cases  of  this  order,  there  is  an  aseptic  softening  of 
the  tissues  due  to  autolysis. 

We  feel  some  hesitation  in  drawing  this  sharp  distinction  between 
infarction  and  mortification.  It  may  well  be  that  in  the  majority  of 
cases  in  which  tissue  death  occurs  from  the  cutting  off  of  the  blood  supply 
of  a  part  there  is  at  least  a  preliminary  stage  of  rigor  and  coagulation 
necrosis.  But  if  so,  this  may  be  imperfect,  involving  certain  tissue  con- 
stituents more  than  others,  and  passing  off  with  relative  rapidity  as 
autolytic  processes  manifest  themselves.  We  approach  here  a  problem 
that  has  not  yet  to  our  knowledge  been  made  the  subject  of  investiga- 
tion, namely,  to  what  extent,  if  any,  does  the  development  of  coagulation 
necrosis  proper  inhibit  autolysis  within  the  living  body.  It  is  not  a  little 
striking  that  in  organs  such  as  the  kidney,  which  out  of  the  body  exhibit 
rapid  autolysis,  we  encounter  white  infarcts  which  evidently  are  many 
days  old,  but  which  have  remained  firm  with  no  signs  of  autolytic  soft- 
ening, or,  at  most,  what  has  been  termed  heterolytic  softening  at  the 
periphery  through  the  agency  of  invading  leukocytes;  whereas,  in  other 
organs,  in  which  infarct  formation  is  not  so  noticeable,  softening  may 
ensue  with  relative  rapidity.  Our  knowledge  of  the  whole  subject,  in 
fact,  of  coagulation  of  the  blood  and  of  the  tissues  is  in  so  chaotic  a  condi- 
tion that  it  is  impossible  to  lay  down  with  any  clearness  why,  when  small 
areas  are  involved,  we  are  more  likely  to  get  infarct  formation;  when 
larger  areas,  the  process  of  mortification  with  softening.  We  see  ob- 
scurely that  for  coagulation  necrosis  to  ensue,  there  must  be  some 
liberation  of  kinase  from  the  dying  cells,  which,  interacting  with  bodies 
afforded  by  the  surrounding  lymph,  favors  the  formation  of  a  thrombine 
or  ferment,  which  in  its  turn  leads  bodies  of  the  nature  of  fibrinogen, 
both  intracellular  and  extracellular,  to  undergo  solidification,  or  coagula- 
tion. We  cannot  lay  down  with  any  clearness  what  it  is  that,  for 
example,  brings  it  about  that  where  a  small  branch  of  the  pulmonary 
artery  becomes  blocked,  an  infarct  is  produced;  where  a  large  branch, 
suc-h  as  that  supplying  a  whole  lobe  of  the  lung,  no  infarction  ensues, 
but  only  mortification.  Presumably  the  capillary  pressure  in  the  two 


46 


VENOUS  OCCLUSION 


FIG.  6 


cases  and  the  extent  of  exudation  play  a  part — but  this  is  only  a  pre- 
sumption. 

Gangrene.  — Under  conditions  in  which,  either  from  coincident  closure 
of  the  various  anastomosing  arteries  of  a  relatively  large  area,  or  absence 
of  anastomosing  arteries  in  the  same,  it  may  happen  that  little  blood  passes 

into  the  dead  area,  and  in  that  case  in 
parts  that  are  exposed  and  subject  to 
evaporation,  there  is  gradual  desic- 
cation and  the  development  of  dry 
gangrene.  Where  bacteria  gain  en- 
trance, putrefaction  ensues,  and  thus 
a  form  of  moist  gangrene — a  form, 
it  may  be  added,  not  so  extreme  as 
regards  distension  of  the  vessels,  exu- 
dation, and  hemorrhagic  infiltration, 
as  is  seen  where  the  efferent  veins  are 
blocked,  the  arteries  still  conveying 
blood  to  the  part  (see  further  vol.  i, 
p.  908). 


Gangrene  of  the  foot.     (Montreal  General 
Hospital.) 


VENOUS  OCCLUSION. 

The  causes  of  closure  of  veins  are 
of  the  same  order  as  those  of  arteries, 
although  certain  exceptions  are  to 
be  noted.  Thus,  neurotic  or  spastic 
occlusion  is  unknown;  the  condition 
also  of  endophlebitis  obliterans,  or 
proliferative  overgrowth  of  the  intima 
sufficient  to  induce  obliteration  is 
almost  unknown ;  at  most,  rare  cases 
have  been  reported.1  So  also  in 

regard  to  the  hematogenous  occlusion,  while  thrombosis  is  common, 
embolism,  from  the  nature  of  the  case,  is  singularly  rare,  and  can  only 
be  due  to  a  retrograde  passage  or  falling  of  foreign  matter  into  a  vein. 
This  retrograde  embolism  will  be  discussed  later.  And  obviously,  by 
itself  this  can  scarce  induce  complete  occlusion;  by  inducing  a  surround- 
ing thrombosis  or,  where  of  the  nature  of  liberated  portions  of  new- 
growth,  by  subsequent  proliferation,  it  may  bring  about  not  an  immediate, 
but  a  gradual  complete  closure. 

Just  as  the  primary  effect  of  closure  of  an  artery  is  to  produce  local 
anemia  of  the  region  of  supply,  the  extent  of  the  anemia  depending 
upon  the  width  of  anastomosing  vessels,  so  the  primary  effect  of  venous 
occlusion  is  the  production  of  congestion,  the  corresponding  artery  or 
arteries  pouring  blood  into  the  part  which  must  become  heaped  up  in  the 


1  See  Meigs,  Jour,  of  Anat.  and  Physiol.,  34: 1900:458 


VENOUS  OCCLUSION  47 

area,  unless  there  be  adequate  anastomoses.  In  general,  venous  anasto- 
moses are  more  abundant  and  larger  than  are  arterial,  and  thus,  in  general, 
where  we  deal  with  the  occlusion  of  a  single  venous  branch  in  an  organ 
the  effects  are  little  noticeable.  It  is  thus  more  especially  when  all  the 
veins  coming  from  a  part  become  occluded  (as  in  the  case  of  an  incar- 
cerated hernia),  or  where  the  occlusion  affects  the  main  vein  coming  from 
an  organ  after  it  has  received  all  its  subsidiary  branches,  that  serious 
results  ensue  (as,  for  example,  when  the  common  iliac,  the  main  portal 
trunk,  or  a  main  renal  vein  becomes  blocked  by  a  thrombus).  Even 
in  these  cases  the  results  are  apt  to  be  temporary  rather  than  permanent. 
Anastomosing  vessels  of  minute  size  undergo  a  progressive  dilatation,  and 
an  abundant  collateral  circulation  becomes  developed.  How  abundant 
may  be  the  paths  of  such  collateral  circulation  may  be  indicated  from 
a  study  of  those  cases  in  which  there  is  obstruction  to  the  main  portal 
vein,  such  as  occurs  in  cirrhosis  of  the  liver.  To  those  various  collateral 
channels  we  have  already  referred  (Chapter  I). 

It  is  noticeable  that,  despite  these  abundant  anastomoses,  it  may  be 
long  before  the  collateral  circulation  is  adequate.  Thus,  when  the 
femoral  vein  becomes  thrombosed,  it  may  be  long  months  before  the 
congestion  and  oedema  of  a  lower  extremity  completely  disappear, 
and  longer  months  or  even  years  before  any  increased  exercise  with 
increased  pouring  of  arterial  blood  into  the  part  is  not  followed  by 
indications  that  the  circulation  is  still  imperfect  and  only  able  to  do 
little  more  than  deal  with  the  usual  amount  of  blood — evidence  in  the 
shape  of  swelling  of  the  limb  and  muscular  exhaustion.  And  in  portal 
cirrhosis  of  the  liver,  even  after  providing  additional  anastomoses  by 
causing  adhesions  between  the  omentum  and  the  liver  and  the  anterior 
abdominal  wall,  the  congestion  and  accompanying  ascites  are  still  apt 
to  continue,  despite  all  the  paths  present  or  provided  for  the  drawing 
away  of  the  portal  blood. 

Where  the  main  venous  trunk  coming  from  an  isolated,  encapsulated 
organ  is  suddenly  blocked,  there  the  opportunity  for  the  carriage  of 
the  venous  blood  is  at  a  minimum.  The  organ  becomes  hugely  swollen 
and  tense;  there  is  complete  stasis  of  the  blood  within  it;  interstitial 
hemorrhages  occur,  and  the  organ  undergoes  either  infarction  or  morti- 
fication. We  have  already  indicated  (p.  41),  and  here  repeat,  that 
hemorrhagic  infarction  may  result  from  venous  obstruction.  Such 
mortification  may  give  place  to  gangrene  in  those  cases  in  which  oppor- 
tunity is  present  for  the  entrance  and  growth  of  bacteria,  as  in  a 
strangulated  hernia  of  the  intestine  or  thrombosis  of  the  veins  of  the  leg. 

Hence  to  recapitulate,  the  results  of  occlusion  of  a  vein  may  be : 

1.  Practically  nil,  when  the  vein  is  small  and  one  of  a  series  of  abun- 
dantly anastomosing  vessels. 

2.  Intense  passive  congestion,  with  exudation  of  fluid  from  the  vessels, 
cedema,  etc.,  and  gradual  development  of  a  collateral  circulation,  the 
congestion  and  dropsy  disappearing  as  the  collateral  circulation  becomes 
more  and  more  adequate. 

3.  Intense  passive  congestion  with  exudation,  etc.,  giving  place  to 


48  EMBOLISM 

stasis  and  hemorrhages  in  those  cases  in  which  a  collateral  circulation 
cannot  be  developed  with  sufficient  rapidity.     This  stasis  results  in : 

4.  Hemorrhagic  infarction. 

5.  Mortification. 

6.  Gangrene. 

EMBOLISM. 

Intimately  associated  with  the  subject  of  vascular  closure  is  that  of 
embolism.  Literally,  an  embolus  is  something  "thrown  in;"  actually, 
it  is  any  body  which,  carried  along  in  the  blood  stream,  reaches  some 
point  at  which  the  narrowing  of  the  vessel  causes  its  arrest,  so  that  it 
either  wholly  or  partially  blocks  the  vessel.  We  are  apt  to  conceive  the 
embolus  as  always  completely  blocking  a  vessel.  This  is  not  essential; 
the  shape  of  many  solid  foreign  particles  must  be  such  that  they  permit 
some  passage  of  blood  around  them.  But  undoubtedly  their  very 
presence  favors  a  secondary  deposit  of  thrombus  material  upon  them, 
so  that  sooner  or  later  the  obstruction  becomes  complete.  According 
to  the  size  and  the  source  of  the  foreign  body  forming  the  embolus,  so 
may  we  encounter  them  in  all  parts  of  the  circulating  system — in  the 
heart,  aorta  and  arteries,  capillaries,  and  veins. 

Cardiac  Embolism. — Cardiac  embolism  is  very  rare;  occasionally 
a  long  thrombus  of  the  femoral  or  iliac  veins  may  become  detached 
and  be  carried  to  the  right  heart,  and  there,  becoming  rolled  upon  itself, 
may  form  a  plug  sufficiently  large  to  become  arrested  in  the  conus 
arteriosus,  blocking  the  entrance  to  the  pulmonary  artery.  We  have 
encountered  one  such  case.  The  ball  thrombus  of  the  left  auricle 
referred  to  later  (p.  70)  is  not  strictly  an  embolus,  i.  e.,  it  does  not 
become  firmly  lodged  in  the  stenosed  mitral  orifice;  there  are,  however, 
one  or  two  cases  on  record  in  which  large,  irregular,  softened  thrombi 
from  the  left  auricle  have  become  loosened  and  wedged  in  the  stenosed 
mitral  orifice,  and  thus  have  acted  as  true  emboli.  It  is  scarcely  neces- 
sary to  state  that  the  result  in  these  cases  is  sudden  death. 

Aortic  embolism  is  also  uncommon.  While  the  aorta  becomes  very 
perceptibly  narrowed  in  its  lower  portion  after  the  renal  arteries  have 
been  given  off,  it  is  rare  to  have  a  detached  thrombus  from  the  left 
heart,  or  of  parietal  nature  from  the  upper  part  of  the  aorta,  so  large  that 
it  blocks  this  portion  of  the  vessel.  Along  with  others,  we  have,  however, 
encountered  a  "riding  embolus"  poised  upon  the  sharp  angle  of  bifur- 
cation of  the  common  iliacs,  which,  itself  loose  and  easily  detached,  had 
led  to  an  obliterating  thrombus  filling  the  last  portion  of  the  aorta  and 
there  adherent. 

I  Arterial  embolism  is  the  most  common  condition.  As  Professor  Welch 
points  out,  it  is  difficult,  if  not  impossible,  to  state  with  any  degree  of 
accuracy  the  frequency  with  which  the  different  arteries  are  involved, 
and  that  because  by  no  means  all  emboli  give  rise  to  symptoms,  and 
as  a  result  they  are  not  sought  after  at  autopsy.  As  indicated  in  the  pre- 
vious chapter  unless  the  embolism  occurs  in  a  functional  end  artery, 


CARDIAC  EMBOLISM  49 

the  results  are  apt  to  be  negligible.  But  observers  are  generally  agreed 
that  the  most  common  site  is  in  branches  of  the  pulmonary  artery,  and 
the  most  common  cause  there  is  detachment  of  thrombus  matter  from 
one  of  the  systemic  veins  or  from  the  right  heart.  Sometimes — and  this 
is  most  common  during  the  puerperium — the  detached  thrombus  liber- 
ated from  some  of  the  pelvic  vessels  is  of  such  size  that  folded  upon 
itself  it  fills  the  main  pulmonary  artery  of  one  or  other  side  at  the  point 
where  it  divides  into  its  main  branches.  Here  again  the  result  is  sudden 
death.  Where  it  is  of  smaller  size  there  may  be  recovery  after  a  period 
of  intense  respiratory  distress  and  dyspnoea.  It  is  possible,  further,  that 
a  parietal  thrombus  in  the  proximal  portion  of  a  pulmonary  artery  may 
become  detached  and  act  as  embolus.  Next  most  frequent,  Welch  is  in- 
clined to  believe,  on  general  principles,  are  emboli  lodging  in  the  arteries 
of  the  lower  extremities,  although  these  are  apt  to  afford  no  symptoms; 
a  detached  cardiac  thrombus  of  small  size,  or  loosened  cardiac  vegeta- 
tions, is  more  apt  to  be  carried  in  the  centre  of  the  arterial  blood  stream 
— is  more  likely  thus  to  be  conveyed  along  the  axial  current  into  the 
common  iliac  of  either  side  and  its  more  direct  continuation,  the  external 
iliac,  than  to  be  directed  into  a  side  branch  of  the  main  trunk.  Never- 
theless, it  is  in  these  side  branches  that  the  effects  of  embolism  are  most 
marked;  they  are  most  frequently  detected,  therefore,  in  the  renal,  splenic, 
cerebral,  iliac  and  other  arteries  of  the  lower  extremities,  axillary, 
and  arteries  of  the  upper  extremity,  cceliac  axis  with  its  hepatic  and 
gastric  branches,  central  artery  of  the  retina,  superior  mesenteric,  in- 
ferior mesenteric,  abdominal  aorta,  and  coronary  arteries  of  the  heart. 
This,  according  to  Welch,  is  the  order  of  relative  frequency,  of  emboli, 
that  is,  leading  to  observable  symptoms  and  gross  disturbances.  These 
arterial  emboli  are,  in  the  main,  portions  of  detached  cardiac  thrombi 
from  the  left  side  of  the  heart  or  from  the  heart  valves  (vegetations), 
more  rarely  portions  of  parietal  aortic  thrombi  or  calcareous  and  necrotic 
matter  from  aortic  atheromatous  ulcers.  It  is  possible  also  that  detached 
thrombi  from  the  pulmonary  veins  give  occasional  origin  to  emboli. 
Some  few  cases  are  on  record  of  what  is  known  as  paradoxical  or  crossed' 
embolism,  in  which  matter  originating  in  the  systemic  venous  system 
or  right  auricle  has  been  found  plugging  the  systemic  arteries.  In  such 
cases  there  is  a  relatively  large  patent  foramen  ovale.  Patency  of 
this  communication  between  the  two  auricles  is  very  common;  in  almost 
one  out  of  every  three  hearts  some  communication  exists,  but  usually 
very  small,  oblique,  and  valve-like,  so  that  increased  pressure  in  one  or 
other  auricle  only  serves  to  close  the  passage.  In  a  certain  number  of 
cases  the  passage  is  wider  and  direct,  and  in  these  any  increase  in  the 
right  auricular  blood  pressure  over  .that  of  the  left  auricle  must  be 
accompanied  by  flow  of  blood  from  the  right  into  the  left  heart.  As 
a  matter  of  fact  thrombotic  material  has  been  found  forming  an  arterial 
embolus  in  the  foramen  ovale;  one  such  case  was  encountered  by  Dr. 
John  McCrae  in  our  postmortem  service  at  the  Royal  Victoria  Hospital. 
Yet  another  form  of  crossed  embolism  can  occur,  though  here  the  em- 
bolic  masses  are  very  minute.  The  capillaries  of  the  lungs  are  relatively 
4 


50 


EMBOLISM 


large  and  distensible,  so  large  that  it  is  possible  for  particles  the  size 
of  tissue  cells  to  pass  through  certain  of  these  without  being  arrested. 
Thus,  we  have  scraped  the  cut  surface  of  a  freshly  excised  rabbit's  liver, 
made  an  emulsion  of  the  scrapings,  injected  this  into  a  systemic  vein  of 
another  rabbit,  and  killing  this  after  a  few  minutes,  and  making  sections 
of  the  different  organs,  have  encountered  isolated  liver  cells  and  minute 


Patent  F,  Ovalis 
S.V.C. 


Srhema  of  crossed  embolism,  to  indicate  the  passage  of  thrombotic  material  from  the  veins  to 
form  emboli  in  the  systemic  arteries  by  passage  through  the  fenestra  ovalis. 


masses  of  the  same  in  the  arterioles  of  the  kidney,  determining  at  the 
same  time  that  the  foramen  ovale  was  closed,  or,  more  accurately,  was 
non-existent. 

Capillary  Emboli. — Such  minute  masses,  it  is  clear,  are  too  small 
to  be  arrested  in  the  arteries;  at  most,  they  can  block  the  capillaries, 
and  doing  this,  so  abundant  are  the  capillary  anastomoses,  that  in  the 
majority  of  instances  they  must  cause  no  recognizable  disturbance. 


VENOUS  EMBOLI  51 

Only  when  (1)  these  capillary  emboli  are  extraordinarily  abundant,  or 
(2)  have  the  capacity  to  propagate  themselves,  do  they  become  mani- 
fest. It  has  been  noticed,  in  the  first  place,  that  the  pigmented  remains 
of  malariaL-pajasites  after  sporulation  are  peculiarly  apt  to  be  arrested 
and  accumulate  in  the  fine  capillaries  of  the  brain  and  kidney,  so  that 
large  numbers  of  the  vessels  become  blocked,  resulting  in  functional 
disturbance  of  these  organs;  in  the  second  place,  we  encounter  both 
microbic  and  neoplastic  cell  emboli.  The  former,  by  their  continued 
growth,  set  up  an  inflammatory  reaction,  and  thus  induce  the  formation 
of  multiple  metastatic  abscesses,  hematogenous  miliary  tubercles,  etc.; 
the  latter  form  the  centres  for  the  development  of  metastatic  new-growths. 
We  are  doubtful  whether  primarily  the  majority  of  so-called  microbic 
emboli  should  strictly  be  regarded  as  such.  When  an  infected  thrombus 
breaks  down,  then  undoubtedly  the  particles  of  softened  matter  form  true 
capillary  emboli ;  they  block  the  small  vessels,  and  the  contained  bacteria 
continue  to  grow  until  they  form  a  dense  mass  filling  and  extending  along 
the  capillary;  but  in  many  cases  of  pyemia,  what  we  deal  with  is  the 
transport  of  individual  microbes  which,  becoming  taken  up  by  the  capil- 
lary endothelium  without  any  circulatory  arrest,  multiply  within  the 
endothelial  cells,  and  only  after  distending  and  breaking  these  down 
grow  into  and  along  the  lumen,  obliterating  the  capillary.  The  chain  of 
events  is  more  akin  to  capillary  thrombosis  than  to  embolism,  or  other- 
wise, the  embolism  is  secondary,  not  primary. 

Venous  Emboli. — We  have  to  distinguish  two  orders  of  venous 
embolism:  that  occurring  in  the  portal  vessels  of  the  liver,  and  that  I 
affecting  the  systemic  veins  proper.  The  portal  vein  in  its  branching 
and  division  into  smaller  and  smaller  vessels  is  strictly  comparable  with 
an  artery,  and  the  general  principles  governing  arterial  embolism  govern 
it  also.  There  may  be  extensive  embolism  in  the  liver  through  the  liber- 
ation of  thrombi  from  the  splenic,  the  mesenteric,  and  other  contributory 
veins  of  the  portal  system;  the  forms  most  frequently  encountered 
are  infective  and  multiple,  secondary  to  suppurative  thrombophlebitis, 
this  in  itself  most  frequently  secondary  to  acute  appendicitis.  The 
liberation  of  the  broken-down,  infectious  material  may  induce  both 
venous  and  capillary  emboli.  Somewhat  less  frequently  we  encounter 
malignant  emboli  due  to  extension  of  malignant  disease  from  the  stomach, 
pancreas,  and  other  abdominal  organs  into  branches  of  the  portal  system, 
and  detachment  either  of  individual  cells,  or  cell  masses. 

In  the  ordinary  veins  which  collect  into  larger  and  larger  trunks,  it 
is  immediately  evident  that  masses  originating  in  and  becoming  detached 
from  a  smaller  trunk  cannot,  under  normal  conditions,  form  an  embolus, 
the  lumen  of  the  vessels  through  which  it  passes  becoming  progressively 
larger.  The  only  possible  conditions  under  which  embolism  can  occur 
are  when  either  the  current  of  the  venous  blood  becomes  reversed,  so 
that  foreign  bodies  are  carried  by  the  stream  into  smaller  and  smaller 
veins  until  they  become  arrested,  or  when  the  very  weight  of  the  foreign 
body  is  such  that  under  the  action  of  gravity  it  falls  against  the  blood 
stream  into  some  more  dependent  vessel.  It  may  be  thought  that 


52 


EMBOLISM 


FIG.  8 


I.V.C.. 


these  are  scarcely  possible  conditions;  that  if  momentarily  the  blood 
current  were  reversed  or  became  slow  enough  for  gravity  to  exert  its 
effects  upon  contained  larger  masses,  with  the  arrest  of  a  mass  at  a  point 
where  two  or  more  smaller  veins  joined  to  form  a  larger  one,  within 
a  very  short  time  the  pressure  of  the  venous  blood  behind  the  obstruction 
would  liberate  it  and  carry  it  forward  toward  the  heart.  But,  as  a  matter 
of  fact,  several  cases  are  on  record  which  can  only  be  explained  as 

examples  of  the  retrograde  em- 
bolism. Only  those  examples 
can  be  taken  as  indubitable  in 
which  all  possibility  of  crossed 
embolism  is  excluded;  we  can 
deal,  therefore,  only  with  cases 
in  which  the  embolism  is  too 
large  to  have  passed  through 
the  pulmonary  capillaries,  and 
in  which  there  has  been  no 
patent  foramen  ovale.  But 
when,  to  quote  Lubarsch's 
case,  there  is  encountered  a 
primary  ossifying  chondrosar- 
coma  of  the  right  tibia,  with 
a  sarcomatous  mass  forming 
a  riding  embolus  of  the  hepatic 
vein,  and  no  other  secondary 
growths  in  the  liver;  or,  to 
instance  one  of  von  Reckling- 
hausen's  cases,  where  along 
with  a  primary  myxochondro- 
sarcoma  of  the  tibia  invading 
the  veins  of  the  leg  there  was 
found  a  similar  myxochondro- 
sarcomatous  mass  in  the  main 
renal  vein  extending  by  growth 
along  the  branches  of  the 
same,  confined  to  this  vessel 
and  its  branches  with  no  other 
malignant  involvement  of  the 

Schema  of    retrograde   embolism,    showing   retro-  ,1  ,\  i  i 

grade  passage  of  thrombotic  material  to  form  emboli  °.rgan;  then  the  Om7  C?nclu- 
in  the  hepatic  and  renal  veins  respectively.  sion  HlUSt  be  that  there  has 

been  retrograde  embolism. 

And,  as  a  matter  of  fact,  Arnold,  Lubarsch,  and  others  have  experi- 
mentally demonstrated  the  possibility  of  such  retrograde  embolism  by 
the  slow  injections  of  thick  emulsions  of  coarse  meal  into  the  neck 
veins,  etc.  More  particularly  when  death  occurred  with  severe  dyspnoea, 
it  was  found  that  the  coarse  grains  had  made  their  way  into  the  coronary 
veins  of  the  heart,  the  renal  and  hepatic  veins,  the  cerebral  sinuses,  etc. 
There  has  been  considerable  debate  as  to  the  exact  mode  by  which  the 


THE  CONSTITUTION  OF  EM  BO  LI  53 

particles  make  their  way  against  the  normal  course  of  the  circulation, 
but  this  seems  to  be  evident  that  both  experimentally  and  in  the  cases  in 
man  so  far  recorded,  there  has  been  evidence  of  impeded  respiration. 
This  suggests  that,  as  laid  down  by  Heller  and  von  Recklinghausen, 
one  essential  for  the  retrograde  transport  is  a  temporary  or  continued 
removal  of  the  negative  intrathoracic  pressure — a  condition  favoring  the 
existence  of  positive  pressure  in  the  veins,  with  its  accompanying  obstruc- 
tion to  the  onward  flow  of  the  venous  blood,  damming  of  the  same  in 
the  large  veins,  and  development  of  reversed  current  in  the  venae  cavse 
at  each  contraction  of  the  right  heart.  Both  Arnold  and  Lubarsch  have 
directly  observed  the  regurgitation  of  foreign  bodies  from  the  inferior 
vena  cava  into  the  exposed  renal  vein  upon  the  onset  of  respiratory  con- 
vulsions in  animals  of  the  laboratory. 

Following  Ribbert's  observations,  we  are,  however,  inclined  to  presume 
that  the  force  with  which  foreign  particles  are  driven  back  into  the  veins 
cannot  be  very  great,  and  that  what  is  necessary  for  the  preservation  of 
the  foreign  body  in  situ  is  a  relatively  rapid  adhesion  of  the  embolus  by 
conglutination  or  the  production  of  fibrin. 

Lymphatic  Emboli. — It  is  appropriate  to  note  at  this  point  that 
what  applies  to  the  systemic  veins  applies  also  to  the  lymphatic  vessels.  ] 
In  them  also  retrograde  embolism  is  apt  to  occur,  apparently  with  fair 
frequency.  More  particularly  does  this  explain  the  development  of  certain 
of  the  malignant  metastases  in  the  lymphatic  system.  The  main  cause 
of  the  retrograde  current  would  seem  to  be  closure  of  the  main  lymphatic 
channels  of  a  part,  with,  as  a  consequence,  reversal  of  flow  in  the  branches, 
that  the  lymph  may  find  an  outlet  by  anastomosing  vessels  into  other 
lymph  channels  that  are  not  impeded.  Such  retrograde  embolism  is 
best  fitted  to  explain  the  appearance  of  cancerous  growth  in  the  head 
of  the  humerus  in  cases  of  mammary  cancer  with  involvement  of  the 
axillary  lymph  glands. 

The  Constitution  of  Emboli.— Any  body  which,  free  in  the  blood, 
may  be  carried  forward  until  it  blocks  a  vessel  is  capable  of  acting  as  an 
embolus.  Emboli  are  thus  of  very  varying  nature : 

1.  By  far  the  commonest  causes  of  embolism  are  detached  masses 
of  thrombotic  material,  and  this  being  so,  it  is  evident  that  when,  for 
example,  we  find  recent  cruor  thrombus  filling  a  vessel,  it  is  at  times 
impossible  to  determine  whether  we  deal  with  embolism  or  local  throm- 
bosis. So,  too,  when  the  condition  is  of  long  standing,  it  is  at  times 
scarce  possible  to  determine  whether  the  whole  occluding  mass  is  throm- 
botic, or  whether  there  has  been  a  primary  embolus  with  subsequent 
thrombosis  developing  upon  it.  In  general,  however,  a  difference  in 
color  can  be  made  out  between  the  primary  mass  that  has  acted  as  an 
embolus,  and  the  secondary  thrombus  that  has  formed  upon  it;  so  also 
the  embolus  can  be  detached  from  the  vessel  wall  and  the  secondary 
thrombus,  and  at  times  it  is  possible  to  straighten  out  the  coiled-up 
embolic  mass  and  detect  the  surface  of  detachment  from  the  thrombus 
of  origin,  as  also  that  thrombus  of  origin  may  be  discovered  elsewhere 
in  the  vascular  system,  and  its  broken  surface  mav  be  seen  and  com- 


54  EMBOLISM 

pared.  It  must  be  again  recalled  that  detached  vegetations  from  the 
cardiac  valves  form  an  important  series  of  emboli  of  this  order.  Of 
other  bodies  found  less  frequently  constituting  emboli  we  may  make  a 
division  into  the  endogenous  and  the  exogenous,  or  otherwise  into  sub- 
stances originating  in  and  from  the  tissues  and  those  of  extraneous 
origin.  Of  the  former  we  may  have: 

2.  Calcareous  and  atheromatous  matter  from  atheromatous  ulcers. 

3.  Tumor  masses  and  cells  detached  from  new-growths  which  have 
penetrated  into  the  vessels;  these,  forming  emboli,  may  continue  to  pro- 
liferate, thus  giving  rise  to  secondary  or  metastatic  new-growths. 

4.  Detached  tissue  cells  or  collections  of  the  same.     Of  such,  more 
particularly,    there    have   been   encountered    placental   cell    (Schmorl) 
and  liver  cell  emboli  (Turner).     The  natural  growth  of  the  chorionic 
villi  of  the  foetus  is  into  the  uterine  blood  sinuses;  conditions  thus  favor 
the  detachment  of  certain  of  the  cells  or  villous  processes,  which  then 
may  form  pulmonary  or  other  emboli.     Ordinarily  such  cells  undergo 
disintegration;  rarely  they  proliferate,  and  so  give  rise  to  the  chorio- 
epithelium  malignum  (vol.  i,  p.  610).     The  size  of  the  liver,  its  abundant 
and  large  vessels,  and  the  intimate  relationship  of  the  liver  cells  to  the 
vessels,  favor  local  dislocation  of  liver  cells  into  the  blood  stream  following 
blows  upon  the  liver  region,  or  disease  in  which  congestion  of  the  organ 
and  degenerative  disturbances  of  its  cells  are  combined,  as  in  eclampsia. 
The  liver  cells  may  be  found  in  the  heart,  pulmonary  vessels,  and  even 
in  those  of  the  systemic  arterial  system.     They  are  to  be  recognized  by 
their  shape,  the  size  of  the  nucleus,  and  the  diagnosis  becomes  con- 
vincing in  those  cases  in  which  the  cells  contain  bile  .pigment.     A  third 
type  of  cell  is  occasionally  encountered  in  the  small  vessels  of  the  lung, 
namely,  the  giant  cells  or  megacaryocytes  of  the  bone  marrow  (Aschoff), 
often  in  a  state  of  characteristic  degeneration,  with  fusion  of  the  multiple 
nuclei  into  a  single,  large,  irregular  mass.     The  observations  of  Aschoff, 
Fox,   Lubarsch,   and   Langemann   throw  light  upon  this   unexpected 
process;  wherever  there  is  produced,  either  naturally  or  experimentally, 
a  condition  of  pronounced  leukocytosis,  with  pouring  of  leukocytes  out 
of  blood  marrow,  there   along  with  the  leukocytes  a  certain  number 
of  these  giant  cells  become  liberated  into  the  delicate  capillaries  with 
which  they  are  in  intimate  association,  and,  passing  thus  into  the  circu- 
lation, become  arrested  in  the  first  series  of  other  capillaries  into  which 
they  become  carried.     Very  rarely,  and  in  cases  of  either  traumatic  or 
operative  injury  of  bone,  osteoclasts  and  fat  cells  from  the  marrow  have 
been  noted  in  the  lung  capillaries. 

5.  Leukocytes. — In  cases  of  myeloid  leukemia,  the  capillaries  of  the 
liver,  kidneys,  and  other  organs  may  be  found  so  densely  packed  with 
leukocytes  that  the  condition  must  be  regarded  as  embolic. 

6.  Fat  Embolism. — Fat  may  be  present  in  the  circulating  blood  in 
one  of  two  forms,  either  ^  fine  emulsion,  as  in  lipemia,  or  of  coarse 
droplets,  as  after  rupture  of  fat  cells  and  discharge  of  their  contents 
either  directly  into  the  bloodvessels  or  into  lymph  channels  or  spaces. 
There  is  doubt  whether  the  first  of  these  conditions  induces  true  capillary 


FAT  EMBOLISM  55 

embolism.  It  is  true  that  in  those  dead  of  diabetic  coma  solid  masses  of 
fatty  matter  have  not  been  infrequently  observed  in  the  lung  capillaries, 
forming  moulds  of  the  same,  but  (1)  these  have  not  been  detected  in 
autopsies  performed  within  two  or  three  hours  after  death;  (2)  they 
are  apt  to  have  a  granular,  non-homogeneous  appearance,  as  though 
formed  from  the  imperfect  fusion  of  minute  droplets;  and  (3)  there  is 
a  characteristic  absence  of  surrounding  hemorrhage  or  infiltration.  All 
these  signs  indicate  a  postmortem  accumulation  or  "  creaming"  of  the 
fine  fatty  particles  rather  than  an  antemortem  fusion  into  large  drops 
capable  of  acting  as  emboli. 

There  is  now  abundant  evidence  that  these  large  drops  or  masses  of 
fat,  liberated  from  fat-containing  cells,  cause  capillary  embolism  in  the 
lungs  with  fair  frequency,  and  in  more  extreme  cases  plug  the  capillaries 
of  the  heart,  the  kidney  (here  more  particularly  the  glomerular  loops), 
the  brain,  and  other  organs,  setting  up  severe  and  often  fatal  functional 
disturbance.  The  main  cause  would  seem  to  be  trauma,  accidental  or 
operative,  such  as  fracture  of  the  long  bones  with  accompanying  rupture 
of  the  fatty  marrow  cells,  forcible  breaking  down  of  immobilized  joints, 
section,  operative  handling,  and  ligature  through  a  large  panniculus 
adiposus,  operative  handling  of  the  fatty  omentum  and  mesenteries  or 
of  other  accumulations  of  fat  in  the  patient,  rupture  or  contusion  of  the 
fatty  liver.  When  there  is  no  actual  fracture  of  the  bones,  sudden 
extensive  concussion  of  the  bony  skeleton,  such  as  follows  falls  from 
some  considerable,  height,  has  been  observed  to  lead  to  fat  embolism  in 
the  lungs.  It  would  seem  that  here  the  fatty  cells  and  fine  capillaries  of 
the  bone  marrow  become  jarred  against  or  violently  torn  away  from  the 
more  rigid  bony  framework.  The  fat  embolism  that  has  been  noted 
after  epileptic  fits,  eclamptic  convulsions,  etc.,  would  seem  best  explained 
as  due  to  a  similar  liberation  from  the  bone  marrow  (Lubarsch).  With 
Ribbert,  then,  we  may  regard  violent  shaking  or  concussion  of  bones  as 
an  important  factor  in  producing  the  condition. 

An  obstruction  of  a  few  capillaries  in  the  lung  by  fatty  globules  leads 
to  no  obvious  disturbance,  or  at  most  minute  areas  of  congestion  and 
hemorrhage;  minute  infarcts  may  be  induced  of  no  serious  import. 
Occasionally  these  capillary  emboli  are  present  in  great  abundance, 
so  abundant  as  to  seriously  obstruct  the  circulation  through  the  lung, 
setting  up  grave  and  sometimes  fatal  dysgncea.  It  is  remarkable  that 
symptoms  of  disturbance  either  manifest  themselves  within  a  few  minutes 
to  six  hours  after  trauma,  or  only  after  four  or  more  days.  In  the  first 
case  we  see  the  direct  effect  of,  more  particularly,  pulmonary  embolism; 
in  the  second  we  are  inclined  to  the  view  that  through  sapohirTcafion  the 
original  fatty  plugs  in  the  capillaries  at  the  site  of  injury,  and  again  the 
emboli  in  the  lungs,  have  become  diminished  and  loosened.  The  con- 
tracted pupils,  convulsions,  and  Cheyne-Stokes  respiration  suggest  that 
now  the  fat  has  passed  to  the  left  heart  and  become  lodged  in  the  capil- 
laries of  the  brain.  For  the  fat  forming  these  emboli Jends^io- undergo 
absorption,  and  that  in  more  than  one  way.  Lipolytic  enzymes  exist 
in  the  blood,  and  gradually  the  droplets  become  saponified  and  dissolved. 


56  EMBOLISM 

The  fatty  state  of  the  endothelium  of  the  affected  capillaries  indicates 
that  these  cells  absorb  it  to  some  extent,  and  very  possibly  pass  it  on  to 
underlying  tissue_<cells,  for  these  also  may  stain  deeply  with  Sudan  III. 
Add  to  this  that  there  may  be  some  accumulation  of  leukocytes  around 
the  fatty  masses,  suggesting  a  phagocytJ£activity  on  the  part  of  these  cells. 
Emboli  due  to  extraneous  matter: 

7.  Air  Embolism. — Occasionally  during  the  course  of  operations  upon 
the  neck  a  suspicious  sucking  sound  is  heard,  due  to  the  entrance  of  air 
into  a  severed  vein.     If  the  vein  be  immediately  closed  before  any  large 
amount  of  air  has  been  inspired,  no  ill  results  may  ensue,  but  at  times 
death  occurs  with  absolute  suddenness,  at  others  it  is  preceded  by  extreme 
dyspnoea,  churning  action  of  the  heart,  cyanosis,  and  convulsions.     The 
nearer  the  heart  and  the  larger  the  vein,  the  greater  the  danger  of  this 
event,  but  cases  are  on  record  in  which  sudden  death  of  this  order  has 
followed  operation  upon  the  head,  upper  extremities,  and  uterus.     The 
condition  has  been  recognized  for  now  more  than  a  century,  although,  with 
Welch,  we  must  ascribe  many  of  the  earlier  cases  of  supposed  uterine 
and  intestinal  origin  not  to  air  embolism,  but  to  gas  production  by  the 
Bacillus  aerogenes  capsulatus  (Bacillus  Welchii)  or  other  gas-producing 
organisms. 

As  regards  the  cause  of  the  sudden  death,  there  are  two  main  theories : 
(1)  That  it  is  essentially  cardiac,  due  to  the  churning  action  in  the  right 
heart,  so  that  a  relatively  small  amount  of  air,  as  it  becomes  warmed  up, 
expands  to  make  a  very  considerable  foam,  which,  accumulating  behind 
the  tricuspid  valves,  effectively  arrests  their  activity;  (2)  that  the  essential 
cause  is  multiple  air  emboli  in  the  pulmonary  capillaries.  That  bubbles 
of  air  do  not  pass  through  the  lung  capillaries,  but  become  blocked 
there,  has  been  proved  by  experiments  on  dogs;  in  experimental  air 
embolism  the  left  heart  is  found  practically  free  from  air,  and  the  lungs 
show  multiple  hemorrhages,  suggesting  most  significantly  that  the  ob- 
struction of  a  large  number  of  capillaries  has  led  to  profound  congestion 
and  rupture  of  some.  The  more  recent  experiments  of  P.  Wolf1  are 
generally  accepted  as  establishing  this  second  theory,  although  the  wide 
difference  between  the  amount  of  air  that  can  experimentally  be  injected 
into  the  veins  of  a  healthy  dog  without  fatal  results  (under  200  c.c.), 
and  the  much  smaller  amounts  that  have  been  estimated  as  causing 
sudden  death  in  the  human  patient,2  suggest  that  the  right  heart  is  a 
factor;  that  a  vigorously  contracting  organ  may  be  able  to  propel  the  air 
out  into  and  through  the  lungs,  distributing  it  thus  to  parts  where  it 
may  become  absorbed. 

8.  Gas  Emboli. — Apparently  of  like  order  are  the  cases  recorded  by 
Janeway  and  Hun,  in  which  grave  cerebral  symptoms  followed  the  in- 
jection of  peroxide  of  hydrogen  into  the  abdominal  and  thoracic  cavities. 

A  more  common  cause  of  gas  embolism  may  lead  to  fatal  results  in 
divers  and  those  working  in  compressed  air;  such  gas  embolism,  in 

1  Virchow's  Archiv,  174: 1903. 

2  See  Greene,  Amer.  Jour.  Med.  Sci.,  1904. 


PLATE   I 


Fat-embolism  of  the    Kidney. 

The  globules  of  fat  are   impacted  within  a  glomerular  tuft  and   are  stained 
red    with   Sudan    III.      Reichert   obj.  No.  7,  without  ocular. 


(From  the  Pathological  Department,  Montreal  General  Hospital.) 


ANIMAL  PARASITES  57 

fact,  would  appear  to  be  the  essential  causative  agent  in  the  so-called 
caisson  disease.  Workers  in  compressed  air,  who  have  for  considerable 
periods  been  subjected  to  pressure  of  more  than  two  atmospheres,  if 
they  emerge  suddenly  into  the  ordinary  air,  are  liable  either  to  show 
symptoms  of  dyspnoea  and  asphyxia,  or  to  become  victims  of  a  series 
of  intractable  nervous  disturbances,  often  fatal  after  a  few  days  or 
weeks.  These  are  of  the  nature  of  various  paralyses,  hemiplegia, 
etc.  Examination  of  sections  of  the  spinal  cords  from  these  cases  shows 
the  presence  of  multiple  areas  of  necrosis  in  the  posterior  and  lateral 
columns,  without  hemorrhages,  but  with  ascending  and  descending  de- 
generations. Von  Schrotter  and  others  have  produced  and  studied  these 
lesions  in  dogs  and  other  animals  subjected  to  compressed  air.  The 
partial  pressure  of  the  gases  in  the  blood  is  dependent  upon  the  atmo- 
spheric pressure;  increase  this  last,  and  the  blood  passively  absorbs  air 
from  the  lungs;  the  greater  the  pressure,  the  more  the  amount  absorbed 
and  held  in  solution.  The  oxygen  of  that  air  becomes  fixed  by  the  tissues, 
which  also  passively  absorb  some  of  the  nitrogen.  If  the  atmospheric 
pressure  be  reduced  suddenly,  the  blood  now — and  the  tissues — can 
no  longer  hold  the  free  nitrogen  in  solution.  As  a  consequence,  the  gas 
separates  in  the  form  of  bubbles,  wrhich  grow  in  size  and,  carried  in  the 
blood  stream,  set  up  gas  emboli  in  the  various  tissues,  the  results  being 
most  serious  in  the  terminal  arteries  of  the  brain  and  spinal  cord,  leading 
to  anemia  and  necrosis  of  the  areas  of  supply. 

The  other  form  of  gas  embolism  we  have  already  indicated,  that, 
namely,  due  to  the  products  of  activity  of  gas-producing  organisms. 
While  in  the  majority  of  cases  the  emphysema  and  gas  in  the  vessels 
is  a  postmortem  development,  there  are  cases  in  which  it  is  recognizable 
during  life,  and  gaseous  embolism  may  be  claimed  as  a  cause  leading 
to  the  fatal  event. 

9.  Pigment  emboli  (see  p.  50). 

10.  Bacterial  emboli  (see  p.  50). 

11.  Animal    Parasites. — The    symptoms    of    sleeping    sickness    are 
ascribed  to  the  accumulation  of  trypanosomes  in  the  cerebral  capillaries, 
with  blocking  of  the  same,  although  it  has  to  be  admitted  that  the  gradual 
development  of  these  symptoms  scarce  suggests  true  embolism.     Never- 
theless, in  trypanosomiasis  the  capillaries  of  more  than  one  organ  have 
been  found  completely  blocked  by  dense  accumulations  of  the  parasites, 
herein  corresponding  with  what  has  occasionally  been  observed  with 
that  other  widespread  protozoan  parasite,   the  organism  of  malaria. 
Metazoan  parasites  may  also  induce  embolism,  notably  the  abundant 
larvae  of  various  strongyles.     The  classical  example  is  afforded  by  the 
Strongylus  armatus  of  the  horse,  whose  larvse,  becoming  arrested  in 
branches  of  the  abdominal  aorta  and  other  vessels,  may  with  their  growth 
and  the  irritation  thereby  induced  cause  thinning  and  giving  way  of  the 
arterial  wall,  with  the  production  of  verminous  aneurisms.     Rare  cases 
are  also  on  record  of  the  rupture  of  echinococcus  cysts  into  a  vein,  with, 
as  a  result,  embolism  of  the  vessels  of  the  lung  by  means  of  the  daughter 
cysts  or  their  membranes. 


58  THROMBOSIS 

12.  Projectiles. — Some  three  undoubted  cases  are  on  record  in  which 
bullets  entering  the  heart  or  larger  vessels  have  been  carried  in  the  blood 
stream  until  they  have  blocked  and  become  fixed  in  some  smaller  artery. 

The  Results  of  Embolism. — These  have  already  been  discussed  in 
the  chapter  upon  the  effects  of  arterial  closure  and  infarct  function, 
and  incidentally  in  the  pages  here  preceding.  One  aspect  of  the  sub- 
ject was  not  touched  upon,  namely,  the  effects  of  embolism  upon  the 
vessel  wall.  Briefly,  when  embolism  does  not  cause  sudden  death^- 
and  is  not  associated  with  rapidly  fatal  disease,  the  embolus,  acting  as 
a  foreign  body,  produces  a-local  irritation  of  the  vessel  walls  with  signs 
of  inflammation,  of  the  chronic  or  of  the  acute  type,  according  as  to 
whether  the  embolus  is  of  the  bland  or  of  the  infectious  type.  There 
may  thus  at  the  one  extreme  BeTdeveloped  a  chronic  thickening  of  the 
wall  with  accompanying  organization  of  the  embolus  and  permanent 
obliteration  of  the  vessel,  or  in  the  other  an  embolic  abscess.  A  less 
severe  termination,  also  due  to  infection,  is  the  production  of  the  embolic 
mycoikL_aneurism;  softening  of  the  arterial  wall,  more  particularly  in 
arteries  not  well  supplied  by  surrounding  tissue,  such  as  the  cerebral, 
and  branches  of  the  mesenteric  vessels,  may  lead  to  giving  way  of  the 
same,  with__ajieiirism  production.  A  form  of  embolic  aneurism  of 
the  second  degree  may  be  brought  about  by  embolic  abscesses  of  the 
vasa  vasorum  of  the  aorta  and  large  arteries,  with  weakening  of  the  wall 
of  the  main  artery  and  formation  of  a  saccular  pouch  upon  it. 


THROMBOSIS. 

When  performing  autopsies,  it  is  a  familiar  experience  to  encounter 
clotted  blood,  either  in  the  cavities  of  the  heart  or  in  the  vessels,  veins, 
or  arteries,  and  immediately  it  becomes  essential  to  determine  whether 
the  clotting  has  occurred  before  or  after  death,  whether,  that  is,  we  deal 
with  a  condition  which  must  have  very  materially  affected  the  onward 
flow  of  the  blood,  and  have  been  a  factor  in  the  production  of  symptoms 
of  various  orders,  or  whether  with  one  which  at  most  throws  light  upon 
the  amount  of  coagulable  material  present  in  the  blood.  The  latter 
condition  differs  in  no  important  feature  from  the  coagulation  in  blood 
escaping  from  the  vessels  during  life;  the  former  presents  many  departures, 
both  in  mode  of  development  and  arrangement  of  constituent  elements. 
To  it  we  give  the  name  of  thrombosis.  To  be  exact,  we  should  never 
speak  of  a  "p.  m.  thrombus;"  that  expression  involves  a  contradiction 
in  terms. 

A  thrombus,  therefore,  is  a  solid  body  or  mass  situated  in  the  cavity 
of  the  heart  or  vessels,  formed  during  life  of  constituents  derived  from 
the  blood.  Some  authorities  would  add  to  this  definition,  "formed  in 
situ."  This  we  regard  as  a  non-essential.  The  product  of  intravital 
and  intra vascular  clotting  of  the  blood  is  no  less  the  result  of  thrombosis, 
and  still  remains  a  thrombus,  even  if  detached  and  carried  to  a  distance. 
Here  at  the  most  it  is  necessary  to  distinguish  clearly  between  a  thrombus 


POSTMORTEM  CLOTTING  59 

and  an  embolus.  An  embolus  is  any  free  matter  which,  conveyed  along 
the  blood  stream,  becomes  arrested  at  some  point  where  the  diminished 
diameter  of  a  vessel  becomes  less  than  its  own  diameter.  Anything 
which  is  capable  of  plugging  a  vessel  constitutes  an  embolus;  in  simple 
English,  it  is  a  plug.  A  free,  solid  mass  of  clotted  blood  may  thus  be 
carried  along  a  vessel  until  it  blocks  it,  and  so  acts  as  an  embolus.  A 
thrombus  may  thus  form  one  order  of  embolus,  and  that  a  very  frequent 
one,  but  by  no  means  all  emboli  are  thrombi.  What,  however,  is  of 
importance  is  that  thrombosis  is,  as  we  shall  point  out  later,  associated 
always  with  local  disturbance  of  the  vascular  wall;  it  is  doubtful  whether 
a  thrombus  ever  originates  as  a  process  of  free  precipitation  out  of  the 
circulating  blood;  it  develops  in  connection  with  an  endothelial  surface, 
the  seat  of  some  abnormal  change. 

Postmortem  Clotting. — Before  entering  into  the  description  of  the 
process  of  thrombosis,  it  will  be  well  to  have  a  perfectly  clear  under- 
standing of  the  appearance  and  characters  of  the  postmortem  clot,  so 
that  they  may  be  differentiated  and  then  set  on  one  side.  Briefly,  such 
postmortem  clot  differs  in  no  essential  respect  from  the  clot  that  forms 
in  blood  removed  from  the  vessels  during  life. 

I.  It  shows  no  stratification,  i.  e.,  the  blood  has  coagulated  en  masse. 
At  most  it  may  show  two  layers — a  paler  upper,  and  a  dark  red  under 
layer.     This  is  an  indication  that  the  clotting  has  not  occurred  immedi- 
ately after  the  circulation  has  ceased,  but  some  little  time  later.     As  can 
be  seen  so  well  after  removal  from  the  body  of  a  slowly  coagulating 
blood,  like  that  of  a  horse,  the  lighter,  white  corpuscles  rise  to  the  sur- 
face and  form  a  "buffy  coat,"  and  upon  coagulation  there  develops  a 
firmer  pale  upper  layer  and  a  dark  red  under  layer,  so  here  the  upper 
layer  is  composed  of  a  fibrin  meshwork  enclosing  leukocytes.    According, 
therefore,  to  (1)  the  rate  of  coagulation,  (2)  the  number  of  contained 
leukocytes,  and  it  may  be,  (3)  the  more  or  less  hydremic  condition  of  the 
blood,  so  may  we  distinguish  three  forms  of  postmortem  clot,  which  pass 
one  into  the  other:  (1)  The  soft,  homogeneous,  red  clot;  (2)  the  clot  with 
the  firm,  rather  dry,  adherent  upper  coat;  and  (3)  the  "chicken-fat"  clot, 
with  abundant  moist,  soft,  glistening  and  semitranslucent,  buffy  coat. 
This  last  form  is  found  more  particularly  in  leukemia  and  other  states 
in  which,  along  with  increase  in  the  circulating  leukocytes,  there  is 
reduction  in  the  erythrocytes  and  some  hydremia. 

II.  Under  the  microscope  the  appearance  is  the  same  as  that  of 
extravascular  blood  clot.     There  is  an  abundant  network  of  fibrin  enclos- 
ing the  corpuscles  in  no  special  order,  save  that  the  white  cells  become 
more  and  more  abundant  toward  the  upper  surface,  that  the  fibrin  is 
more  abundant  and  closer  set  in  the  upper  buffy  coat,  being  most  abun- 
dant in  the  thin,  upper  layer  of  the  second  type.     Blood  platelets  are 
characteristically  few  and  far  between,  save  it  may  be  on  the  very  surface. 

III.  The  clot  is  loose  and  easily  removed  from  the  cavity  in  which 
it  has  developed.    At  most  it  may  encircle  the  chordae  tendinese  and  free 
columns  of  the  musculi  pectinati  in  the  heart;  it  encircles  these  loosely, 
without  being  attached. 


60  THROMBOSIS 

IV.  The  clot  is  of  a  moister  consistency  and  not  nearly  so  friable  as 
the  thrombus. 

While  laying  down  these  differences  so  sharply,  and  while  emphasizing 
the  fact  that  these  considerations  in  the  majority  of  cases  make  it  easy 
to  distinguish  between  postmortem  clot  and  antemortem  thrombus,  we 
have  to  admit  that  we  occasionally  encounter  conditions  in  which  we 
are  at  a  loss  to  decide  whether  the  clot  has  been  formed  immediately 
before  or  after  death.  We  are  still,  that  is,  not  wholly  decided  regarding 
the  existence  and  frequency  of  agonal  thrombosis,  of  a  thrombosis  occur- 
ring during  the  death  agony  which  may,  indeed,  be  the  immediate  cause 
of  death.  We  know  that  very  often  in  the  last  hours  of  life  there  is  a 
pronounced  terminal  leukocytosis.  If,  as  we  believe,  the  leukocytes 
play  a  part  in  liberating  the  certain  bodies  essential  to  fibrin  formation, 
it  is  conceivable  that  a  rapid  liberation  of  these  bodies  might  induce  a 
widespread  coagulation  of  the  blood.  There  are  still  those,  for  example, 
who  hold  that  the  extensive  blood  clots  in  those  dying  from  acute  lobar 
pneumonia,  which  fill  the  cardiac  chambers  and  extend  thence  into  the 
large  vessels,  are  of  this  agonal  nature.  The  abundant  leukocytosis 
in  this  disease  is  certainly  significant;  but  the  absence  of  any  lamination 
must,  we  think,  negative  the  view  that  this  is  of  antemortem  or  moritural 
development. 

Considerations  of  space  prevent  us  from  treating  this  important 
process  at  length;  for  full  details  the  student  is  referred  to  the  "locus 
classicus"  in  our  language,  Professor  Welch's  article  in  Allbutt's  System 
of  Medicine.1  We  shall  but  call  attention  to  the  main  data  necessary 
for  a  sound  understanding  of  the  subject. 

The  thrombus  differs  from  the  postmortem  clot  in  the  following 
particulars:  (1)  It  is  firmly  adherent  on  one  surface  to  the  heart  or 
vessel  wall,  or,  if  not  adherent,  a  study  of  the  vessels  will  reveal  the  site 
at  which  it  originated  and  had  previously  been  attached.  (2)  It  is 
of  drier,  more  friable  nature.  (3)  Under  the  microscope  it  shows  an 
arrangement  of  its  constituents  which,  even  when  fibrin  is  present, 
differs  from  that  seen  in  the  ordinary  blood  clot. 

This  last  statement  may  seem  somewhat  vague;  we  make  this  broad 
statement  because  next  it  must  be  pointed  out  that  there  are  thrombi 
of  various  orders  and  of  different  constitution.  Thus  we  recognize : 

1.  Hyaline  Thrombi. — These  in  general  are  small:  they  are  charac- 
terized by  a  homogeneous,  colorless  appearance.  In  general  they  do 
not  afford  the  staining  reactions  for  fibrin,  although  at  their  edges 
there  may  be  some  development  of  fibrin  threads.  It  is  impossible  at 
the  present  time  to  make  a  positive  statement  regarding  the  mode  of 
formation  of  every  thrombus  of  this  order:  this,  however,  may  be  said, 
that  opinion  is  more  and  more  swaying  to  the  belief  that  they  are  due 
largely  to  a  process  of  agglutination  or  conglutination  rather  than  to 

1  In  German  the  fullest  recent  article  on  the  subject  is  contained  in  Lubarsch's 
Allgemeine  Pathologic,  vol.  i,Part  1.  In  both  these  places  the  allied  subject  of 
embolism  is  treated  with  equal  thoroughness. 


THE  PURE  BLOOD  PLATELET  THROMBUS  61 

coagulation.    It  would  seem  that  we  may  distinguish  at  least  two  if  not 
three  forms. 

(a)  The  Pure  Blood  Platelet  Thrombus. — As  first  shown  by  Eberth 
and  Schimmelbusch  if  a  needle  or  other  fine  foreign  body  be  introduced 
through  the  walls  of  a  vessel  into  its  lumen,  a  thrombus  becomes  formed. 
Microscopic  examination  shows  such  a  thrombus  to  be  finely  granular 
in  appearance:  higher  powers  resolve  the  granules  into  massed  blood 

FIG.  9 


>M$& 

"%£ 


.•;: 


Hyaline  thrombus  in  dilated  venule  of  a  hemorrhoid.     This  was  perfectly  homogeneous. 
Reichert,  obj.  7a,  ocular  4.     Camera  lucida,  reduced  one-third. 

platelets,  which  in  general  take  on  the  eosin  stain  somewhat  deeply. 
Here  and  there  a  leukocyte  may  be  included,  but  the  mass  is  composed 
essentially  of  blood  platelets.  Almost  imperceptibly  the  more  granular 
portions  of  such  a  thrombus  may  pass  into  a  completely  fused  hyaline 
mass. 

There  can  be  no  doubt  that  the  majority  of  white  parietal  thrombi  in 
the  heart  and  larger  vessels  originate  thus  by  an  accumulation  of  blood 
platelets.  Those  blood  platelets,  we  may  recall,  are  normally  present  in 
the  healthy  blood,  and  Wright  has  shown  that  some  of  them  at  least, 
and  these  perfectly  typical,  are  derived  from  the  megacaryocytes  of  the 
bone  marrow. 


62  THROMBOSIS 

(b)  Hyaline  Thrombi  due  to  Conglutination  of  Erythrocytes. — But  more 
particularly  in  the  smaller  veins  of  various  organs,  in  those  of  the  liver 
and  kidney  for  example,  we  encounter  frequently  translucent  hyaline 
thrombi,  sometimes  filling  up  the  whole  lumen,  more  often  partly  occlud- 
ing the  vessel,  and  careful  study  of  a  series  demonstrates,  more  particu- 
larly along  the  free  side,  that  these,  as  Klebs  was  the  first  to  point  out, 
are  composed  of  the  colorless  shadows  of  red  blood  corpuscles.     The 
appearance  suggests  strongly  a  preliminary  hemolysis  and  diffusion  of 
the  hemoglobin  followed  by  agglutination  into  a  hyaline  mass.     Flexner, 
Pearce,  and  others,  by  employing  hemolytic  agents,  have  experimentally 
produced  thrombi  of  this  type  in  the  smaller  vessels.     Leo  Loeb  has 
demonstrated  the  wide  extent  of  this  agglutination  process  throughout 
the  animal  world.     It  is  of  interest  to  note  that  in  man  thrombi  of 
this  order  are  more  particularly  encountered  in  (a)  infectious  disorders, 
suggesting  that  the  bacterial  toxins  have  been  responsible  for  the  hemo- 
lysis, and  (b)  in  states  characterized  by  destruction  of  the  erythrocytes, 
in   carbolic  acid   poisoning,  for  example,  and  eclampsia,  and  experi- 
mentally by  the  exhibition  of  ricin,  diphtheria  toxin,  etc. 

(c)  Hyaline  Thrombi  Associated  with  Plasmoschisis. — But  occasionally 
in  the  examination  of  these  small  thrombi  we  meet  with  appearances 
which  it  is  difficult  to  translate,  save  on  the  assumption  of  an  interme- 
diate process.     One  portion  may  be  completely  hyaline  and  colorless: 
bordering  upon  this  may  be  a  granular  area  composed  of  masses  of 
hyaline  granules  of  the  appearance  of  blood  platelets,  among  these  may 
at  times  be  seen,  now  the  transparent  shadows  of  red  corpuscles,  now 
granulated  red  corpuscles,  and  these  pass  directly  into  a  third  layer  of 
closely  packed  red  corpuscles  of  which  all,  save  it  may  be  an  isolated 
corpuscle  here  and  there,  are  faded  and  have  lost  much  if  not  all  of  their 
hemoglobin.     The  appearances  strongly  suggest  the  phenomenon,  de- 
scribed by  Arnold,  of  plasmoschisis,  of  the  disintegration  of  the  erythro- 
cytes into  particles  of  the  size  and  appearance  of  blood  platelets.     We 
have  observed  this  not  a  few  times  and  are  strongly  of  the  opinion  that 
Arnold  is  correct,  and  that  prior  to  conglutination  the  corpuscles  may 
undergo   this   dissociation  into   bodies  curiously  like  blood   platelets. 
Such  appearances  are  capable  of  interpretation  in  two  ways :  either  we 
must  assume  that  not  all  blood  platelets  are  derived  from  the  mega- 
caryocytes,  but  some  gain  origin  from  dissociation  of  erythrocytes,  or  that 
not  all  appearances  of  blood  platelets  in  thrombi  are  truly  of  this  nature. 

2.  Fibrin  Thrombi. — Thrombi  formed  apparently  from  the  onset  of 
typical  fibrin  and  of  this  alone  are  rare  and  small.  They  have  been 
described  as  occurring  in  the  vessels  of  the  pneumonic  lung,  and  Herzog 
has  encountered  them  in  the  vessels  of  the  renal  glomeruli  in  cases  of 
bubonic  plague. 

From  these  smaller  thrombi  we  pass  now  to  the  larger  forms  present 
in  the  larger  vessels  and  easily  recognizable  by  the  naked  eye,  and  here 
we  find  a  series  of  transitional  forms  leading  up  to  a  condition  which, 
unless  we  examine  carefully,  we  find  difficult  to  distinguish  from  the 
product  of  ordinary  postmortem  intra vascular  coagulation. 


THE  "WHITE  THROMBUS 


63 


3.  The  "White  Thrombus."— In  the  chambers  of  the  heart  or 
adherent  to  the  parietes  of  the  larger  vessels  we  may  encounter  sessile 
or  subpedunculate  thrombi.  In  the  heart  the  surface  of  these  polyp- 
like  masses  is  not  smooth,  but  irregular  and  ridged,  with  intervening 
depressions;  in  the  large  vessels,  where  the  masses  are  more  plaque-like, 
the  surface  is  apt  to  be  smooth.  Sections  of  these  thrombi  show  a  surface 
layer  composed  mainly  of  white  corpuscles  lying  upon  and  intermingled 

FIG.  10 


Thrombus  originating  in  the  left  auricular  appendix  and  increasing  in  size  until  it  has  largely 
occluded  the  mitral  orifice.  If  its  pedicle  of  attachment  in  the  appendix  became  broken  across 
the  liberated  main  mass  would  form  a  ball  thrombus.  (Pathological  Museum,  McGill  University.) 

with  a  fibrinous  layer.  The  interior  of  the  mass,  when  it  has  not  under- 
gone the  autolysis  to  be  presently  noted,  exhibits  a  characteristic  structure. 
Stained  sections  show  areas  which  under  the  low  power  appear  to  be 
homogeneous,  under  the  high  power  are  seen  to  be  composed  of  closely 
packed  blood  platelets.  Among  these  may  be  relatively  rare  leukocytes. 
These  areas  are  seen  to  be  coral-like  bands  or  strands  cut  in  various 
directions;  they  may  be  separated  from  each  other  by  a  close  network 


64  THROMBOSIS 

of  fibrin,  or  by  fibrin  and  leukocytes,  or  again,  by  accumulation  of  red 
corpuscles,  leukocytes,  and  fibrin.  We  thus  encounter  various  grades  of 
the  white  thrombus,  from  (a)  that  composed  in  the  main  of  blood  platelets 
with  occasional  strands  of  fibrin,  through  (6)  that  in  which  leukocytes  in 
clusters  alternating  with  masses  of  blood  platelets  are  the  characteristic 
feature,  to  (c)  that  in  which  the  admixture  of  red  and  white  corpuscles  is 
very  noticeable,  where,  in  fact,  we  have  "the  mixed  thrombus."  More 
particularly,  it  would  seem,  where  there  is  any  extensive  admixture  of 
leukocytes,  the  central  area  of  the  thrombus  is  apt  to  undergo  autolysis 
or  heterolysis  (vol.  i,  p.  339),  and  so  to  exhibit  solution,  the  thrombus 
becoming  reduced  to  a  thin  shell  of  still  solid  fibrinous  matter  with  inter- 
mingled leukocytes,  which  is  very  friable,  breaking  very  easily  under 
the  fingers,  and  then  discharging  a  dirty  creamy  fluid,  the  result  of 
self-digestion  and  solution. 

4.  The  Red  Thrombus. — From  the  above  we  pass  on  to  states  of 
more  rapid  and,  in  general,  more  extensive  thrombosis,  characterized 
by  a  more  abundant  imprisonment  of  red  corpuscles  between  the  strands 
or  layers  of  fibrin,  so  that  the  mass  is  of  redder  color,  and  with  this  the 
blood  platelets  become  less  and  less  evident.  We  approach  nearer, 
that  is,  to  the  picture  with  which  we  are  familiar  in  extra  vascular  clotting. 
We  recognize  two  forms  of  the  red  thrombus: 

(a)  The  Laminated  Red  Thrombus. — This  we  find  in  aneurismal  sacs 
that  have  undergone  progressive  filling  up  with  thrombus,  as  again  in 
the  larger  veins,  such  as  the  femoral  and  its  branches,  that  have  under- 
gone eventual  occlusion.  What  has  happened  in  these  cases  is  that  over 
some  one  area  necrosis  has  taken  place,  or  removal  of  the  endothelial 
lining.  Here  it  would  seem  that  at  first  the  blood  platelets  have  col- 
lected and  undergone  conglutination,  next  a  fibrin  layer  with  imprisoned 
leukocytes  has  formed  over  this,  and  as  some  of  these  have  broken 
down  and  liberated  their  fibrin  ferment  more  fibrin  has  been  formed, 
enmeshing  red  corpuscles ;  more  leukocytes,  and  it  may  be,  blood  platelets, 
have  become  arrested  on  the  surface  of  the  clotted  mass,  and  in  this  way 
layer  after  layer  is  laid  down,  each  layer  consisting  of  a  denser,  more 
fibrinous,  deeper  portion  representing  the  zone  of  earlier  deposit  of 
leukocytes,  and  a  looser  fibrinous  meshwork  enclosing  abundant  ery- 
throcytes.  The  breadth  of  these  layers  is  determined  largely  by  the 
rate  of  the  blood  stream;  where  this  is  rapid,  as  over  a  parietal  thrombus 
in  the  aorta,  the  layers  are  very  thin  and  close  packed.  We  have  placed 
on  record1  a  case  of  dissecting  aneurism  of  the  aorta  in  which  through 
rupture  of  the  intima  in  the  lower  thoracic  region,  the  blood  dissected 
a  channel  between  the  layers  of  the  media,  until  it  gained  reentry  into 
the  original  channel  through  the  femoral  on  the  one  side,  the  iliacs  on 
the  other.  This  channel  was  lined  by  a  relatively  thin  layer  of  dense, 
mainly  fibrinous,  thrombus,  which  had  become  covered  by  a  coat  of 
endothelium.  Once,  that  is,  the  channel  had  become  patent  and  com- 
municating, with  rapid  flow  through  it,  the  very  rapidity  of  that  flow, 

1  Adami,  Montreal  Med.  Jour.,  24:  1895-96:  945  and  25:  1896-97:  23. 


THE  ACUTE  RED  THROMBUS 


65 


\v<>  must  presume,  prevented  the  arrest  of  leukocytes  and  platelets 
on  the  exposed  surface  of  the  thrombus,  and  with  this  arrest  of  fibrinous 
deposit  the  endothelium  had  spread  over  the  surface.  There  had  been 
some  dissection  also  from  the  original  rupture  in  an  upward  direction, 
but  here  no  second  communication  had  been  establ'.shed;  no  current 
had  become  developed.  As  a  result,  this  upper  cul-de-sac  was  com- 
pletely filled  with  a  red  thrombus  of  the  type  now  to  be  mentioned. 


FIG.  11 


Sacculated  aneurism  of  the  ascending  aorta,  largely  filled  up  by  firm  laminated  red 
thrombus.     (McGill  Medical  Museum.) 

(6)  The  Acute  Red  Thrombus. — Where  there  is  complete  arrest  of 
blood  current,  as  in  the  above-mentioned  case,  or  as  occurs  when  a  vessel 
is  ligatured  with  injury  to  the  endothelial  lining,  then  the  column  of 
stagnant  blood  undergoes  clotting  throughout  its  whole  mass,  and  if  it 
be  examined  soon  after  the  occurrence,  before  secondary  changes  have 
taken  place,  no  distinction  is  to  be  made  between  the  mass  and  a  post- 
mortem or  extravascular  clot,  save  perhaps  this,  that  at  the  region  of 
origin  and  attachment  of  the  thrombus,  a  collection  of  blood  platelets, 
or  more  hyaline  deposit  with  some  accumulation  of  leukocytes,  is  to  be 


66  THROMBOSIS 

made  out.  The  origin,  that  is,  is  of  the  same  order  as  in  other  forms  of 
thrombosis,  but  in  the  absence  of  blood  current  there  has  been  no  arrest 
to  the  process  of  fibrin  formation,  which  thus  has  extended  through  the 
whole  mass  of  stagnant  blood.  The  thrombus  in  an  artery  extends 
upward  to  the  next  branch  of  any  size;  once  again  its  growth  is  arrested 
by  the  actively  moving  blood  stream;  in  a  vein  it  extends  downward 
along  the  branches  until  a  region  or  regions  of  anastomosis  and  collateral 
circulation  is  reached  (see  Fig.  12,  p.  71). 

We  have  here  described  the  different  forms  of  thrombus  in  the  reverse 
order  of  that  usually  employed,  and  have  done  this  in  order  to  lay 
emphasis  upon  the  divergencies  between  the  main  forms  of  thrombosis 
and  extravascular  coagulation.  It  is  obvious  that  although  the  end 
result  is  the  same,  namely,  the  production  of  solid  matter  in  the  place  of 
fluid  blood,  that  result  is  attained  in  more  than  one  way.  As  to  the 
relative  frequency  and  importance  of  these  different  modes  of  formation, 
namely,  by  the  conglutination  of  blood  platelets,  by  the  conglutination 
of  red  corpuscles,  and  by  the  development  of  a  fibrinous  network  en- 
meshing the  corpuscular  elements  we  are  still  undecided;  at  most  it  can 
be  stated  that  the  conglutinative  forms  are  being  more  and  more  recog- 
nized as  the  more  usual.  For  ourselves,  judging  from  the  specific 
staining  reactions,  we  believe  that  fibrin  and  the  hyaline  matter  of  white 
thrombi  are  chemically  closely  allied  and  of  the  same  order,  and  so  are 
developed  as  the  result  of  similar  reactions.  The  constant  attachment 
of  the  beginning  thrombus  to  an  area  of  the  vascular  wall,  which  in  most 
cases  can  be  seen  to  have  undergone  injury  or  degeneration,  indicates 
that  either  the  damaged  endothelium  or  the  underlying  cells  supply 
something — probably  of  the  nature  of  fibrin  ferment — which  initiates 
the  conversion  of  the^  substance  of  the  blood  platelets  or  erythrocytes 
into  fibrin-like  material,  and  that  once  started,  the  process  would  seem 
apt  to  continue  into  the  plasma  outside  and  beyond  as  true  fibrin  forma- 
tion. What  part  the  blood  platelets  play  in  the  ordinary  process  of 
coagulation  is  as  yet  undetermined;  at  most  it  is  significant  that  in  con- 
ditions in  which  their  number  is  diminished,  as  in  purpura,  the  coagu- 
lative  power  of  the  blood  is  likewise  seen  to  be  diminished.  But  unless 
we  accept  the  observations  of  Wlassow,1  that  in  ordinary  fibrin  forma- 
tion in  shed  blood  the  conglutination  of  blood  platelets  is  the  first  stage, 
it  is  difficult  to  bring  into  line  with  the  prevalent  theories  of  coagulation 
these  observations  upon  thrombus  formation,  observations,  be  it  noted, 
not  of  one  isolated  observer,  but  of  a  long  series  of  trained  pathologists, 
from  Eberth  and  Schimmelbusch  in  1888  onward. 

It  deserves  note  that  Wooldridge  derived  his  Afibrinogenfromthe  blood 
platelets,  and  he  held  that  fibrin  is  formed  (without  intervention  of  fibrin 
ferment)  by  a  junction  between  this  and  other  fibrinogens  present  in 
the  plasma.  His  views  have  never  gained  general  acceptance,  and 
practically  all  modern  physiologists  recognize  the  part  played  by  a  fibrin 
ferment,  even  if  most  regard  this  as  not  liberated  direct  into  the  plasma, 

1  Ziegler's  Beitrage,  15:1894:543. 


THE  ACUTE  RED  THROMBUS  67 

but  require  a  prothrombin,  present  in,  and  capable  of  liberation  from, 
leukocytes  and  most  body  cells,  which,  under  the  action  of  a  zymoplastic 
substance,  liberated  from  red  corpuscles  and  other  cells  proper,  gives 
origin  to  thronibin,  or  fibrin  ferment  proper.  This  thrombin  in  its  turn 
is  regarded  as  acting  upon  the  paraglobulin  of  the  plasma,  converting 
it  into  fibrinogen,  or  metaglobulin,  and  eventually  into  soluble  fibrin, 
and  this  in  the  presence  of  calcium  salts  becomes  converted  into  the 
more  solid  fibrin.  So  many  and  so  diverse  are  the  theories  of  coagulation, 
that  we  do  not  in  the  least  believe  that  the  process  here  indicated  is  what 
actually  occurs.  No  one  appears  to  be  satisfied  that  we  have  as  yet 
gained  a  sure  knowledge  of  the  process.  We  have  a  certain  satisfaction 
in  leaving  to  the  physiologists  the  teaching  of  a  subject  which  they  have 
for  long  years  made  peculiarly  their  own.1  At  most,  we  would  urge 
that  evidently  something  is  involved  which  is  common  to  the  blood 
platelets,  to  the  erythrocytes,  to  the  white  corpuscles  (for  it  is  where 
these  are  accumulated  in  greatest  numbers  that  the  fibrin  is  laid  down 
most  densely),  and  it  may  be  also  to  the  plasma  (for  in  rapidly  de- 
veloping thrombi  the  fibrin  threads  extend  into  the  plasma  between 
the  cells),  and  this  something  would  seem  to  be  of  the  nature  of  a 
globulin  or  globulins,  for  such  are  common  to  both  cells  and  plasma, 
and  there  would  appear  to  be  an  agreement  among  the  physiological 
chemists  that  fibrinogen,  the  precursor  of  fibrin,  is  a  metaglobulin. 
The  fact  that  the  normal  circulating  blood  does  not  coagulate,  and 
that  the  ordinary  thrombus  is  seen  to  develop  in  connection  with  the 
vessel  wall  at  a  region  of  disease  or  injury  or  loss  of  the  endothelium, 
would  suggest  that  something  not  ordinarily  present  in  the  circulating 
blood  is  given  off  at  such  an  area,  and  initiates  the  conversion  of  the 
dissolved  protein  into  its  more  solid  modification,  fibrin.  The  whole 
process  points  to  the  liberation  of  an  enzyme.  We  do  not  pretend  to  say 
that  the  blood  platelets  are  absolutely  essential  for  the  process;  but  the 
way  in  which,  once  the  process  has  started  in  stagnating  blood,  the  strands 
of  fibrin  spread  rapidly  through  a  relatively  large  mass,  and  the  abun- 
dance of  dense  fibrin  in  immediate  relationship  to  clusters  of  white  cor- 
puscles on  the  surface  of  ordinary  white  thrombi  and  elsewhere,  prepare 
us  to  see  that  other  cells  besides  those  of  the  vessel  walls  may  liberate 
the  enzyme  or  initiator  of  the  conversion  of  soluble  protein  into  fibrin. 
Nay,  more,  although  this  is  contrary  to  the  generally  received  doctrine, 
just  as,  experimentally,  by  the  injection  of  certain  tissue  extracts,  it  is 
possible  to  induce  an  almost  universal  thrombosis  throughout  the  vascular 
system,  so  is  it  possible  to  imagine  the  existence  of  conditions,  such  as 
extensive  disintegration  of  the  circulating  leukocytes,  which  would  lead 
to  a  process  of  thrombosis  independent  of  any  disturbance  of  the  vessel 
wall,  conditions  in  which  the  multiple  thrombi  or  masses  of  clotted 
blood  would  have  no  primary  attachment.  Admitting  this,  it  has  also 
to  be  admitted  that  the  more  we  study  actual  cases  of  thrombosis,  the 

1  A  very  full  review  of  the  data  and  theories  of  coagulation  is  given  by  Buck- 
master  in  Science  Progress,  2;  1907:  51 


68  THROMBOSIS 

more  we  become  impressed  by  the  co-existence  of  lesions  of  the  vascular 
wall. 

The  Factors  Favoring  Thrombosis.— The  analyses  made  thus  far 
of  the  incidence  of  thrombosis  in  man  in  various  conditions  of  disease, 
and  the  experiments  made  upon  the  lower  animals,  indicate  that  very 
diverse  orders  of  disturbance  favor  the  development  of  thrombi — so 
diverse,  in  fact,  that  it  is  a  matter  of  extraordinary  difficulty  to  arrive 
at  any  sure  conclusion,  and  as  a  result  we  find  that  different  observers 
arrive  at  very  different  conclusions  regarding  their  relative  importance. 
We  will  here  record  these  factors,  giving  the  more  important  data  and 
the  conclusions  that  have  been  drawn,  and  then  proceed  to  weigh  the 
evidence  in  the  light  of  what  has  already  been  laid  down  concerning 
the  actual  process. 

1.  Slowing  and  Stagnation  of  the  Blood. — Thrombosis  is  more  common 
in  the  venous  system  than  in  the  arterial — according  to  Lubarsch,  in 
the  proportion  of  more  than  4:  1 — as  also  it  is  peculiarly  apt  to  occur 
in  regions  of  dilatation  of  the  channel  where  the  current  necessarily 
becomes  slow,  e.  g.,  in  the  auricular  appendages,  in  the  sinuses  or  depres- 
sions between  the  muscular  bands  of  the  heart  chambers,  in  varicose 
veins,  and  in  aneurisms.     Where  the  blood  stream  is  rapid,  despite  the 
existence  of  other  favoring  factors,   thrombosis  does  not  necessarily 
show  itself,  e.  g.,  there  may  be  an  extensive  atheromatous  ulceration  of 
the  aortic  wall,  with  loss  of  endothelium,  and  no  sign  of  thrombosis. 
This  led  Virchow  to  regard  slowing  of  the  blood  stream  as  the  prime 
factor. 

2.  Eddying  of  the  Blood. — A  factor  upon  which  von  Recklinghausen 
would  lay  great  stress  is  the  formation  of  eddies  rather  than  the  existence 
of  simple  stagnation  in  regions  in  which  the  vascular  channel  undergoes 
expansion.      There  is  not,  he  urged,  absolute  stagnation  in  the  pockets 
of  the  valves  of  the  veins;  nevertheless,  these  are  peculiarly  favorable 
seats  for  the  origin  of  venous  thrombi.     If  we  conceive  an  eddy  as  a  whirl, 
our  first  idea  is  that  in  it  the  blood  flow  is  faster,  and  that,  therefore, 
the  conditions  are  unfavorable  rather  than  favorable  for  the  deposit  of 
blood  platelets  and  other  cells.     This,  however,  is  not  a  complete  con- 
ception; while  at  the  edge  of  an  eddy  there  is  relatively  rapid  flow,  at  its 
centre  there  may  be  relative  stagnation.     What  is  more,  the  very  whirling 
nature  of  the  flow  does  away  with  any  peripheral  cell-free  layer  of  the 
plasma,  so  that  once  cells  become  arrested  in  the  quiescent  area,  the 
conditions  distinctly  favor  the  adhesion  and  accumulation  of  other 
platelets  or  cells. 

3.  Hemolysis  and  Destruction  of  Corpuscles. — It  is  those  agents,  exogen- 
ous and  endogenous,  which  lead  to  corpuscular  disintegration,  which 
characteristically,  when  exhibited,  bring  about  extensive  thrombosis. 
There  is  a  long  list  of  exogenous  poisons  having  such  properties — salts 
of  mercury,  lead,  arsenic,  etc.,  potassium  chlorate,  sulphates  and  sul- 
phites, nitrobenzole,  toluylenediamin,  and  other  toluylene  compounds, 
various  aniline  derivatives,  phenylhydrazin,  etc.,  carbolic  and  salicylic 
acids,  various  compounds  of  vegetable  origin,  ricin,  extracts  of  amanita 


PREDISPOSING  CAUSES  69 

and  other  poisonous  mushrooms;  others  of  animal  origin,  e.  g.,  snake 
venom  and  sundry  enzymes,  pepsin,  etc.  Of  the  endogenous  poisons, 
the  diffusible  products  from  extensive  burns,  the  (unknown)  toxic  agents 
in  eclampsia  and  possibly  toxic  substances  present  in  the  blood  in  severe 
secondary  anemias  (e.  g.,  that  accompanying  cancer)  may  be  mentioned. 
In  all  these  conditions  there  is  a  marked  tendency  to  thrombosis,  either 
multiple  and  small  in  the  capillaries,  or  sometimes  of  larger  size  in  the 
larger  veins.  Stress  is  laid  upon  these  data  more  particularly  by  those 
upholding  conglutination  as  a  main  factor. 

4.  Bacteria  and  Their  Products. — But  it  has  to  be  admitted  that  intoxi- 
cations pure  and  simple,  whether  endogenous  or  exogenous,  are  rela- 
tively infrequent  compared  with  infectious  and  bacterial  intoxication. 
Most  people  die  from  terminal  infections,  and  attention  is  being  increas- 
ingly drawn  to  the  fact  that  even  in  what  are  recognized  as  bland  thrombi 
bacteria  are  to  be  detected,  or  cultures  gained,  while  conversely,  as 
shown  by  Welch  and  Lubarsch,  if  known  cases  of  infection  be  carefully 
studied — suppurative  cases,  lobar  pneumonia,  typhoid,  appendicitis, 
diphtheria,  acute  rheumatism,  measles,  influenza,  etc. — capillary 
thrombi  in  the  brain,  lungs,  kidneys,  and  intestinal  walls  are  found  to 
be  remarkably  frequent. 

There  are  different  views  as  to  how  the  bacteria  act.  Thus,  certain 
observers  have  dwelt  upon  the  hemolytic  action,  and  have  pointed  out 
that  the  pyococcus  aureus  is  most  actively  hemolytic,  and  is  found 
associated  with  thrombi  very  frequently.  But  thrombi  are  frequent 
in  acute  tuberculosis  and  in  typhoid,  and  the  microbes  of  neither  of  those 
diseases  have  pronounced  hemolytic  powers.  It  is  true  that  as  Professor 
Welch  points  out,  in  many  of  these  cases  examination  reveals  not  the 
microbes  of  the  main  disease,  but  those  of  some  secondary  infection, 
streptococci,  bacillus  coli,  etc.  There  is  diversity  of  opinion,  again, 
regarding  the  actual  presence  of  the  bacteria  in  the  blood  stream;  some 
observers  point  out  that  the  filtered  culture  fluids  of  forms  like  bacillus 
typhosus,  bacillus  coli,  and  bacillus  diphtherise,  when  injected  into  the 
veins,  have  little  effect  in  inducing  thrombosis  (Jakowski);  others,  like 
Talke,  demonstrate  that  parenchymatous  inoculation  of  pyococci  leads 
in  the  majority  of  cases  to  thrombosis  of  the  vessels  in  the  immediate 
neighborhood,  and  this  without  of  necessity  any  bacteria  being  present 
in  the  thrombi.  In  our  laboratory  Leo  Loeb  demonstrated  that  the 
addition  of  cultures  of  certain  organisms  accelerated  the  rate  of  coagula- 
tion of  extravascular  blood,  while  cultures  of  other  species  had  no  effect. 
But  accepting  his  data,  his  results  bear  little  apparent  relationship  to  the 
data  of  disease;  thus,  for  example,  the  feebly  pathogenic  Micrococcus 
prodigiosus  was  found  to  have  a  greater  accelerating  power  than  the 
typhoid  bacillus,  and  the  streptococcus  was  without  affect.  Nor,  so  far 
as  we  can  see,  did  his  results  tally  with  the  hemolytic  powers  of  the 
species  tested. 

5.  Disease  and  Injury  of  the  Vascular  Wall. — We  have  already  laid 
stress  upon  this  as,  in  our  opinion,  a  most  important  factor,  but  must 
again  impress  upon  the  reader  that  it  is  not  everything.  It  is  quite 


70  THROMBOSIS 

true  that  when  we  mechanically  injure  or  destroy  the  endothelium  of 
the  living  vessel,  there  we  surely  gain  thrombus  formation  over  the 
region  of  injury.  But  the  extent  depends  very  largely  upon  the  rate 
of  blood  flow  over  the  injured  area;  the  more  rapid  the  rate,  the  less 
the  resulting  thrombosis.  Nevertheless,  we  can  recall  no  observations  in 
which  destruction  or  grave  injury  to  the  previously  intact  endothelium 
has  not  been  followed  by  some  grade  of  thrombosis.  Here  a  fine,  but 
what  must  be  considered  an  important,  distinction  must  be  made.  We 
do  not — because  we  cannot — hold  that  the  thrombosis  follows  the  ex- 
posure of  what  is  dead  or  foreign  material  to  the  circulating  blood.  We 
know,  for  example,  that  the  exhibition  of  foreign  matter  in  the  blood 
is  not  necessarily  followed  by  coagulation.  It  is  possible  to  place  balls 
of  perfectly  smooth  glass  in  the  larger  vessels  or  heart  cavity  without  any 
clot  forming  round  them.  Guthrie,  of  St.  Louis,  has  afforded  the  most 
remarkable  instance  to  the  point.  Taking  a  length  of  the  vena  cava 
of  the  rabbit,  he  has  hardened  this  in  formalin  solution  for  several  days, 
washed  out  the  formalin  and  dehydrated  with  strong  alcohol,  and 
then  impregnated  the  piece  with  liquid  paraffin,  following  upon  which 
he  has  implanted  the  segment  in  the  course  of  the  rabbit's  carotid  artery. 
And  notwithstanding  the  piece  of  dead  vein  has  apparently  functionated 
perfectly  for  twenty-two  days  with  no  sign  of  thrombosis.  It  is  true 
that  foreign  bodies  with  rough  surface  introduced  into  the  blood  stream 
become  covered  with  a  layer  of  fibrin;  blood  platelets  and  leukocytes 
adhere  to  the  surface  irregularities,  and  their  disintegration  leads,  we 
suggest,  to  the  liberation  of  the  substance  which  initiates  coagulation  and 
fibrin  formation.  If  the  circumstances  are  such  that  this  substance  is 
not  given  off,  or,  being  given  off,  is  carried  too  rapidly  by  the  blood  stream, 
then  thrombosis  cannot  ensue.  It  is  in  this  way  that  we  would  account 
for  the  absence  of  thrombosis  over  exposed  (dead)  calcareous  plaques 
and  ulcers  of  the  aortic  wall.  But  admitting  this,  we  have  to  acknowledge 
that  there  is  a  group  of  cases,  more  especially  of  capillary  thrombosis, 
in  which  microscopic  examinations  reveal  no  recognizable  departure  of 
the  endothelium  from  the  normal,  or,  at  most,  a  grade  of  fatty  degenera- 
tion which  is  common  in  infectious  processes,  and  most  often  found 
unaccompanied  by  thrombosis.  Here  we  again  revert  to  the  same 
order  of  phenomena  noted  in  connection  with  the  coagulation  occurring 
around  foreign  bodies;  it  is  in  the  slighter  cases  of  this  order  that  we 
occasionally  encounter  vessels  in  which  either  desquamated  endothelial 
cells,  or  leukocytes,  or  what  are  apparently  clumps  of  blood  platelets 
lying  isolated  in  the  blood,  away  from  the  vessel  wall,  are  surrounded 
by  a  coarse  radiation  of  fibrin  filaments.  Evidently  all  these  orders  of 
cell  substance  can  give  off  the  body — ferment  or  pro-ferment — which, 
interacting  on  substances  in  the  plasma,  gives  origin  to  fibrin. 

Localization  of  Thrombi. — They  may  occur  in  any  portion  of  the 
blood  system.  Here  we  distinguish : 

1.  Cardiac  Thrombi. — (a)  Of  these,  the  commonest  form — although 
we  are  apt  not  to  regard  them  as  such — are  the  vegetations  upon  the 
valves  in  acute  endocarditis.  (6)  The  more  typical  thrombi,  globular, 


CARDIAC  THROMBI 


71 


FIG.  12 


sessile,  or  pedunculate  are  found  more  particularly  in  the  auricular  ap- 
pendices and  at  the  apices  of  the  ventricles — found  in  obstructive  heart 
disease  leading  to  cardiac  insufficiency  with  retardation  of  the  blood 
flow,  and  chronic  diseases  of  the  lungs,  arteries,  and  kidneys,  as  again 
in  cachectic  states.  We  deal  in  these  cases  with  retardation  rather  than 
with  actual  stasis  of  the  blood,  and  this  retardation  is  most  manifest  in 
the  "pockets"  of  the  heart.  Along  with  this, 
microscopic  examination  reveals  degeneration 
of  the  endocardium,  (c)  Another  group  of 
more  flattened  mural  thrombi  may  be  found 
in  various  positions  either  in  the  auricles  or 
ventricles.  These  are  situated  over  localized 
areas  of  necrosis  or  ulceration  of  the  endo- 
cardium, due  either  to  infection,  infarction  of 
the  wall,  or  partial  aneurism,  (d)  A  form 
apart  is  the  ball-thrombus,  found  loose  in  the 
left  auricle;  some  score  of  cases  of  this  condi- 
tion have  been  described.  The  condition  is 
that  of  a  relatively  firm,  globular  mass,  vary- 
ing in  size  from  that  of  a  walnut  to  that  of 
a  hen's  egg,  lying  loose  in  the  cavity,  and 
eventually  causing  sudden  death  by  acting  as 
a  ball  valve  over  the  stenosed  mitral  orifice. 
Some  examples  have  shown  a  rough  area, 
indicating  the  recent  detachment  of  a  globular 
thrombus  from  the  auricular  endocardium, 
upon  which  a  thrombotic  area  of  origin  has 
been  detected;  others  have  been  smooth  or 
homogeneous  over  the  whole  surface,  indi- 
cating that  the  thrombus  has  been  free  for 
some  considerable  time  before  the  fatal  event, 
and  that  by  the  deposit  of  successive  laminae 
of  fibrin,  it  has  increased  in  size  while  rotat- 
ing in  the  auricle.  Von  Recklinghausen  has 
doubted  whether  these  necessarily  cause  sud- 
den death,  and  much  is  to  be  said  in  favor  of 
his  arguments;  we  may  conclude  that  they 
do  not  necessarily  bring  about  sudden  death, 
but  that  at  the  same  time  they  must  be 
regarded  as  producing  it  in  the  majority  of 
cases.  In  only  one  other  region  do  we  know 
of  the  existence  of  similar  ball-thrombi,  and 

this  is  an  individual  observation  of  our  own.  In  a  case  of  sudden  death 
we  encountered  a  firm,  oval,  free  mass  occupying  the  cavity  of  the  left 
coronary  cusp  of  the  aortic  valve,  and  occluding  the  left  coronary  orifice. 
We  have  recently  encountered  an  almost  identical  case  of  sudden  death, 
but  in  this  the  globular  mass  was  still  attached  by  a  delicate  pedicle  to 
the  eroded  aortic  wall  immediately  above  the  left  coronary  orifice. 


Acute  red  thrombus  of  iliac  vein. 
(McGill  Medical  Museum.) 


72  THROMBOSIS 

2.  Arterial  Thrombi. — Such  may  be  found  either  parietal  or  occluding 
in  the  aorta  or  any  of  its  branches,  and  then  in  general  as  the  result  of 
either  mechanical  injury  (i.  e.,  ligature)  or  of  arteriosclerotic  changes, 
or  lastly,  as  secondary  to  embolism.     Most  frequently,  however,  they 
are  encountered  in  the  arteries  of  the  extremities,  and  more  especially 
of  the  lower  extremities.     An  important  group  occur  in  connection  with 
atheromatous  degeneration  of  the  cerebral  arteries;  another  group,  often 
but  not  always  of  embolic  origin,  in  the  pulmonary  arteries. 

3.  Venous  Thrombi. — These  are  in  many  respects  of  the  greatest 
medical  interest  and  the  most  widespread.     Indeed,  many  factors  com- 
bine to  make  the  veins  the  seat  of  election  for  thrombosis — the  poorer 
quality  of  the  blood,  its  slower  flow,  the  absence  of   pulsation,  the 
presence  of  pockets  behind  the  valves,  the  thinness  of  the  walls,  and  low 
blood  pressure,  leading  easily  to  local  arrest  of  the  blood  flow  in  con- 
sequence of  pressure  from  without.     As  Welch  points  out,  thrombosis 
most  often  begins  in  vessels  where  these  conditions  are  together  most 
operative,  namely,  in  the  middle  sized  veins  rather  than  in  the  smaller, 
or  those  unprovided  with  valves.     Thus,  characteristically,  thrombosis 
shows  itself  first  in  the  femorals,  and  not  in  the  smaller  veins  of  the 
lower  extremity,  or  in  the  vena  cava,  in  the  cerebral  sinuses  more  often 
than  in  the  cerebral  veins.     Once  they  originate,  they  are  apt  to  extend 
progressively  in  both  directions,  although  the  smallest  veins  of  a  part 
usually  do  not  become  involved.     It  is  the  left  common  iliac  vein  that 
has  the  larger  and  more  obstructed  course,  the  left  innominate  vein  that 
is  the  larger  and  the  more  oblique  channel  for  return  flow;  thus  we  find 
thrombi  more  common  in  the  veins  of  the  left  leg  and  arm. 

4.  Capillary  Thrombi. — We  have  already  referred  to  the  frequency  of 
small  hyaline  thrombi  in  the  smaller  vessels,  evidently  of  local  origin; 
it  is  interesting  in  this  relationship  to  call  attention  to  what  has  been 
stated  above,  namely,  that  the  ordinary  retrograde  thrombus  does  not 
in  general  extend  back  into  the  small  veins  and  capillaries.     Thrombosis 
of  the  ordinary  type  can,  however,  show  itself  in  capillaries,  and  that 
in  inflammations,  and  more  particularly  in  infectious  conditions,  as  in 
the  zone  surrounding  a  focal  infectious  condition,  an  abscess,  or  even 
a  granuloma. 

Effects  of  Thrombosis. — To  discuss  the  effects  of  thrombosis  of  the 
arteries,  veins,  and  capillaries  is  to  pass  in  review  the  results  of  closure 
of  all  the  vessels  of  the  body,  as  that  affects  the  different  individual 
parts  and  organs.  That  is  impossible;  at  most  here  it  may  be  stated 
in  the  broadest  way  that  there  are  certain  main  factors  that  determine 
those  effects,  namely,  first  and  foremost,  the  extent  to  which  the 
obliteration  of  a  given  vessel  affects  the  nutrition  of  a  part;  and 
secondly,  and  of  yet  greater  importance  for  the  organism,  the  amount  of 
tissue  thrown  out  of  gear  relative  to  the  importance  of  that  tissue.  Thus, 
for  example,  a  very  small  vessel  supplying  just  one  small  collection  of 
nerve  cells  in  the  brain,  if  thrombosed,  by  leading  to  the  destruction  of 
an  important  centre,  may  have  very  profound  if  not  rapidly  fatal  effects, 
whereas  thrombosis  of  one  renal  vein,  although  leading  to  almost  com- 


CANALIZATION  73 

plete  destruction  of  the  whole  kidney,  through  the  compensating  activity 
of  the  other  kidney  may  be  followed  by  very  little  general  disturbance. 
Remembering  that  here  we  deal  with  phenomena  that  are  the  common 
end  results  not  only  of  thrombosis,  but  also  of  embolism,  and  in  addition, 
of  obliteration  of  vessels  by  very  many  means,  it  has  seemed  wise  to 
discuss  the  broad  general  results  of  closure  of  vessels  in  an  earlier 
chapter. 

The  Changes  that  Occur  in  the  Thrombus. — Whatever  the 
cause — whether  the  greater  relative  amount  of  fibrin  entering  into  its 
composition,  or  its  very  architecture,  whereby  the  contraction  of  the 
fibrin  is  more  effective  in  driving  out  the  serum — the  thrombus  is,  even 
when  recent,  of  a  drier  nature  than  the  ordinary  blood  clot.  If  of  long 
continuance,  various  changes  may  occur,  some  leading  to  greater  firm- 
ness, others  to  softening,  which,  however,  is  quite  distinctive.  These 
changes  are  of  the  following  orders: 

1.  Absorption. — When   a    thrombus    is    of   moderate   size,    we   have 
evidence  that  it  may  wholly  disappear,  the  vascular  channel  becoming 
restored.    The  main  agent  at  work  in  such  cases  is  apparently  leukocytic 
activity,  with  solution  and  absorption  of  the  fibrin. 

2.  Central  Auto  lysis. — This  would  seem  to  be  closely  allied  to  the  pre- 
vious change.     It  is  most  commonly  encountered  in  old  globular  and 
mural  cardiac  thrombi.     The  thrombus  apparently  solid,  with  character- 
istic netted  surface,  is  found  to  be  a  mere  brittle  shell  filled  with  a 
discolored,  puriform  fluid.     But  this  fluid  is  in  no  sense  pus.     It  contains 
granular  debris,  fatty  globules,  red  corpuscles,  and  occasional  fat-con- 
taining leukocytes,  and  is  the  result  of  self-digestion  of  the  thrombus, 
or,  perhaps  more  accurately,  of  heterolysis,  by  the  agency  of  leukocytes, 
both  those  originally  present  and  those  wandering  in  from  the  surface. 

3.  Organization. — This  is  yet  another  natural  process.     The  presence 
of  foreign  matter  in  the  lumen  of  the  vessel  acts  as  a  chemiotactic  irritant, 
so  that,  in  the  first  place,  leukocytes  are  attracted  into  the  substance  of 
the  thrombus  from  the  vessel  wall,  not  so  much  where  the  lining  endo- 
thelium  is  intact,  as  where  it  is  damaged  and  wanting,  at  the  site  of 
adhesion;  and  here  also,  following  the  leukocytic  invasion,  there  would 
seem  to  be  a  similar  chemiotactic  entrance  of  capillary  processes  and 
loops.      In  this  way,  the  leukocytes  first  digesting  the  fibrin,  there  is 
an  entrance  of  granulation   tissue  into  the  thrombus,  and  this   may 
advance  and  progress  until  the  whole  thrombus  undergoes  in  part  a 
slow  heterolysis  and  absorption,  in  part  replacement  by  well-formed  and 
vascularized   connective   tissue.     Through   the  absorption,    or,    again, 
through  the  contraction  of  the  "  cicatricial"  tissue,  what  had  been  a 
large,  obliterating  thrombus  comes  to  be  represented  by  a  small  con- 
tracted mass  of  fibrous  tissue. 

4.  Canalization. — Nor  does   this  necessarily  completely  occlude  the 
vessel.     On  the  contrary,  an  old  thrombus  may  come  to  be  represented 
by  one  or  more  bands  or  bridles  crossing  the  almost  completely  restored 
lumen  of  a  vessel.     Or  the  vessel  may  be  narrowed  and  contracted  at 
the  site  of  the  old  thrombus,  presenting  one  or  more  narrow  channels 


74 


THROMBOSIS 


through  which  communication  is  regained  between  the  proximal  and 
distal  portions  of  the  hitherto  obstructed  vessel.  This  process  of  ''canal- 
ization" presents  not  a  few  points  of  interest.  The  channels,  large  or 
small,  are  lined  with  endothelium  which  is  in  direct  continuity  with 


FIG.  13 


Schema  of  mode  of  canalization  of  a  thrombus:  1,  thrombus  occluding  a  vessel;  2,  diseased 
intima  of  vessel  to  which  the  thrombus  is  adherent;  3,  endothelium  of  vessel  above  the  thrombus, 
growing  over  the  thrombus  and  at  4  passing  downward  into  depression  on  its  surface,  one  of 
its  cells  at  5,  sending  downward  a  process  to  join  with  similar  endothelial  process  from  one  of  the 
capillaries  entering  the  thrombus  at  6,  in  the  process  of  organization  of  the  same.  The  process 
at  5  will  ultimately  form  a  capillary  channel  of  communication,  which  will  undergo  progress  in 
enlargement. 


that  lining  the  normal  vessel  above  and  below.  How  have  they  come 
to  be  formed  ?  It  is  easy  to  understand  that  a  parietal  non-obliterating 
thrombus  should  become  covered  in  process  of  time  by  an  extension 
of  the  endothelium  lining  the  vessel  immediately  beyond  the  site  of 


CALCIFICATION  75 

adhesion.  This  would  explain  the  formation  of  a  lateral  channel; 
it  does  not  explain  the  presence  of  central  channels.  For  these  the 
only  adequate  explanation  would  seem  to  be  the  establishment  of  free 
communications  between  certain  of  the  capillaries  of  the  replacement 
granulation  tissue  and  the  lumen  of  the  obliterated  vessel  above  and 
below\  Once  such  communication  is  established,  and  the  blood  finds 
its  way  from  the  lumen  above  to  the  lumen  below,  then  along  the 
lines  of  Thoma's  principles,  the  difference  in  bloqd  pressure  between 
the  two  parts  and  the  rate  of  flow  through  the  new  channel  would  lead 
to  its  progressive  dilatation,  until  from  a  mere  capillary  a  wide  channel 
becomes  developed. 

5.  Putrefactive  Softening. — We  have  already  noted  that  even  in  what 
are  of  the  nature  of  bland  thrombi,  bacteria  have  been  detected;  it  is 
not,  therefore,  all  bacteria  that  lead  to  disintegration  of  thrombi.     More 
particularly  when  we  isolate  the  pyogenic  bacteria,  more  particularly 
again  in  the  condition  of  thrombophlebitis,  do  we  meet  with  a  true  sup- 
purative  softening  of  a  thrombus  and  replacement  by  true  pus.      It  is 
not  merely  that  these  bacteria  multiplying  in  the  thrombus  cause  soften- 
ing of  the  same  through  the  action  of  their  proteolytic  enzymes,  but  in 
addition,  through  the  diseased  walls  of  the  vein,  leukocytes  are  attracted 
in  great  numbers.     Streptococci  and  bacillus  coli  are  most  frequently 
found  in  these  conditions,  although  the  latter,  with  others  of  the  group  of 
intestinal  bacteria,  may  also  be  found  in  conditions  in  which  there  is 
not  so  much  an  attraction  of  leukocytes  as  pure  putrefactive  disinte- 
gration. 

6.  Loosening  of  Thrombi  and  its  Effects. — Such  softening  of  thrombi, 
more  particularly  in  the   heart   (endocardial  vegetations)  and  in   the 
veins,  leads  to  liberation  of  the  whole  or  portions  of  the  same,  and  so 
to  the  formation  of  emboli.     But  embolism  may  be  due  also  to  portions 
of  bland  thrombi — cardiac  vegetations,  and  polypoid  thrombi  of  the  heart, 
and  mural  thrombi  of  the  aorta;  these  may  become  dislodged  mechan- 
ically by  the  force  of  the  blood  stream  or  unusual  movements  either  in 
the  blood  or  the  containing  vessel.     Similarly,  the  cases  are  fairly  fre- 
quent in  which,  through  some  forced  or  unusual  movement,  relatively 
long  thrombi  in  the  systemic  veins  become  broken  across.     The  super- 
vention of  pulmonary  embolism  and  sudden  death  is  the  gravest  danger 
in  these  cases.     The  effects  of  embolism  will  be  considered  in  another 
chapter. 

7.  Calcification. — In  certain  regions  of  the  body,  notably  in  the  pros- 
tatic  and  uterine  plexuses,  and  to  a  less  extent  in  the  spleen,  thrombi  of 
moderate  dimensions  become  the  seat  of  calcareous  deposits,  and  so 
form  phleboliths.     This   change  has   already  been  discussed    (vol.   i, 
p.  862). 


CHAPTER    III. 

THE  BLOOD— HEMORRHAGE. 

STRICTLY  speaking,  hemorrhage1  is  a  "blood  burst/'  or  is  the  process 
of  escape  of  the  blood  out  of  the  vessels  in  which  normally  it  is  confined ; 
in  practice  the  term  covers  not  only  the  act  of  escape,  but  also  the  state 
of  blood  out  of  place,  and  thus  in  discussing  hemorrhage  we  take  into 
account  not  merely  the  means  of  escape  and  the  results  to  the  organisms 
at  large  of  such  escape,  but  also  the  changes  that  occur  in  the  extrava- 
sated  fluid. 

Causes. — We  recognize  two  orders  of  escape:  (1)  That  brought 
about  by  gross  breach  of  continuity  of  the  vessel  walls — hemorrhage 
per  rhexin — and  (2)  that  occurring  in  capillary  vessels  without  gross 
breach  of  continuity,  through  interstices  of  the  wall — hemorrhage  per 
diapedesin.  The  causes  of  the  first  of  these,  of  rupture,  (a)  may  act 
from  without,  through  injury  or  trauma,  through  inflammation,  through 
atrophy  secondary  to  pressure  from  tumors  in  apposition,  etc.,  and 
through  removal  of  the  normal  pressure  upon  and  support  exerted  by 
surrounding  tissues;  (6)  may  be  due  to  disease  and  weakening  of  the 
vessel  wall;  and  (c)  may  act  from  within  through  increased  blood  pressure. 
Two  of  these  may  be  in  action  at  the  same  time,  as  when  in  an  artery 
sudden  rupture  occurs  when  the  wall  has  become  weakened  through 
disease,  the  immediate  cause  being  a  sudden  increase  in  blood  pres- 
sure, through  exertion  or  other  cause;  or  again,  the  first  and  third 
may  be  involved,  as  in  the  multiple  hemorrhages  of  high  elevations, 
when  there  may  co-exist  heightened  pressure  within  the  vessels  of  certain 
areas,  together  with  great  lowering  of  the  external  pressure  upon  the 
vessels.  Mere  increase  of  blood  pressure  will  not  cause  rupture  of  the 
healthy  arteries,  or  larger  veins,  nor  again  of  the  heart,  but  only  of  the 
capillaries  and  smaller  veins;  sudden  increase  in  pressure  has  been  known 
to  lead  to  rupture  of  the  heart  when  the  muscle  is  diseased.  Thus, 
our  museum  at  McGill  contains  a  specimen  contributed  by  Dr.  Osier 
of  a  heart  exhibiting  advanced  fatty  degeneration,  in  which  rupture 
occurred  when  the  late  owner  was  walking  up  a  hilly  Montreal  street. 

Hemorrhage  per  diapedesin  occurs  in  the  capillaries  and  venules  as 
a  result  of  dilatation  of  the  same  in  conditions  of  hyperemia,  either  active 
or  passive.  The  nature  of  this  has  been  studied  more  particularly  by 
Arnold,  who  first  taught  the  existence  of  special  spaces — stigmata  and 
stomata — occurring  here  and  there  between  the  pavement  endothelial 
cells.  From  this  view  he  has  withdrawn,  and  following  his  studies  it  is 
generally  held  that  the  passage  occurs,  between  the  endothelial  cells,  it 

1  From  aifj.a)  blood,  and  the  stem,  pay,  from  p^ywfiit  to  burst  or  break. 


CAPILLARY  HEMORRHAGE  77 

is  true,  but  as  a  result  of  stretching  and  enlargement  of  the  spaces 
between  the  normally  existing  bridges  joining  the  endothelial  cells  (see 
vol.  i,  p.  33),  or,  more  probably,  with  distension  of  the  vessels  some  of 
these  bridges  break  down,  and  larger  openings  are  thus  provided,  through 
which,  under  the  blood  pressure,  the  fluid  and  corpuscles  of  the  blood 
are  forced  through  between  the  cells.  When  the  endothelium  is  already 
in  a  state  of  lowered  nutrition,  as  happens  in  passive  hyperemia,  the 
hemorrhage  is  still  more  apt  to  occur.  So,  also,  a  favoring  factor  is 
lack  of  adequate  support  to  the  capillaries.  Thus,  hemorrhages  of  this 
nature  are  peculiarly  apt  to  occur  under  conditions  of  great  vascular 
dilatation  in  regions  where  capillary  networks  exist  immediately  beneath 
a  delicate  pavement  endothelium,  e.  g.,  into  the  alveoli  of  the  lungs,  in 
conditions  of  pronounced  passive  congestion,  or  pneumonic  conditions; 
from  the  mucous  membrane  of  the  nostrils;  from  the  pleural,  pericardial, 
and  peritoneal  surfaces. 

Capillary  Hemorrhage. — Studying  the  different  forms  of  capillary 
hemorrhage,  it  has  to  be  admitted  that  time  and  again  it  is  difficult  if  not 
impossible  to  differentiate  clearly  between  cases  due  to  rhexis  and  those 
due  to  diapedesis;  there  appears,  indeed,  to  be  evidence  that  in  the  same 
area  and  under  the  same  circumstances  both  may  occur.  It  is  more 
satisfactory,  therefore,  remembering  that  both  processes  may  occur,  to 
consider  these  capillary  hemorrhages  in  the  light  of  their  causation. 
Doing  this  we  can  distinguish  several  groups,  according  as  we  deal 
with  increased  internal  pressure  and  no  previous  disease  of  the  capillary 
endothelium;  a  combination  of  increased  internal  pressure  with  morbid 
state  of  the  endothelium;  and  disease  of  the  endothelium  without  in- 
creased internal  pressure.  Examples  strictly  of  the  first  order  are  rare. 
Perhaps  the  commonest  example  is  met  with  in  the  hemorrhages  of  the 
scalp  and  membranes  of  the  brain  which  accompany  prolonged  labor, 
and  due  to  the  intense  congestion  involving  the  head  when  this  is  free 
and  the  rest  of  the  infant  still  tightly  compressed.  Occasionally  the 
small  vessels  rupture  as  the  result  of  some  vigorous  effort,  with  consequent 
great  rise  of  blood  pressure,  but  this  more  often  in  the  elderly  than  in 
the  young,  so  that  in  general  the  existence  of  some  previous  weakness 
or  disease  of  the  walls  may  be  suspected.  Very  closely  allied  are  the 
petechial  hemorrhages  of  serous  surfaces  seen  when  death  has  been 
due  to  asphyxia;  these  must  almost  certainly  be  attributed  in  the  main 
to  intense  capillary  congestion,  although  the  accompanying  extreme 
venous  state  of  the  blood  and  its  effects  upon  the  capillary  endothelium 
cannot  be  wholly  left  out  of  account.  Of  the  second  order  are  the 
capillary  hemorrhages  of  chronic  passive  congestion,  in  which  we  have 
well-marked  indications  of  malnutrition  of  the  capillary  walls.  Here 
are  to  be  included  the  hemorrhages  of  the  lungs,  kidney,  and  other  organs 
in  obstructive  heart  disease,  hemorrhagic  infarcts,  and  the  multiple 
minute  hemorrhages  which  result  from  multiple  capillary  emboli  (p. 
50).  Hemorrhages  of  a  similar  character  found  in  many  of  the  acute 
infections  are  found  to  be  associated  with  the  presence  of  hyaline  thrombi 
in  the  capillaries,  which  obviously  have  the  same  obstructive  effect, 


78  HEMORRHAGE 

arresting  the  blood  stream,  leading  to  local  stasis  at  either  side  of  the 
block,  and  producing,  in  fact,  minute  hemorrhagic  infarcts.  In  many 
of  the  cases  of  idiopathic  purpura  similar  thrombi — or  emboli — have 
been  detected,  and  this  in  the  absence  of  any  bacteria ;  in  cases  of  burns 
and  frostbite  (here  in  the  kidneys  and  stomach.  Such  are  a  possible 
explanation  of  the  ulcers  of  the  duodenum  sometimes  encountered  in 
the  former  of  these  conditions);  in  certain  cases  of  embolism,  by  body 
cells,  placental  cells,  liver  cells,  etc.;  in  leukemia  by  leukocytes.  In 
many  infections,  however,  we  have  to  deal  with  events  of  the  third  order, 
namely,  with  direct  toxic  injury  to  the  capillary  endothelium  and  with 
nothing  else.  To  such  must  be  ascribed  the  multiple  capillary  hemor- 
rhages of  the  acutest  types  of  the  acute  exanthemata — of  hemorrhagic 
smallpox,  scarlet  fever,  etc.  The  course  of  events  in  these  cases  may  be 
of  one  of  two  orders — -either  toxic,  due  to  the  specific  action  of  toxic  sub- 
stances upon  the  capillary  endothelium,  with  giving  way  of  the  same, 
or  infectious,  due  to  actual  growth  of  the  bacteria  or  infectious  agents 
within  the  endothelial  cells  and  lumen  of  the  capillaries,  destruction  of 
the  cells,  and  escape  of  the  blood.  We  meet  with  both  events — the  latter 
more  particularly  in  cases  of  bacteriemia,  in  streptococcal,  and  in  other 
terminal  infections.  As  regards  the  former,  it  is,  indeed,  doubtful  whether 
many  of  the  hemorrhages  of  infectious  cases  accompanied  by  hyaline 
capillary  thrombi  are  not  of  this  nature — whether,  that  is,  the  localized 
coagulation  of  the  blood  is  not  secondary  to  epithelial  degeneration. 
Similar  toxic  capillary  hemorrhages  may  accompany  certain  exogenous 
intoxications  (phosphorus,  mercury,  etc.),  and  these  also  must  be  attri- 
buted to  direct  action  of  the  agents  upon  the  endothelium,  and  lastly, 
the  purpura  and  hemorrhages  of  those  remarkable  conditions,  scurvy 
and  Barlow's  disease  (or  infantile  scurvy),  would  seem  to  be  due  to 
endogenous  intoxications — either  to  the  presence  in  the  blood  of  toxic 
albuminous  bodies,  the  product  of  malnutrition,  or,  on  the  other  hand, 
to  the  absence  from  the  blood  of  certain  elements  necessary  for  the  due 
nutrition  of  the  capillary  endothelium.  Both  conditions,  it  may  be 
noted,  are  seen  to  be  due  to  qualitative  deficiencies  of  the  food,  and  that 
deficiency  is  of  the  same  order  in  the  two  cases,  scurvy  being  dije  to  a  con- 
tinued diet  of  what  may  be  termed  preserved  or  dead  foods,  Barlow's 
disease  to  a  long-continued  feeding  of  infants  upon  sterilized  milk  and 
prepared  milk  products.  Both  are  recovered  from  by  giving  fresh 
food — in  the  adult,  more  particularly  fresh  vegetables;  in  the  infant, 
unsterilized  milk,  and  both  may  be  warded  off  by  the  administration  of 
small  amounts  of  vegetable  acids,  lemon  juice,  etc. 

It  is  the  custom  to  refer  to  hemorrhages  in  different  regions  by  different 
names.  Thus,  punctate  capillary  hemorrhages  are  known  as  petechise 
or  ecchymoses;  more  diffuse  subcutaneous  or  interstitial  hemorrhages 
as  suggillations ;  cerebral  hemorrhage  as  apoplexy;1  escape  of  blood  from 

1  This  term  more  accurately  denotes  the  clinical  symptoms  that  follow  such  hemor- 
rhage, the  stroke  or  loss  of  consciousness  and  power  (cnronM/aou,  active,  to  strike  to 
earth;  passive,  to  be  struck  or  to  lose  one's  senses).  It  is  thus  utterly  incorrect  to 
refer  to  pulmonary  hemorrhage  as  pulmonary  apoplexy 


LOCAL  EFFECTS  79 

the  stomach  as  hematemesis,  from  the  lungs  as  hemoptysis,  from  the  nose 
as  epistaxis,  from  the  urinary  channels  as  hematuria,  from  the  uterus 
as  menorrhagia  and  metrorrhagia,  from  the  sweat  glands  as  hemati- 
drosis.  Accumulation  of  blood  in  the  various  spaces  of  the  body  is 
known  as  hemopericardium,  hematothorax,  hematocele.  This  last  term 
is  by  prescription  more  accurately  confined  to  accumulation  in  the  tunica 
vaginalis  testis,  but  is  also  employed  for  limited  accumulations  of 
extravasated  blood  elsewhere.  When  the  blood  forms  a  cyst-like  accu- 
mulation in  the  subcutaneous  tissues,  the  term  hematoma  is  not  infre- 
quently employed.  Purpura  is  the  name  given  to  relatively  small  multiple 
cutaneous  hemorrhages  not  due  to  injury,  but  to  various  diseased  states; 
melena,1  to  blood  which,  escaping  into  the  stomach  or  intestines,  is  so 
changed  by  the  action  of  the  digestive  juices  as  to  be  discharged  in  a 
black  condition.  The  localized  infiltration  of  tissue  with  blood  forming 
a  hemorrhagic  infarct  we  have  already  noted. 

Local  Effects. — As  the  result  of  rupture  close  to  one  of  the  surfaces 
of  the  body,  or  of  trauma,  whereby  deeper  lying  vessels  are  exposed  to 
the  surface,  the  main  effect  is  escape  of  blood  and  complete  loss  of  the 
same  to  the  economy.  According  to  (1)  the  nature  of  the  ruptured  vessel, 
(2)  the  extent  of  the  rupture,  and  (3)  the  duration  of  the  escape  of  blood, 
so  does  this  escape  tell  (a)  upon  the  nutrition  of  the  region  supplied  by 
or  supplying  blood  to  the  ruptured  vessel,  and  (&)  upon  the  system  at 
large.  Very  similar  results  follow  rupture  of  vessels  into  one  of  the  body 
cavities,  though  here,  in  addition,  the  changes  taking  place  in  the 
extravasated  blood  become  a  feature.  Even  in  the  first  order  of  events, 
the  blood  coming  into  contact  with  tissues  other  than  the  normal  endo- 
thelial  lining  of  the  vessels  tends  to  undergo  coagulation.  This  coagu- 
lation, with  the  progressive  lowering  of  the  blood  pressure  and  diminution 
of  rate  of  flow,  with  the  contraction  of  the  ruptured  vessels  as  the  internal 
pressure  is  removed,  and  in  the  case  of  arteries  cut  transversely,  with  the 
retraction  and  curling  up  of  the  middle  coat,  all  together  tend  to  bring 
the  hemorrhage  to  a  natural  termination.  Whether  this  natural  arrest 
is  accomplished  or  not,  depends  upon  several  factors — the  nature  of  the 
ruptured  vessel  or  vessels;  the  direction  of  the  rupture  (e.  g.,  in  arteries, 
rupture  in  the  direction  of  the  longitudinal  axis  cannot  undergo  closure 
by  retraction,  whereas  this  can  take  place  when  the  rupture  is  transverse); 
the  force  of  the  heart  beat  (/.  e.,  where  the  circulation  is  powerful  there 
may  be  so  rapid  a  loss  of  blood  that  death  ensues  before  the  natural 
arrest  can  come  into  play);  and  again  the  state  of  the  blood,  since,  as 
will  be  discovered  later  (p.  85),  the  coagulating  power  of  the  blood 
varies  considerably.  Blood  extravasated  into  the  cavities  of  the  body, 
into  the  pericardial  or  pleural  cavities,  for  instance,  if  not  discharged 
in  sufficient  quantities  to  lead  to  death,  may  remain  fluid  for  some  little 
time,  and  in  this  fluid  state  may  undergo  some  reabsorption  through  the 
lymphatics,  as  regards  both  its  fluid  and  its  corpuscles.  The  tendency, 
however,  is  to  undergo  coagulation,  and,  following  upon  this,  a  slower 

1  From  //eAaf,  fern.,  //tvlamz,  black. 


80  HEMORRHAGE 

process  of  absorption  under  the  combined  action  of  autolysis  (vol.  i, 
p.  337)  and  leukocytic  action. 

Where  we  deal  with  suffusion,  infiltration  of  the  tissues,  or  hematoma 
formation,  there  we  have  to  recognize  both  local  changes  in  the  tissue 
involved,  and  changes  in  the  extravasated  blood.  Not  only  does  the 
diversion  of  the  blood  from  the  normal  channels  bring  about  malnutrition 
of  the  area  involved,  but  the  presence  of  the  blood  under  pressure  in  that 
area  compresses  the  capillaries  of  the  same,  and  arrests  the  circulation 
within  them;  while,  thirdly,  the  force  with  which  the  blood  escapes  may 
lead  to  extensive  laceration  and  destruction  of  the  tissue.  This  last  is 
particularly  well  seen  in  organs  of  a  soft  consistence,  in  cases,  for  example, 
of  cerebral  hemorrhage.  Where  the  infiltrations  are  general  and  local, 
as  in  petechise,  purpura,  etc.,  and  due  to  diapedesis  rather  than  rupture, 
the  local  effects  may  be  of  the  slightest  order;  there  may  be  relatively 
rapid  absorption  with  little  tissue  injury  (although  even  here  there  is 
local  deposit  of  pigment  from  disintegration  of  the  escaped  erythrocytes, 
which  pigment  undergoes  slow  removal,  so  that  for  long,  cutaneous 
petechise  leave  behind  them  small  brownish  flecks).  Where  more  exten- 
sive, we  recognize  a  series  of  changes,  of  which  the  more  important  are: 
(1)  Escape  of  hemoglobin  from  the  extravasated  erythrocytes,  causing 
a  hemoglobin  imbibition  which  is  accompanied  by  increased  venosity 
and  darkening  of  the  suffused  blood;  (2)  disintegration  of  the  escaped 
hemoglobin,  with  production  of  hemosiderin,  and,  it  may  be,  in  more 
central  parts  of  the  infiltrated  area,  of  hematoidin  also  (vol.  i,  p.  882). 
The  successive  stages  in  the  disintegration  of  the  hemoglobin  cause  the 
succession  of  vivid  tints  seen  in  the  not  unfamiliar  "black  eye."  (3) 
Progressive  absorption  of  the  extravasated  blood  and  its  modified  con- 
stituents. Again,  it  has  to  be  observed  that  the  extent  of  this  absorption 
depends  upon  the  extent  and  nature  of  the  extravasation.  In  cases  of 
even  extensive  subcutaneous  suffusion  it  may  be  complete;  in  cases  of 
hematoma-like  accumulations  it  may  be  incomplete  and  accompanied 
by  formation  of  granulation  tissue  replacing  the  destroyed  tissue.  The 
result  may  be  either  the  formation  of  a  firm  fibrous  cicatrix,  or  of  a 
cyst  with  fibrous  wall  and  fluid  contents  (vol.  i,  p.  795),  whose  contents 
at  an  early  stage  are  deeply  pigmented,  but  eventually  through  diffusion 
and  leukocytic  action  become  a  clear  colorless  serum. 

General  Effects. — According  to  the  extent  and  rate  of  the  hemor- 
rhage so  may  we  observe: 

1.  Sudden  death  within  a  minute  or  two,  as  after  rupture  of  the 
heart  or  bursting  of  a  thoracic  aneurism  into  the  pleural  cavity,  trachea, 
or  oesophagus. 

2.  Death  preceded  by  collapse  and  all  the  symptoms  of  grave  cerebral 
anemia. 

3.  Collapse  followed  by  hydremia  and  eventual  recovery. 

4.  Syncope,  or  temporary  cerebral  anemia  with  rapid  recovery. 

5.  No  disturbances  due  to  cerebral  anemia,  but  (in  cases  of  internal 
extravasation)  the  development  of  a  febrile  state  due  to  diffusion  of 
disintegration  products  from  the  extravasated  blood  (vol.  i,  p.  445). 


HEMOPHILIA  81 

0.  In  cases  of  multiple  repeated  hemorrhages  of  moderate  grade  there 
may  be  eventual  exhaustion  of  the  hematopoietic  tissues,  and  the  develop- 
ment of  a  condition  of  the  blood  resembling  that  seen  in  true  pernicious 
anemia,  with  poikilocytosis,  presence  of  normoblasts,  etc.,  but,  unlike 
that  condition,  showing  in  the  liver  and  other  organs  no  indications  of 
the  results  of  excessive  disintegration  of  the  erythrocytes. 

We  have  in  our  first  book  and  in  other  chapters  of  this  discussed  most 
of  these  conditions.  Here,  therefore,  they  need  but  be  called  to  mind. 
At  most,  it  is  necessary  to  add,  as  giving  the  basis  of  a  scale  for  deter- 
mining the  incidence  of  sundry  of  these  sequelae,  that  according  to  the 
generally  accepted  estimate,  the  organism  can  withstand  the  loss  of  about 
3  per  cent,  of  the  body  weight  without  death  ensuing,  and  that  when 
"  bleeding"  was  in  vogue  it  was  not  unusual  to  remove  thirty  ounces 
from  healthy  adults  without  the  supervention  of  any  grave  effects. 

Hemophilia. — In  this  relationship  reference  must  be  made  to  the 
very  remarkable  condition  of  the  hemorrhagic  diathesis  or  hemophilia, 
a  condition  conveyed  from  one  generation  to  another  in  a  very  striking 
manner  (see  the  genealogical  table  given  in  our  first  volume,  facing 
p.  144),  and  one  in  which  the  slightest  trauma,  e.  g.,  slight  bruising  or 
contusion,  or  some  insignificant  surgical  operation,  such  as  the  removal 
of  a  tooth,  is  apt  to  be  followed  by  almost  intractable  bleeding,  lasting 
for  days.  It  is  still  undetermined  what  is  the  essential  cause  of  this 
condition.  Virchow,  it  is  true,  observed  a  characteristic  smallness  and 
thinness  of  the  walls  of  the  aorta  in  these  cases,  and  supposed  an  incom- 
plete development  or  abnormal  thinness  of  the  vessels ;  but  that  has  never 
been  absolutely  proved.  What  .would  seem  more  definite  is  a  lowered 
power  of  coagulation  of  the  blood.  Just  as  in  Barlow's  disease  and  in 
scurvy,  we  see  nowadays  an  explanation  of  the  multiple  hemorrhages 
in  the  presence  in  or  absence  from  the  blood  of  some  element,  owing  to 
defective  nutrition,  so  the  tendency  favored  by  the  recent  abundant 
studies  upon  immunity  and  cytolysis  is  to  regard  hemophilia  as  due  to 
some  inherited  deficient  reaction  between  the  blood  and  the  endothelium 
of  the  smaller  vessels,  leading,  it  may  be,  either  to  a  state  of  weakness  of 
that  endothelium  or  to  excessive  development  of  bodies  of  the  nature 
of  antithrombin.  The  injection  of  horse  serum  has  recently  been  noted 
to  be  followed  by  arrest  of  hemorrhage  in  these  cases. 

There  is  yet  another  form  of  capillary  hemorrhage  that  must  not  be 
overlooked;  we  refer  to  the  nervous.  Examples  of  this  are  seen  in  certain 
cases  of  hysteria  in  which  apparently  almost,  at  will,  the  individual 
develops  hemorrhagic  suffusion  of  the  skin  or  mucous  membranes; 
closely  allied  to  this  would  seem  to  be  the  "stigmata"  produced  upon 
the  hands  and  feet  in  cases  of  extreme  religious  exaltation  or  ecstasy, 
and  other  cases  of  bloody  sweat  or  hematidrosis.  Lubarsch  would 
include  here  the  hemorrhage  of  menstruation,  pointing  out  that  it  is 
associated  with  definite  nervous  phenomena.  It  is  a  matter  of  present 
debate  whether  the  menstrual  flow  is  or  is  not  intimately  associated  with 
the  development  of  an  internal  secretion  from  the  ovary,  or  again  is 
preceded  by  degenerative  changes  in  the  endothelium  of  the  submucous 
6 


82  HEMORRHAGE 

vessels  of  the  endometrium.  While  admitting  the  very  possible  presence 
of  a  nervous  factor,  we  are  inclined  to  doubt  whether  this  be  the  control- 
ling agent.  In  the  other  uncomplicated  cases  the  course  of  events  is 
problematical;  the  suddenness  with  which  the  hemorrhages  may  be  in- 
duced by  hysterical  patients  would  seem  to  militate  against  local  trophic 
changes;  on  the  other  hand,  it  is  difficult  to  ascribe  the  escape  of  blood 
purely  to  local  action  of  the  arterial  vasodilators. 


CHAPTER  IV. 

THE  BLOOD— QUALITATIVE  BLOOD  CHANGES. 

THE  study  of  the  qualitative  changes  in  the  blood  has  now  become 
so  special  and  so  specialized  a  branch  of  pathology,  with  text-books  and 
journals  devoted  to  it,  that  to  enter  into  the  subject  in  all  its  modern 
detail  would  in  itself  demand  a  volume  of  fair  size.  Under  the  circum- 
stances, the  sense  of  proportion  demands  that  we  lay  down  at  most  the 
main  outlines,  at  the  same  time  emphasizing  the  fact  that  these  are  but 
the  outlines,  and  that  for  a  fuller  knowledge  the  student  must  master 
such  works  as  Swing's  Clinical  Pathology  of  the  Blood  or  Cabot's  Clini- 
cal Examination  of  the  Blood,  arid  for  the  latest  developments  must 
consult  that  excellent  journal,  the  Folia  Hematologica. 

In  such  a  rapid  survey  we  have  to  take  into  account,  first,  modifications 
in  the  different  constituents — in  the  constitution  of  the  plasma,  and  in 
the  relative  proportions  of  the  corpuscular  elements,  erythrocytes,  leuko- 
cytes, and  blood  platelets,  and  then  pass  in  review  certain  of  the  more 
important  blood  dyscrasias  as  separate  entities  (chlorosis,  secondary 
anemias,  pernicious  anemia,  etc.). 

THE  PLASMA. 

It  is  no  false  humility  to  state  that  despite  the  amount  of  research 
that  has  been  devoted  to  the  blood  during  late  years,  we  are  but  at  the 
beginning  of  a  knowledge  of  its  pathology.  We  have  gained  some 
knowledge  regarding  the  red  corpuscles  and  their  function,  and  the 
significance  of  disturbances  affecting  the  same;  our  knowledge  of  the 
white  corpuscles,  their  mode  of  origin,  relationships,  functions,  and 
morbid  states  has  been  materially  widened,  though  much  has  yet  to  be 
determined;  but  as  regards  the  plasma,  the  main  medium  of  nourish- 
ment and  interchange,  the  data  we  possess  are  painfully  deficient.  That 
plasma  is  the  great  medium  of  interchange;  from  it  are  constantly  being 
abstracted  materials  needed  for  the  elaboration  of  the  different  tissues; 
into  it  are  poured  many  products  of  cell  activities,  internal  secretions, 
enzymes,  hormones,  etc.  The  evidence  is  very  clear  that  despite  this 
constant  change,  the  composition  remains  in  health  remarkably  con- 
stant. We  are  apt  to  attribute  to  the  liver  and  kidneys  the  main  function 
of  removing  the  deleterious  substances  that  would  otherwise  tend  to 
accumulate — and  here,  it  may  be,  we  are  correct,  although  we  must  take 
into  account  how  other  organs,  like  the  ductless  glands,  evidently  play 
a  part  in  neutralizing  certain  bodies  of  a  toxic  type  circulating  within  it. 


84  THE  BLOOD 

But  the  study  of  immunity  has  revealed  the  existence  of  substances 
in  the  plasma  in  minute  amounts — enzymes  and  proteins — possessing 
properties  obviously  of  extraordinary  importance  for  the  general  well- 
being  of  the  organism  as  a  whole;  we  cannot  isolate  these,  we  can  only 
conclude  that  they  are  of  proteid  nature;  we  cannot  surely  state  what 
is  their  origin — we  repeat  glibly  that  the  solid  matters  of  the  blood  serum 
constitute  9.2  per  cent.,  and  of  this  7.6  per  cent,  is  protein,  in  the  main 
serum  albumin  and  serum  globulin.  But  having  said  this,  we  are  igno- 
rant of  the  exact  source  of  these  obviously  most  important  constituents, 
of  the  respective  parts  played  by  the  two  in  nutrition,  and  consequently 
of  the  significance  of  disturbances  in  their  relative  proportions.  We  are 
only  slowly  realizing  that  those  names,  serum  albumin  and  serum  globu- 
lin, cover  not  single  entities,  but  groups  of  substances.  And  if  this  is 
true  of  the  most  abundant  as  well  as  the  most  sparse  constituents  of  the 
plasma,  the  poverty  of  our  real  knowledge  stands  revealed. 

Briefly  we  may  divide  the  constituents  of  the  plasma  into  water, 
proteins,  and  salts.  The  proportion  of  water  maintains  in  health  a 
remarkable  constancy,  so  that  the  specific  gravity  varies  but  slightly. 
In  disease  greater  variations  show  themselves,  and  the  condition  of 
hydremia  is  not  infrequent.  But  here  it  has  to  be  noted  that  a  hydremic 
condition  of  the  blood  may  be  brought  about  (1)  by  actual  increase  in 
the  amount  of  blood  by  increase  in  its  watery  content  (true  hydremic 
plethora),  as  in  obstructive  heart  disease;  (2)  by  no  increase  in  the  total 
amount  of  blood,  but  actual  deficiency  of  proteins  of  the  plasma,  as  after 
severe  hemorrhage;  and  (3)  by  increase  in  the  salts  of  the  blood,  attract- 
ing more  fluid  out  of  the  tissues  in  order  to  preserve  the  normal  "tone" 
of  the  plasma.  As  already  pointed  out,  this  retention  of  salts  is  by  some 
held  to  explain  the  hydremia  of  nephritis.  Not  until  we  possess  a  fuller 
series  of  simultaneous  observations  on  the  total  amount  of  blood  (by 
Haldane  and  Smith's  or  other  method)  and  of  the  proportions  of  the 
different  main  constituents  of  the  blood  shall  we  be  able  to  speak  with 
decision  about  these  matters.  In  short,  with  our  present  lack  of  knowl- 
edge, it  is  difficult  to  do  other  than  consider  together  those  three  possible 
variants,  water,  proteins,  and  salts.  A  more  watery  condition  of  the 
blood,  however  brought  about,  is  encountered  in  obstructive  heart  and 
lung  and  in  kidney  diseases,  in  severe  infections  and  malignancy,  and 
after  extensive  hemorrhages.  It  must  not  be  thought  that  in  every  case 
manifesting  these  particular  morbid  states,  the  condition  of  hydremia 
necessarily  manifests  itself.  In  by  no  means  all  cases  of  cancer  or 
sarcoma  is  there  reduction  of  the  circulating  proteins,  and  as  Grawitz1 
has  shown,  in  the  different  stages  of  tuberculosis  marked  variations  are 
to  be  made  out  in  the  composition  of  the  blood  serum;  not  infrequently, 
instead  of  being  hydremic,  the  blood  is  found  more  concentrated  and 
viscid.  In  conditions  of  nephritis  it  is  more  particularly  the  acute  paren- 
chymatous  cases  that  exhibit  hydremia  (which  it  is  to  be  noted  is  asso- 
ciated with  extensive  loss  of  albumin  through  the  kidneys) ;  it  may  be 

1  Deutsch.  med.  Woch.,  19: 1893:1347. 


THE  PLASMA  85 

wholly  absent  in  cases  of  chronic  interstitial  nephritis,  and  is  not  neces- 
sarily present  in  those  of  chronic  parenchymatous  nephritis.1  And 
as  regards  obstructive  heart  and  lung  disease,  a  word  of  caution  needs 
to  be  given.  Where  there  is  passive  congestion  there  is  coincidently  an 
increased  passage  of  fluid  from  the  blood  into  the  lymphatics,  with,  as 
a  result,  a  marked  concentration  of  the  ery throcytes  in  the  capillaries 
from  which  the  exudation  takes  place.  In  these  cases,  as,  indeed,  in  all, 
it  is  wrong  to  make  any  conclusions  as  to  the  quantity  or  quality  of  the 
plasma  from  an  estimation  of  the  number  of  erythrocytes  per  cubic 
millimeter.  So  also  in  this  same  series  of  cases  the  fact  of  this  escape  of 
fluid  into  the  tissues  has  to  be  taken  into  account.  By  the  ordinary 
means  of  piqure  to  gain  capillary  blood,  that  blood  may  be  gained  exten- 
sively diluted  with  oedema  fluid.  This,  however,  has  been  definitely 
determined,  that  the  blood  serum  deprived  of  corpuscles  obtained  from 
those  cases  of  cardiac  insufficiency  in  the  stage  of  imperfect  compensa- 
tion presents  a  recognizable  reduction  in  its  solids. 

Of  the  proteins  of  the  plasma  as  contrasted  with  the  serum,  fibrinogen 
must  not  be  overlooked.  To  the  modern  views  regarding  this  protein 
and  its  relationship  to  the  coagulation  process  we  have  already  referred 
(p.  60).  Here  it  has  to  be  noted  that  the  amount  exhibits  very 
considerable  variation,  as  evidenced  by  the  varying  quantity  of  fibrin 
obtained  from  different  bloods.  From  the  normal  amount  of  0.1  to 
0.4  per  cent,  by  weight  there  may  be  a  rise  to  1  per  cent,  and  higher. 
Such  hyperinosis  is  met  with  more  particularly  in  certain  infections, 
notably  in  acute  lobar  pneumonia,  in  acute  rheumatism,  and  in  some  cases 
of  acute  pleurisy.  In  other  infections,  notably  typhoid,  there  is  found 
a  condition  of  hypinosis,  or  reduction  of  the  fibrin.  The  fact  that  hyperi- 
nosis and  hypinosis  in  these  febrile  states  show  a  remarkable  parallelism 
with  the  presence  or  absence  of  leukocytosis  suggests  that  it  is  not  so 
much  the  fibrinogen  as  the  fibrin  ferment  that  undergoes  variation. 
This,  however,  would  not  seem  to  be  the  case.  The  fibrinogen  itself 
undergoes  variations  independently  of  the  leukocytosis  in  leukemia2  and 
in  phosphorus  poisoning,3  where  it  may  be  completely  absent.  Varia- 
tions in  the  amount  of  the  ferment  affect  the  coagulation  time  of  the 
blood  rather  than  (so  long  as  any  fibrin  ferment  is  present)  the  amount 
of  fibrin  produced. 

As  regards  the  salts  of  the  plasma,  again  it  has  to  be  noted  that  we 
possess  scattered  data4  rather  than  a  full  knowledge  of  their  variation 
in  different  morbid  states.  These  salts  consist  in  the  main  of  sodium 
salts,  of  chlorides  and  phosphates.  They  evidently  bear  an  intimate 
relationship  to  the  state  of  solution  of  the  proteins.  Their  nature  is 

1  Hammerschlag,  Ztsch.  f.  klin.  Med.,  21 : 1892:  475. 

2  Pfeiffer,  Th.  Zentrbl.  f.  innere  Med.,  25:1904:809. 

3  Jakoby,  Zeitschr.  f.  phys.  Chemie,  30:1900:174. 

4  These  data  will  be  found  collected  and  discussed  in  Hamburger,  Osmotische 
Druck  und  lonenlehre,    1906,  and  Limbeck,  Pathologie  des  Blutes,  2d  edition, 
Jena,  1896. 


86  THE  ERYTHROCYTES 

such  that  the  normal  blood  is  definitely  alkaline,  and,  as  already  pointed 
out  in  discussing  acidosis  (vol.  i,  p.  348),  reduction  in  this  alkalinity  is 
associated  with  the  gravest  metabolic  disturbances. 

Lipemia. — One  other  constituent  of  the  blood  plasma  deserves 
notice.  There  is  always  to  be  isolated  from  the  fluid  of  normal  blood 
a  minute  quantity  of  fat.  As  to  the  exact  state  in  which  this  is  present 
there  is  still  debate,  the  evidence  on  the  whole  favoring  the  view  that 
some  at  least  is  in  the  form  of  the  more  soluble  salts  of  the  fatty  acids 
or  soaps.  What  we  have  already  said  regarding  the  absorption  of  fats 
from  the  intestine  (vol.  i,  p.  79)  prepares  us  to  find  that  after  meals 
rich  in  fats  the  amount  is  definitely  increased.  Occasionally  there  is 
encountered  a  condition  of  lipemia — of  extraordinary  increase  in  this 
fat.  In  some  cases  of  diabetes  there  may  be  as  high  a  content  as  20 
per  cent.,  the  blood  taking  on  a  milky  appearance,  and  the  fat  being 
present  in  an  emulsified  state  in  the  form  of  fine  globules.  Once  more 
it  has  to  be  acknowledged  that  we  know  little  or  nothing  about  the  con- 
ditions leading  to  this  lipemia — whether  we  deal  with  the  absence  of  a 
lipolytic  ferment  either  from  the  blood  or  from  the  tissue  cells  which 
normally  absorb  the  fat,  or  again,  of  some  constituent  which  converts 
the  absorbed  fat  into  a  soluble  salt,  thereby  preparing  it  to  be  taken 
up  by  the  cells,  or  lastly  to  the  presence  of  acids  splitting  up  the  soluble 
soaps  present  in  the  blood  and  liberating  thus  the  fatty  acids  and  fats. 
In  addition  to  diabetes,  lipemia  has  been  observed  in  conditions  charac- 
terized by  defective  oxidation  (and  increased  carbon  dioxide  content  of 
the  blood),  such  as  pneumonia,  phosphorus  poisoning,  and  anemias. 


THE  ERYTHROCYTES. 

Of  the  physiology  of  the  red  corpuscles,  much  may  be  said;  regarding 
their  pathology,  we  must  be  comparatively  brief. 

Variations  in  Number. — We  have  emphasized  already  that  moderate 
increase  or  decrease  in  the  number  of  erythrocytes  per  cubic  millimeter  in 
itself  tells  us  little  without,  at  the  same  time,  there  be  present  indications 
of  either  increased  production  or  excessive  destruction  of  the  corpuscles, 
rather  than  variation  in  the  amount  of  the  fluid  of  the  blood.  Where 
there  is  great  departure  from  the  normal  it  is  difficult  to  believe  that  in 
all  cases  the  concentration  or  dilution  of  the  blood  alone  is  at  fault. 
That  it  may  be,  is  shown  by  the  great  apparent  increase  in  the  number 
of  red  corpuscles  seen  in  cases  of  cholera  nostras,  cholera  asiatica,  and 
other  conditions  in  which  there  is  great  drain  of  fluid  from  the  intestinal 
canal.  But  in  such  cases  there  is  abundant  clinical  evidence  of  loss  of 
fluid,  as,  for  example,  the  difficulty  in  obtaining  blood  from  the  finger 
or  pinna  of  the  ear,  the  feeble  heart  action,  the  tarry  condition  of  the 
blood  itself — all  indicating  reduction  in  the  quantity  of  the  blood  and 
concentration  of  the  more  solid  constituents  of  the  same. 

Polycythemia. — The  polycythemia  of  high  altitudes  has  been  studied 
by  a  large  number  of  observers  (Paul  Bert,  Miescher,  Mosso,  Abder- 


POLYCYTHEM1A  87 

halden,  and  others),  and  is  very  striking.  It  affects  all  vertebrates. 
Thus,  in  the  South  American  Cordilleras,  at  a  height  of  more  than  12,000 
feet,  Viault1  found  that  the  count  in  the  llama  was  as  high  as  16,000,000. 
There  has  been  great  debate  as  to  its  meaning,  but  this  seems  to  be 
established:  (1)  That  the  total  amount  of  blood  remains  unaltered, 
and  thus  the  increase  is  not  due  to  concentration;  (2)  that  the  arterial 
blood  shows  the  increase,  and  thus  we  do  not  deal  with  concentration 
in  the  superficial  capillaries ;  (3)  that  it  supervenes  with  relative  rapidity 
(Gaule  records  increase  to  8,500,000  in  a  balloon  ascension);  (4)  that 
it  soon  disappears  upon  descent  toward  the  sea  level. 

Making  due  allowance  for  variations  in  individual  reaction,  it  is  diffi- 
cult to  regard  the  increase  as  other  than  adaptive,  than  a  reaction  to 
the  need  for  more  oxygen  carriers  in  an  atmosphere  in  which  the  amount 
of  oxygen  is  diminished.  A  similar  increase,  it  may  be  noted,  has  been 
observed  by  Nasmith  and  Graham2  in  animals  made  to  breathe  air 
containing  carbon  monoxide.  That  carbon  monoxide  is  useless  to  the 
economy,  but  has  a  far  greater  affinity  for  hemoglobin  than  has  oxygen; 
the  organism  evidently  produces  more  hemoglobin  and  hemoglobin 
holders  to  counteract  the  using  up  of  part  of  the  circulating  hemoglobin. 
We  do  not  pretend  that  there  are  not  facts  in  connection  with  the  phe- 
nomenon that  are  difficult  to  explain.  When  the  condition  supervenes 
rapidly  we  have  to  postulate  a  rapid  production  of  hemoglobin  and 
rapid  discharge  of  young  erythrocytes  from  the  bone  marrow,  and  so 
far  only  one  observer,  Gaule,  has  recorded  the  presence  of  (immature) 
nucleated  red  corpuscles  in  the  circulating  blood;  others  have  failed 
to  find  them.  So  also  what  becomes  of  the  excess  of  erythrocytes  upon 
return  to  lower  levels  remains  unsolved.  These  are  matters  regarding 
which  we  have  incomplete  knowledge,  rather  than  facts  absolutely 
opposed  to  the  view  here  taken.3  This  may  be  spoken  of  as  physio- 
logical polycy themia ;  pathological  polycythemia  is  a  condition  which 
has  come  into  recognition  only  during  the  last  few  years,4  some  fifty 
cases  being  on  record.  In  these  cases  counts  of  8,000,000  are  common, 
and  those  of  10,000,000,  13,000,000,  and  even  14,000,000  corpuscles  have 
been  determined.  There  is  an  accompanying  duskiness  or  cyanotic 
appearance  of  the  skin,  and  frequent  (though  not  constant)  pronounced 
enlargement  of  the  spleen.  Cases  have  been  reported  of  all  ages,  most 
frequently  in  advancing  life.  The  presence  of  normoblasts  and  megalo- 
blasts  show  clearly  that  there  is  an  active  production  of  erythrocytes, 
and  some  of  the  few  postmortems  have  demonstrated  increase  of  the 

1  Compt.  rend,  de  1'Acad.  des  Sci.,  112: 1891 :  295. 

2  Journ.  of  Physiol.,  35:1906:32. 

3  A  thoughtful  study  of  this  problem  is  afforded  by  Krehl,  Pathol.  Physiologic, 
5th  German  edition,   Leipzig,   1907:  193.      See  also  A.  W.  Hewlett's  translation, 
Lippincott,  1907. 

4  The  first  case  was  recorded  by  Rendu  and  Widal  in  1892.     For  bibliography, 
see  Engelbach  and  Brown,  Jour,  of  Amer.  Med.  Assoc.,  October  20,  1906,  and  for 
a  general  description,  see  Cabot,  Osier  and  McCrae's  System  of  Medicine,  4 : 1908 :  678. 


88  THE  ERYTHROCYTES 

marrow,  and  one  at  least  has  confirmed  Widal's  view  that  the  splenic 
enlargement  is  due  to  resumption  by  the  spleen  of  its  foetal  properties 
of  production  of  erythroblasts.  With  this  there  is  in  some  cases  a  moder- 
ate grade  of  neutrophilic  leukocytosis.  The  cause  of  the  condition  is 
wholly  undetermined. 

Variations  in  Size  and  Shape. — The  normal  erythrocyte  is  from  5  to 
8.5  fj.  in  diameter.  In  conditions  of  anemia  and  disturbed  erythrocyte 
production,  notably  in  pernicious  anemia,  cells  10  to  20  /*  in  diameter 
are  encountered  (megalocytes)  along  with  others  which  may  be  but 
2  to  3  y.  in  diameter  (microcytes).  In  these  cases  of  extreme  anemia 
cells  of  very  irregular  form  are  also  encountered — poikilocytes,  pear- 
shaped,  elongated,  and  sausage-shaped,  etc. 

Alterations  in  Structure  and  Staining  Reactions. — The  normal  ery- 
throcyte is  homogeneous,  its  hemoglobin  scattered  evenly  through  it,  and 
granules  are  absent  when  the  ordinary  processes  of  staining  are  employed. 
In  a  mixture  of  acid  and  basic  aniline  dyes  (as  in  Roman owsky's  stain 
and  its  many  modifications)  it  takes  up  the  acid  dye. 

If,  however,  the  development  be  followed  in  the  red  marrow,  a  suc- 
cession of  stages  may  be  made  out,  from  the  large  erythroblast,  with 
large,  loosely  skeined  nucleus  and  without  hemoglobin  in  its  cytoplasm, 
through  forms  with  scattered  granules  and  masses  of  hemoglobin  and 
small,  condensed  nucleus,  the  hemoglobin  in  these  younger  cells  taking 
up  the  basic  stain  with  more  or  less  intensity  and  appearing  purplish 
or  even  blue,  to  forms  which  have  lost  their  nucleus  and  have  the  hemo- 
globin diffused  evenly  throughout  them,  but  still  tend  to  take  a  purplish 
rather  than  a  reddish  or  orange  color.  These  various  immature  forms 
of  red  corpuscles  may  be  encountered  in  disease,  and  then  indicate  a 
pouring  into  the  blood  from  marrow  or  spleen  of  imperfectly  developed 
erythrocytes;  indicate,  that  is,  a  condition  of  such  stimulation  of  the 
hematopoietic  system  that  with  active  proliferation  of  the  erythroblasts 
the  immature  cells  are  discharged  into  the  blood. 

Polychromatophilia  and  the  presence  of  nucleated  red  corpuscles,  of 
megaloblasts  (as  the  large,  paler  forms  are  termed),  and  of  normoblasts 
(hemoglobin-holding  cells  of  the  normal  size  but  nucleated)  must  then 
be  regarded  as  evidence  of  excessive  demand  made  upon  the  blood- 
producing  tissues.  Such  conditions  are  most  frequently  seen  in  grave 
anemias.  At  the  same  time  it  has  to  be  recognized  that  basic  and  irregu- 
lar staining  of  the  erythrocytes  in  another  but  widely  different  series  of 
conditions  is  evidence  not  of  regeneration,  but  of  degeneration ;  in  areas 
of  interstitial  hemorrhage,  as  again  in  areas  of  fresh  thrombosis,  such 
polychromatophilia  is  to  be  distinguished. 

In  the  latter  case  it  is  associated  with  indications  of  disintegration.  Of 
this,  two  forms  have  been  distinguished  by  Arnold,  namely,  Plasmorrhexis 
and  Plasmoschisis.  The  former  is  the  condition  often  observed  when 
erythrocytes  are  studied  in  film  under  the  microscope,  namely,  the  devel- 
opment of  crenation  and  of  a  mulberry-like  appearance  with  formation 
of  peripheral  globules  of  varying  size,  some  very  minute  (resembling 
Miiller's  "dust  bodies"),  others  it  may  be  as  large  as  blood  platelets; 
and  these  become  liberated,  with  progressive  diminution  in  size  of  the 


VARIATION  IN  AMOUNT  OF  HEMOGLOBIN  89 

parent  corpuscle.  In  plasmoschisis  what  is  observed  is  a  rapid  breaking 
up  of  the  whole  body  of  the  corpuscle  into  small  globules,  which  as 
they  separate  are  seen  to  be  free  from  hemoglobin  and  to  be  indistinguish- 
able from  blood  platelets.  Another  modification  of  which  the  signifi- 
cance is  not  understood  is  seen  in  certain  cases  of  lead  poisoning  in 
which  ring-like  accumulations  of  matter  taking  on  the  basic  stain  are 
seen  within  occasional  erythrocytes.  Since  in  this  condition  we  are  apt 
to  meet  with  "stippling"  or  free  granules  of  basic  staining  matter  within 
the  red  corpuscles,  with  normoblasts  and  megaloblasts,  the  indications  are 
that  we  deal  with  an  anemia  and  accompanying  overstimulation  of  the 
red  marrow;  and  the  suggestion  is  that  here  we  deal  with  cells  in  which 
the  conversion  of  the  prehemoglobinic  matter  is  not  complete. 

Variation  in  Amount  of  Hemoglobin;  Hemolysis. — By  the  use  of  the 
hemoglobinometer  we  can  determine  the  amount  of  hemoglobin  in  a 
given  quantity  of  blood  by  comparing  its  color  with  that  of  a  sample  of 
normal  blood  of  known  dilution  (or  its  colored  equivalent).  By  the 
hemocytometer  we  can  determine  the  number  of  erythrocytes  per  cubic 
millimeter  of  the  same  blood.  Utilizing  these  two  enumerations,  we  can 
arrive  at  the  color  index,  e.  g.,  at  the  ratio  of  hemoglobin  per  corpuscle, 
taking  the  normal  corpuscle  with  normal  hemoglobin  content  as  1.  A 
blood,  for  example,  containing  the  normal  5,000,000  corpuscles,  but  only 
75  per  cent,  of  the  normal  hemoglobin,  would  have  a  color  index  of  0.75; 
having  only  2,500,000,  with  a  hemoglobin  content  of  75  per  cent.,  would, 
on  the  other  hand,  have  a  color  index  of  1.5,  i.  e.,  each  corpuscle  would 
possess  half  as  much  hemoglobin  again  as  does  the  normal  corpuscle. 
And,  as  a  matter  of  fact,  we  find  very  considerable  variations  in  this 
color  index.  In  chlorosis,  for  example,  it  is  reduced,  in  pernicious  anemia 
markedly  increased.  And  these  findings  tally  with  the  clinical  observa- 
tion that  in  chlorosis  very  slight  exertion  brings  on  breathlessness — 
through  lack  of  adequate  oxygenation — whereas,  in  pernicious  anemia 
the  patient  in  general  only  consults  his  doctor  for  weakness  when  the 
number  of  red  corpuscles  has  fallen  to  2,000,000  or  less. 

Reduction  of  the  color  index  may  either  indicate  (as  in  chlorosis)  a 
primary  inadequate  production  of  the  hemoglobin,  or,  on  the  other  hand, 
a  diffusion  of  the  same  out  of  the  corpuscles  into  the  plasma.  Such 
hemolysis  occurs  under  a  variety  of  conditions.  Our  attention  has  been 
drawn  to  it  of  late  years  more  particularly  by  the  studies  upon  cytolytic 
action,  and  the  remarkable  effects  produced  both  by  foreign  sera  from 
normal  animals,  and  by  the  sera  of  animals  of  the  same  or  other  species 
which  have  received  injections  of  cells  of  various  orders.  Such  sera 
come  to  contain  bodies  which,  both  in  the  removed  blood  and  when 
injected  into  the  vessels,  cause  a  marked  "laking"  of  the  blood,  so  that 
the  corpuscles  become  represented  by  colorless  shadows.  There  are 
many  other  agencies  which  have  a  like  effect — cold,  as  in  paroxysmal 
hemoglobinuria;  heat,  as  in  burns;  and  many  chemical  agents — potassium 
chlorate,  ricin,  toluylenediamin,  glycerin,  pyrogallic  acid,  etc.  What  is 
of  particular  interest  as  possibly  explaining  some  at  least  of  the  second- 
ary anemias,  is  the  hemoly tic  action  of  not  a  few  of  the  pathogenic 
microorganisms — streptococcus,  pyococcus  aureus,  bacillus  pyocyaneus, 


90  THE  ERYTHROCYTES 

bacillus  coli,  the  pneumococcus,  etc.  These  organisms  may  be  grown 
in  defibrinated  blood  solutions,  when  the  laking  can  be  readily  observed 
by  the  change  in  the  appearance  of  the  medium,  or  laking  may  be  directly 
induced  by  the  addition  of  the  fluids  of  culture. 

In  all  these  cases  it  would  seem  that  we  deal  with  more  than  the  mere 
diffusion  out  of  the  hemoglobin.  Some  changes  must  occur  in  the 
physical  state  of  the  corpuscles  before  the  pigment  becomes  liberated, 
and  once  it  is  liberated  the  corpuscle  is  rendered  useless.  We  possess  no 
evidence  that  the  erythrocyte  has  the  power  to  regenerate  hemoglobin 
after  loss.  The  fate  of  the  affected  corpuscles  is  to  be  removed  from 
the  circulation  by  the  agency  of  the  spleen.  Any  condition,  therefore, 
bringing  about  severe  or  continued  hemolysis  causes  with  it  a  condition 
of  (so-called)  anemia,  i.  e.,  a  reduction  in  the  number  of  circulating 
erythrocytes.  The  anemia  so  produced  has  been  studied  in  our  labora- 
tory by  Charlton,  who  injected  over  long  periods  into  rabbits  non- 
lethal  doses  of  a  relatively  non-virulent  colon  bacillus  isolated  from 
the  intestine  of  a  normal  rabbit.  Bunting  employed  similarly  repeated 
small  doses  of  ricin.  Charlton  was  able  in  several  instances  to  bring 
down  the  number  of  erythrocytes  to  1,000,000,  and  found  that  so 
soon  as  the  number  reached  2,500,000,  poikilocytosis  with  occasional 
megaloblasts  became  developed;  he  found,  however,  little  obvious 
change  in  the  bone  marrow.  In  Bunting's  cases  the  evidence  of  increased 
activity  of  the  bone  marrow  was  well  marked,  the  marrow  closely  resem- 
bling that  seen  in  pernicious  anemia.  Other  observers,  like  Hunter  with 
his  researches  upon  the  effects  of  toluylenediamin,  have  studied  the  more 
immediate  effects  of  hemolytic  agents,  as  shown  by  the  excretion  of  the 
liberated  hemoglobin  through  the  liver  and  kidneys,  in  the  former  case 
as  bile  pigment. 

Chlorosis.1 — Here  it  will  be  well  to  pass  in  review  certain  of  the  more 
important  disorders  characterized  by  altered  states  of  the  red  corpuscles. 
And  first  we  may  consider  chlorosis,  a  condition  formerly  very  common, 
but  now,  according  to  Cabot,  becoming  relatively  uncommon,  at  least 
in  North  America.  It  affects  young  women  (94  per  cent,  of  Cabot's 
cases  were  between  the  ages  of  fifteen  and  thirty  years) ;  rare  cases  have 
been  recorded  in  young  males,  so  rare  that  many  writers  doubt  their 
existence.  Save  that  the  onset  suggests  some  disturbance  of  the  recently 
established  menstrual  function,  and  that  the  majority  of  cases  are  of 
girls  in  household  service,  who  have  exchanged  the  freer  life  in  the 
country  for  one  spent  largely  indoors  under  not  the  best  hygienic  con- 
ditions, we  have  practically  no  indications  suggesting  the  causation  of 
the  disease — which  in  itself  is  not  fatal,  although  it  may  predispose  to 
tuberculosis  and  other  more  fatal  conditions. 

The  characteristic  features  are  the  cachexia,  the  bloodless,  pale 
complexion  ("green  sickness"),  with  pallor  of  the  lips,  the  weakness 
and  lassitude  following  upon  slight  exertion,  the  dyspepsia,  capricious 

1  The  fullest  recent  study  of  this  condition  is  by  Cabot,  in  Osier  and  McCrae's 
Modern  Medicine,  4 :  1908 :  639,  based  on  the  study  of  497  cases. 


CHLOROSIS:  SECONDARY  ANEMIAS  91 

appetite,  with  gastric  acidity,  constipation,  and  palpitation,  and  the 
state  of  the  blood.  There  is  a  slight  but  definite  reduction  of  the  number 
of  erythrocytes,  a  marked  reduction  in  the  amount  of  hemoglobin  per 
corpuscle;  the  color  index  averages  about  0.5,  but  may  be  as  low  as  0.1. 
With  this,  as  clearly  demonstrated  by  Haldane  and  Lorrain  Smith,  there 
is  pronounced  increase  in  the  amount  of  blood  plasma,  an  increase  more 
than  sufficient  to  explain  the  reduction  in  the  number  of  erythrocytes 
per  cubic  millimeter.  There  is  a  true  serous  plethora.  Save  that  more 
might  have  been  said  regarding  the  symptoms,  there  is  little  to  add  that 
is  definite  in  our  knowledge  of  the  disease,  with  the  exception  that  the 
rapid  general  improvement  which  follows  the  proper  administration  of 
iron,  accompanied  by  improved  hygienic  conditions,  suggests  that  we 
deal  very  largely  with  a  deficient  building  up  of  iron  into  the  iron  con- 
taining hemoglobin  of  the  developing  erythrocytes;  that,  in  short,  the 
reduction  of  the  color  index  is  the  central  feature  of  chlorosis.  Several 
observers  have  called  attention  to  the  accompanying  constipation  and 
the  improvement  that  follows  when  this  is  overcome;  thus  possibly  an 
intestinal  toxemia  may  play  some  part  in  the  production  of  the  condition. 

Secondary  Anemias. — With  the  exception  of  the  above  condition, 
when  we  can  determine  what  appears  to  be  a  satisfactory  cause  for  the 
diminution  in  the  number  of  erythrocytes,  we  speak  of  a  secondary 
anemia,  and  doing  so,  we  leave  as  a  class  apart  one  condition  of  unknown 
causation,  namely,  idiopathic  or  primary  pernicious  anemia.  Such  sec- 
ondary anemia  may  be  acute,  as  after  profound  loss  of  blood.  In  these 
cases  what  has  already  been  said  will  have  prepared  the  reader  to  find  a 
blood  greatly  diluted,  owing  to  passage  into  it  of  lymph  and  tissue  fluids, 
in  order  to  preserve  the  amount  of  circulating  fluid.  The  erythrocytes 
at  first  are  normal  in  appearance  and  hemoglobin  content,  but  eventually, 
if  the  loss  of  blood  has  been  great  and  the  pouring  of  new  cells  out  of 
the  bone  marrow  excessive,  there  may  appear  a  few  immature  erythro- 
cytes and  cells  of  the  megaloblastic  type.  Writh  this  there  may  be  a  tem- 
porary actual  increase  in  the  number  of  circulating  leukocytes  (mainly 
neutrophile)  and  a  lowering  of  the  color  index,  due  to  the  fact  that  the 
immature  erythrocytes  do  not  contain  the  normal  amount  of  hemoglobin. 
Extensive  hemolysis  may  also  bring  about  an  acute  anemia ;  this  may  be 
induced  by  severe  infections,  and  cases  are  on  record  in  which,  without 
the  supervention  of  hemorrhage,  the  blood  count  has  dropped  to  1,500,000 
within  a  few  days  of  high  fever.  Certain  drugs  taken  accidentally  (e.  g., 
acetanilide)  may  induce  active  hemolysis  and  give  a  similar  picture. 

Chronic  Secondary  Anemias. — In  these  acute  cases  we  have  in  the  main 
the  picture  of  great  reduction  in  the  number  of  erythrocytes,  followed 
by  stages  of  an  imperfect  hematopoiesis.  In  the  chronic  forms  imperfect 
hematopoiesis  may  be  said  to  hold  the  field.  The  main  features  are 
an  abundance  of  cells  smaller  than  normal,  and  a  low  color  index. 
Much,  however,  depends,  both  as  regards  the  erythrocytes  and  the 
leukocytes,  upon  the  grade  of  the  anemia,  as  again  upon  the  cause.  Thus, 
in  the  anemias  due  to  intestinal  parasites  the  milder  cases  show  at  most 
microcytosis  with  a  characteristic  eosinophilia,  but  grave  cases,  notably 


92  THE  ERYTHROCYTES 

those  due  to  the  Dibothriocephalus  latus,  the  fish  tapeworm,  present 
a  picture  indistinguishable  from  pernicious  anemia.  Lead  poisoning, 
again,  presents  an  undue  proportion  of  nucleated  reds  with  "  stippling," 
i.  e.,  with  basic  granulation  of  the  erythrocytes.  In  splenic  anemia, 
the  leukocytes  are  characteristically  diminished  in  number,  and  the 
color  index  is  very  low.  In  infancy,  the  anemias  present  aberrant 
characters.  In  accordance  with  the  other  tissues,  the  regeneration  of 
the  blood  cells  is  very  active,  with  the  result  that  abundant  cells  of 
erythroblastic  type  gain  entrance  into  the  blood,  and  the  color  index, 
instead  of  being  low,  is,  as  a  rule,  high. 

As  a  general  principle,  it  may  be  laid  down  for  these  secondary  anemias 
that  the  more  severe  and  the  more  long  continued  the  causative  condi- 
tions, the  greater  are  the  signs  of  exhaustion  and  using  up  of  the  erythro- 
blastic mother  tissues,  until,  in  the  advanced  cases,  we  obtain  pictures 
very  similar  to,  and  in  fact  at  times  indistinguishable  from,  those  of 
pernicious  anemia,  to  be  presently  noted. 

Pernicious  Anemia. — The  picture  presented  by  the  sufferer  from  per- 
nicious anemia  is  striking:  the  peculiar  yellow  cachexia,  the  absence  of 
emaciation,  and  in  its  place  the  laying  on  of  flabby  fat,  the  weak  heart 
action  and  pulse,  and  striking  general  weakness.  At  autopsy,  the  bright 
yellow  color  of  the  fat,  the  bloodlessness  and  anemia  of  all  the  organs, 
the  pale  color  of  what  blood  there  is,  the  pronounced  fatty  degeneration 
of  the  heart,  the  presence  of  increased  iron,  more  particularly  in  the 
liver,  shown  by  either  Quincke's  or  Perl's  test,  the  increased  red  marrow 
of  the  bones,  and  the  frequent  presence  of  evidence  of  a  chronic  gastritis, 
all  combine  to  form  a  combination  that  cannot  be  mistaken. 

The  most  striking  of  these  disturbances  is  the  blood  condition.  That 
blood  is  both  diminished  in  amount  and  characterized  by  a  marked 
diminution  in  the  number  of  erythrocytes.  The  number  may  fall  to 
below  500,000.  We  have  at  the  same  time  evidence  of  active  disintegra- 
tion of  the  red  corpuscles  (indicated  by  the  increased  storage  of  iron  in 
the  liver)  and  of  active  regeneration  (indicated  by  the  hyperplasia  of  the 
red  marrow  and  the  relatively  abundant  nucleated  red  corpuscles  of 
different  orders  in  the  circulating  blood).  But  the  regeneration  does 
not  proceed  at  a  rate  sufficient  to  compensate  for  the  destruction.  What 
is  characteristic  is  that  the  individual  erythrocytes  are  many  of  them 
larger  than  normal,  and  they  contain  increased  hemoglobin;  so  that  a 
main  feature  is  the  very  high  color  index.  That  the  corpuscles  are  not 
healthy  is  indicated  by  the  marked  poikilocytosis;1  they  are  flabby  and 
assume  various  shapes.  But  despite  the  high  index,  owing  to  the  great 
reduction  in  the  number  of  corpuscles  and  reduction  in  the  amount  of 
blood,  the  total  amount  of  hemoglobin  is  greatly  lowered.  To  this 
lowered  oxygen-carrying  capacity  of  the  blood  must  be  ascribed  the 
diminished  metabolism  and  the  storage  of  fat  (a  similar  tendency  to 
lay  on  fat  is  often  observable  in  chlorosis),  and  possibly  the  continued 
malnutrition  in  itself  is  responsible  for  the  fatty  degeneration  of  the  heart 

f,  varied  or  various  (referring  to  shape). 


OF   THE 

UNIVERSITY 

OF 


PLATE    II 

FIG.   1 


Pernicious  Anaemia.     (Cabot.) 

The  field  shows  marked  anisocytosis  and  poikilocy tosis :  Ml,  young  megaloblast  (early  generation); 
M2  M8  M4,  later  generations  of  the  megaloblast  series;  S  S  S,  "stippled"  red  cells;  R,  ring  body  (nuclear 
remnant?);  L,  lymphocyte. 

FIG.  2 


Eosinophilia  (Triehiniasis).     (Cabot.)  , 

Copy  of  an  actual  field  containing  three  normal  poiynuclear  eosinophiles,  one  broken  eosinophile,  one 
polynuclear  neutrophile,  and  one  lymphocyte. 


PERNICIOUS  ANEMIA  93 

and  other  organs,  although  this  may  also  be  ascribed  to  the  unknown 
toxic  agent  causing  the  hemolysis  and  continued  blood  destruction. 

What  this  toxic  agent  is  we  still  are  in  ignorance;  obviously  it  is  of 
hemolytic  nature,  and  acts  over  long  periods.  One  striking  feature  of 
the  disease  is  that  it  is  already  far  advanced,  as  a  rule,  before  the  patient 
feels  himself  ill  enough  to  consult  a  physician;  another,  that  apparently, 
despite  the  varied  nature  of  the  treatment  afforded — save  that  the  com- 
mon basis  of  all  forms  of  treatment  is  rest  and  simple  diet — there  occurs 
in  the  majority  of  cases  a  distinct  remission;  the  number  of  erythrocytes 
increases,  the  general  condition  improves,  until  the  blood  returns  prac- 
tically to  the  normal  and  the  patient  is  apparently  restored  to  health. 
This  may  persist  for  but  a  month  or  two,  as  long  as  four  years — only 
rarely  is  the  improvement  permanent.  Then,  without  obvious  cause, 
a  relapse  occurs.  Cabot,  from  an  analysis  of  several  hundred  cases, 
states  that  the  average  time  of  remission  is  for  one  year,  and  quotes  cases 
in  which  the  relapse  has  occurred  at  the  same  season  in  successive  years; 
our  impression  is  that  too  great  stress  must  not  be  laid  upon  this  regular- 
ity. The  only  suggestion  that  can  be  made  to  explain  these  remissions 
is  that  the  enforced  rest  and  dieting  gives  the  system  the  opportunity 
to  counteract  the  intoxication.  More  and  more  Hunter's  conclusion 
that  we  deal  with  some  alimentary  intoxication  is  gaining  ground. 
The  frequency  of  a  septic  condition  of  the  mouth,  or  of  evidences  of  a 
chronic  atrophic  gastritis,  the  very  frequent  absence  of  hydrochloric 
acid  from  the  gastric  juice,  the  gastric  distress,  and  the  frequent  con- 
dition of  diarrhoea,  all  call  attention  to  the  digestive  tract.  And  here 
three  possibilities  exist — either  that  we  deal  with  (1)  the  absorption  of 
hemolytic  products  of  abnormal  digestion,  (2)  the  absorption  of  similar 
hemolytic  products  of  abnormal  bacterial  fermentation,  or  (3)  the  pres- 
ence and  growth  in  the  digestive  tract  of  organisms  of  a  low  pathogenic 
type,  and,  as  a  consequence,  the  development  of  a  state  of  subinfection 
(vol.  i,  p.  425),  the  increased  carriage  of  organisms  into  the  splanchnic 
blood  stream  being  favored  by  inflammatory  states  of  the  alimentary 
mucosa. 

Certain  considerations  favor  either  of  the  latter  two  theories,  namely, 
(1)  the  known  power  of  acid  reaction  of  the  stomach  contents  to  arrest 
bacterial  proliferation,  and  the  converse,  that  absence  of  hydrochloric 
acid  from  the  upper  intestinal  tract  favors  bacterial  growth  and  abnormal 
fermentation;  (2)  the  observed  anemia  that  accompanies  foul-smelling 
putrefactive  states  of  the  contents  of  the  lower  intestines. 

Here  at  the  present  time  the  study  of  pernicious  anemia  may  be  said 
to  rest.  We  are  coming,  that  is,  to  the  conclusion  that  pernicious  anemia 
is  not  idiopathic;  that  the  typical  forms  have  associated  with  them  bac- 
terial overgrowth  in  the  alimentary  canal  of  such  an  order  that  hemo- 
lytic agents  are  developed  in  undue  quantities,  but  still  have  to  determine 
what  particular  order  of  bacteria  are  concerned,  and  how  they  act. 
In  conclusion,  we  would  repeat  that  it  has  to  be  kept  in  mind  that  a 
similar  syndrome  and  extreme  alteration  of  the  blood  may  be  produced 
by  known  agencies,  by  hemolytic  substances,  such  as  repeated  small 


94  THE  LEUKOCYTES 

doses  of  ricin  (Bunting),  by  the  presence  of  the  Dibothriocephalus  latus 
in  the  intestines  (whatever  be  its  mode  of  action),  and,  rarely,  in  the 
latter  stages  of  malignant  disease.  In  this,  Crile  and  others  have 
determined  the  existence  of  hemolytic  substance  in  the  blood.  We  have 
seen  the  condition  develop  also  in  a  patient  the  subject  of  repeated  small 
hemorrhages  extending  over  two  years,  from  a  villous  papilloma  of  the 
bladder.  In  that  case  the  picture  was  complete,  save  for  the  absence  of 
excess  of  iron  in  the  liver,  i.  e.,  there  had  been  no  in tra vascular  destruc- 
tion of  red  corpuscles,  but  a  steady  loss  of  the  same  from  the  system. 
So  also  there  was  no  recognizable  disturbance  of  the  gastro-intestinal 
tract. 

Aplastic  Anemia. — Rare  cases  are  on  record  of  a  yet  further  stage 
in  what  appears  to  be  the  same  process.  In  this  it  would  seem  that  the 
overgrowth  of  the  red  marrow  and  excessive  production  of  erythrocytes 
is  followed  by  a  stage  of  exhaustion,  so  that  upon  examining  the  medulla 
of  the  sternum  or  femur,  etc.,  instead  of  finding  an  overabundance  of 
red  marrow,  that  red  marrow  is  reduced  in  amount,  and  replaced,  it 
may  be  wholly,  by  yellow  fat;  instead  of  hyperplasia  there  is  aplasia; 
or,  judging  from  the  rapid  course  of  many  of  the  cases,  it  may  be  urged 
that  from  some  congenital  weakness  or  other  cause  the  agent  setting 
up  the  hemolysis  does  not  excite  an  adequate  hyperplasia  from  the  first, 
and  the  erythroblasts  are  quickly  used  up.  As  indications  of  this 
exhaustion  are  to  be  noted  the  absence  of  normoblasts  and  megaloblasts, 
and  the  low  color  index.  Poikilocytosis  and  anisocytosis1  are  found  largely 
wanting,  as  are  also  the  leukocytes  proper,  causing  a  relative  abundance 
of  lymphocytes.  Hemorrhages  are  frequent.  Cabot  has  collected 
twenty-two  cases  of  this  nature  from  the  literature. 


THE  LEUKOCYTES. 

With  the  present  continued  doubt  regarding  the  relationship  of  the 
different  forms  of  leukocytes  in  the  circulating  blood,  it  is  essential  that 
before  discussing  variations  in  the  relative  numbers  of  the  different  forms 
appearing  in  the  blood,  we  should  at  least  place  upon  record  our  opinions 
regarding  that  relationship;  not  that  we  consider  our  views  so  well 
founded  that  they  are  unlikely  to  undergo  change,  but  because  the  whole 
of  our  treatment  of  leukocytosis  is  influenced  by  these  views.  We 
have  discussed  them  elsewhere.2 

Here,  as  regards  the  blood  as  distinct  from  the  inflamed  tissues,  we 
recognize  two  well-defined  groups:  (1)  The  granular  leukocytes,  and  (2) 
the  lymphocytes,  and  these  we  hold  are  of  separate  origin.  The  differ- 
ent forms  of  the  first  group  would  seem  all  to  originate  from  myeloblasts, 
large,  non-granulated  cells,  which,  in  the  adult,  are  present  throughout 
life  as  "  mother  cells"  in  the  bone  marrow.  These  originate,  as  do  also 


,  unequal  (referring  to  size). 
2  Inflammation.     London,  Macmillan  &  Co.,  4th  edition,  1909. 


THE  LEUKOCYTES  95 

the  erythroblasts,  in  intimate  connection  with  the  capillary  endothelium,1 
whereas  the  lymphocytes  are  derived  from  the  mother  cells  or  lympho- 
blasts  of  lymphoid  tissue.  The  myelocytes  give  origin  to  cells  which 
exhibit  granulations  of  different  orders — either  acidophilic,  staining  with 
the  acid  aniline  dyes,  or  basophilic — while  occasionally  we  may  encounter 
cells  which  morphologically  are  of  intermediate  type.  Despite  the  abun- 
dant studies  that  have  been  made,  we  still  lack  sure  evidence  that  the 
fully  developed  cell,  with,  for  example,  coarse  acidophile  granules  (eosino- 
phile),  undergoes  conversion  into  one  with  finely  granular  basophile 
(neutrophile)  granulations,  or  vice  versa.  All  the  evidence  indicates 
that  the  neutrophile  or  ordinary  polynuclear  leukocyte,  once  it  reaches 
this  stage,  remains  a  neutrophile  and  is  incapable  of  conversion  into 
other  form.  Still  less  do  we  have  any  indication  of  intermediate  stages 
between  the  lymphocyte  and  the  group  of  granular  leukocytes.  It  is 
true  that,  contrary  to  general  teaching,  by  special  methods  of  staining 
the  lymphocyte  can  be  seen  to  exhibit  extremely  fine  granulations,  but 
these  are  of  a  special  order,  and  are  not  demonstrable  by  the  ordinary 
methods  whereby  the  leukocytic  granulations  are  brought  out. 

As  to  the  functions  of  these  cells  under  normal  conditions  in  the 
circulating  blood,  while  we  have  many  indications,  we  know  little  that 
is  absolute.  We  know  much  more  concerning  that  function  when  they 
have  made  their  way  out  of  the  vessels  either  into  the  lymph  spaces  or 
on  to  the  surface  of  the  body.  These  latter  functions  we  have  already 
discussed  in  our  chapters  upon  Inflammation  (vol.  i,  p.  375).  It  is 
quite  possible  that  in  the  blood  stream  they  act  as  scavengers,  although 
normally,  judging  from  the  great  rarity  of  any  signs  of  inclusion,2  this 
property  is  little  called  into  play. 

There  are  observations  favoring  the  view  that  in  the  intestinal  villi 
the  circulating  leukocytes  may  actively  take  up  fatty  globules,  but  it 
may  be  questioned  whether  such  leukocytes  have  not  actively  taken 
up  these  globules  outside,  and  then  migrated  into  the  veins.  Again, 
according  to  Metchnikoff  s  views,  it  is  the  leukocytes  that  are  the  great 
manufacturers  and  storehouses  both  of  immune  bodies  and  comple- 
ments. At  the  same  time  he  holds  strongly  that  where  this  is  the  case, 
the  healthy  leukocyte  does  not  liberate  these  bpdies  into  the  blood 
plasma.  On  the  whole,  it  would  seem  more  probable  that  in  the  normal 
blood  the  leukocytes  exert  little  functional  activity,  that  the  blood  in 
the  main  acts  as  carrier  for  those  cells,  that  their  carriage  throughout 
the  body  is  to  subserve  their  function  of  acting  as  patrols,  so  that,  con- 
veyed to  any  capillary  region,  they  may  make  their  way  out  into  the 
tissues  through  chemiotactic  attraction.  Their  activity,  that  is,  would 
seem  to  be  more  extravascular  than  intravascular.  In  saying  this  we 
do  not  deny  that  circulating  toxins  have  an  influence  upon  the  number 
of  leukocytes  in  the  circulating  blood;  this  influence,  however,  would 

1  Vide  Schridde,  Centralbl.  f.  Pathol.,  19: 1908;  865. 

2  One  of  us  once  encountered  a  definite  bacillus  in  a  polynuclear  leukocyte  of  his 
own  blood,  he  being  at  the  time  in  good  health. 


<Mi  nil'.   LEUKOCYTES 

seem  to  be  exerted  primarily  upon  the  bone  urn  nmv  ;m<l  lympli  glandular 
(issue,  determining  a  greater  or  less  discharge  of  the  different  orders  of 
cells.  It  is  <<>  this,  mid  not  to  any  proliferation  of  flic  leukocytes  in  the 
blood  that  any  leukocytosis  or  the  reverse  condition  of  Icukopcnia.  must 
be  aseribed. 

Leukocytosis. — The  presence  of  any  increase  in  the  total  number  of 
white  corpuscles  in  the  peripheral  Mood  above  the  normal  constitutes 
leukocytosis.  I'sually  one  or  other  form  is  iu  relatively  greater  abun- 
dance; as  subgroups  we  distinguish  (<i)  a  polynuclear  Inikoci/iosis;  (b) 
eosinophilia;  and  (c)  lymphocytosis,  together  with  other  conditions  of 
the  appearance  of  aberrant  or  immature  forms  constituting  conditions 
of  (d)  leukemia.  Strictly  speaking,  we  do  not  consider  that  we  deal  with 
leukemia  until  the  number  of  these  aberrant  leukocytes  in  the  blood 
exceeds  15,000.  Strictly  speaking,  therefore,  we  may  have  a  myelocytic 
leukocytosis,  for  example,  that  is  not  a  leukemia. 

Using  the  term  in  its  broader  sense,  we  distinguish  next  between 
physiological  and  pathological  leukocytosis. 

Physiological  leukocytosis  shows  itself  in  a  variety  of  conditions.  In 
the  first  place,  there  is  in  the  young  a  well-marked  relative  leukocytosis. 
During  the  first  week  of  life  the  number  of  leukocytes  varies  between 
15,000  and  30,000;  during  the  first  ten  years  of  life  it  varies  between  1  (),()()() 
and  12,000.  In  the  female  toward  the  end  of  pregnancy  there  is  a  well- 
marked  rise,  reaching  to  15,000  to  20,000  at  the  time  of  parturition.  A 
distinct  rise  is  to  be  noted  after  violent  exercise,  massage,  cold  baths,  and 
the  application  of  electricity;  so  also,  full  proteid  diet  favors  what  is  known 
as  alimentary  leukocytosis.  Lastly,  a  terminal  leukocytosis  in  the  last 
hours  of  life  is  so  common  that  it  may  be  regarded  as  physiological, 

Pathological  Leukocytosis. — (a)  Polynuclear  or  neutrophilic  leukocy- 
tosis. It  is  in  inflammatory  and  infectious  conditions  that  we  more 
particularly  gain  this  form  of  leukocytosis.  We  can  produce  it  experi- 
mentally by  the  inoculation  of  many  pathogenic  organisms,  r.  </.,  by 
inoculating  non-lethal  doses  of  the  Pyococcus  aureus  into  the  peritoneal 
cavity,  when  for  the  first  few  hours  there  is  a  leukopenia,  followed 
next  day  by  a  pronounced  polynuclear  leukocytosis.  This  is  by  no 
means  a  universal  reaction  to  infection;  notably  it  is  wanting  in  typhoid, 
malaria,  tuberculosis  of  the  more  chronic  type,  and  when  without  second- 
ary infection,  leprosy,  measles,  mumps,  and  most  cases  of  inlluen/a. 
But  where  there  is  localized  or  generalized  suppuration,  it  becomes 
most  pronounced;  in  pneumonia,  again,  there  may  be  a  leukocytosis  of 
100,000,  with  95  per  cent,  of  polynuclears.  Our  contention  that  these, 
the  most  actively  phagocytic  leukocytes,  do  not  so  much  function  in  the 
blood  as  appear  there  to  be  utili/ed  later  in  the  tissues,  is  borne  out  by 
the  fact  that  in  malaria,  trypanosomiasis,  and  other  conditions  in  which 
animal  microparasites  multiply  in  the  blood  rather  than  in  the  tissues, 
a  polynuclear  leukocytosis  is  characteristically  wanting;  whereas  in 
pneumonia  and  the  suppiirative  diseases  in  which  the  irritants  multiply 
outside  the  bloodvessels,  there  this  form  of  leukocytosis  is  most  extreme. 

There  is  another  group  of  conditions  in  which  we  encounter  this  type 


LEI'  -    - 

-  -  Here  are  to  be  included  posthemorrhagic 

.  -     :"  hectic  cirrhosis,  and  other  states  eharae- 

. isturbanee  of  the  liver  cells  v  uremia,  etc.  .  gout,  the  later 

Stages  of  malignant  disease,  ptomaine  poisoning,   coal-gas  poisoning, 

;s  of  certain  drugs,  antipyretics,  salicylates,  pilocarpinc. 
-cnce,  that  is,  of  certain  products  of  abnormal  metabolism  and 
cell  disintegration  on  the  one  hand,  and  of  certain  ex..          -  .  hemical 
bodies  on  the  other,  set  up  an  increased  discharge  of  polymorphonuelear 
cells  from  the  bone  marrow  into  the  blood. 

Eosmophilia. — A  distinct  relative  increase  in  the  eosinophiles  of 
irculating  blood  is  s  the  majority  of  cases  of  helminth;. .  >  5 

nee  of  parasitic  worms  in  the  economy    vol.  i.  p.  319); 
irritative  skin  diseases    pemphigus,  dermatitis  herpeti- 
formis,   (  vompam      |  loid   leukemia:    ,4^   in  bronchial 

asthma,  and  to  a  slic  in  a  variety  of  conditions  which  it  is  diffi- 

cult to  correlate:  certain  malignant  cases  of  neoplasia,  ovarian  disease 
re    non-malignant  and  .  non-suppurative\   and    postfebrile    con- 
ditions* 

We  know  little  regarding  the  signi'1.  <inophiles.     The 

\yphile  granules  recall  strikingly  the  granules  of  gland  cells,  but 

the  observations  of  Kanthack  and  Hardy,  to  the  effect  that  these  may  be 

actively  excreted,  are  strongly  contested  by  most  hematologists,  although 

we  personally  are  prepared  to  accept  the  statements  of  these  most 

-ate  workers.     They  certainly  play  a  part  in  the  early  stages  of 

acute  inflammation,  being  the  first  cells  to  be  attracted:  they  accumulate 

in  the  omental  vessels  in  cases  of  peritoneal  inflammation:  and  very 

—  My  the  eosinophilia  of  diffuse  cr.  -  irritative  lesions  is  of  the 

same  local  type,  ?'.  r..  a  determination  of  eosinophiles  into  the  ve-    i 

of  the  inflamed  areas. 

Lymphocytosis. — Actual  or  relative  increase  in  the  lymphocytes  of 
the  blood  is  seen: 

1.  In  many  diseases  of  young  children,  notably  those  affecting  the 
\vintestinal  tract.    It  will  be  recalled  that  the  infant  shows  a  marked 

relative  and  absolute  lymphoeytosis.  This  is  doubtless  associated  with 
the  greater  relative  amount  of  lymphoid  tissue  in  the  young  individual, 
which  continues  into  childhood,  and  is  especially  marked  in  the  intestinal 
-ic  lymph  glands,  so  that  irritation  of  this  group  of  glands 
more  especially  is  accompanied  by  a  greater  discharge  of  lymplux 
into  the  blood. 

2.  In  septic  and  other  conditions  in  the  adult,  characterized  by  exces- 
sive enlargement  of  more  than  one  group  of  lymph  glands. 

3.  In  whooping  cough.     Here  the  lymphoeytosis  is  almost  pathog- 
nomonic.     A  lymphoeytosis  of  20,000  and  more  is  very  frequent,  and 

5   s       e  on  record  of  counts  in  the  neighborhood  of  iOO,000  vSteven, 

4.  In    debilitating   disease — scurvy,    rickets,    chlorosis,    and    various 
cachexias.     Here  the  lymphoeytosis  is  generally  of  a  moderate  grade. 

In  all  the  above  cases         deal  with  the  normal  small  lymphocytes. 


98  THE  LEUKOCYTES 

(d)  Leukemia. — We  have  in  our  first  volume  given  our  views  regard- 
ing the  relationship  of  the  leukemias  to  simple  hyperplasia  on  the  one 
hand  and  neoplasia  on  the  other.  Here  we  must  briefly  indicate  the 
main  features  of  the  leukemic  state,  and  the  effects  upon  the  organism. 
By  leukemia,  as  distinct  from  leukocytosis,  we  signify  not  merely  the 
excess  of  leukocytes  in  the  blood,  but  the  sum  total  of  the  disturbances 
which  accompany  the  continued  presence  of  a  great  excess  of  such  leuko- 
cytes. We  do  not  employ  the  term  when  the  number  is  below  15,000; 
indeed,  when  the  excess  is  below  that  figure  the  syndrome  of  disturb- 
ances is  scarcely  recognizable.  That  it  must  have  a  beginning  at  an 
earlier  period  is  obvious,  but  in  general  it  is  either  progressive  weakness, 
or  the  detection  by  the  patient  himself  of  a  greatly  enlarged  spleen  that 
first  leads  to  an  examination  of  the  blood  and  recognition  of  an  already 
advanced  condition.  And  then  one  of  two  orders  of  disturbance  is  to  be 
made  out;  either  a  great  excess  of  myelocytes  or  of  lymphocytes  in  the 
blood. 

MYELOGENIC  LEUKEMIA. — Myelogenic  leukemia  is  a  disease  affecting 
the  male  more  often  than  the  female,  most  often  in  early  adult  life, 
and  showing  itself  especially  by  disturbances  of  the  blood-forming 
organs  and  the  results  of  the  same.  Those  blood-forming  organs,  to 
repeat,  are  the  bone  marrow,  and,  under  pathological  conditions,  the 
spleen,  liver,  and  lymph  glands,  and  all  these  may  be  involved,  although 
the  extent  of  involvement  varies  in  different  individuals.  The  bone 
marrow  would  seem  always  to  be  affected,  exhibiting  a  replacement  of 
the  fat  by  hyperplastic  gray  marrow,  in  which  cells  of  the  myelocyte 
type  preponderate,  with  transitional  stages  from  myeloblast  through 
myelocyte  to  the  neutrophile  leukocyte.  Eosinophile  cells  may  also 
be  encountered,  erythrocytes  and  nucleated  red  corpuscles,  along  with 
a  distinct  increase  in  the  number  of  giant  cells  (megacaryocytes).  Lym- 
phocytic  elements  are  relatively  rare.  Normally  the  spleen  after  birth 
shows  little  evidence  of  myelocyte  formation,  but  now  it  is  pronounced, 
so  that  this  organ  takes  on  very  much  the  appearance  of  the  lymphoid 
Malpighian  bodies.  All  the  orders  of  cells  seen  in  the  leukemic  marrow 
are  encountered  in  this  organ,  which  undergoes  an  enormous  over- 
growth, so  that  at  times  it  may  extend  down  to  the  pubes,  still,  however, 
retaining  its  shape  and  general  proportions.  In  the  more  chronic  cases 
there  is  in  addition  an  extensive  fibrosis  of  the  organ.  Like  the  spleen, 
the  liver  is  actively  involved  in  blood  formation  during  antenatal  life, 
and  now  it  may  revert  to  its  foetal  activities.  Regarding  this  there  is 
still  debate;  many  authorities  hold  that  the  great  size  of  the  organ  and 
the  appearances  seen  on  section  are  due  entirely  to  accumulation  in  the 
capillaries  of  cells  conveyed  from  the  spleen,  and  that  the  atrophy  of 
the  liver  cells  (which  may  be  so  extreme  that  considerable  areas  are 
encountered  showing  nothing  but  myelocytes)  is  due  to  the  engorgement 
of  and  pressure  exerted  by  the  distended  capillaries.  Others  see  an 
activity  on  the  part  of  the  vascular  endothelium,  leading,  as  in  the  embryo, 
to  a  production  of  myelocytes  and  megacaryocytes  both  inward  into  the 
vessels  and  outward  to  form  an  extravascular  accumulation.  To  ex- 


.TE.  Ill 


Myelogenie  Leukemia.     (Cabot.) 

jpied  from  an  actual  field:  P,  polynuclear  neutrophilic  leukocytes;  M,  neutrophilic  myelocytes; 
,  transitional  neutrophile;  M  A,  mast  cell;  L,  "marrow  lymphocyte;"  E  E,  polynuclear  eosinophiles; 
stippled"  erythrocyte;  A',  normo^1 


LEUKOCYTOSIS  99 

plain  this  divergence  it  must  be  recalled  that  not  all  parts  of  the  hemato- 
poietic  system  are  necessarily  involved  at  the  same  time  and  to  the  same 
extent,  and  that  thus  it  happens  that  in  many  cases  the  liver  takes  no 
active  part.  This  organ  also  may  attain  great  size.  The  lymph  glands 
are,  as  a  rule,  only  moderately  involved.  Often,  save  for  some  accumu- 
lation of  myelocytes  in  their  vessels,  they  are  normal;  but  occasionally 
some  undergo  the  changes  which  make  them  closely  resemble  the  modi- 
fied bone  marrow  and  spleen.  Accumulations  of  the  modified  leuko- 
cytes may  fill  the  capillaries  in  the  lungs,  kidneys,  myocardium,  and  other 
organs. 

The  diagnostic  feature  is,  however,  the  blood.  Here  the  average 
number  of  white  corpuscles  is  between  400,000  and  500,000  (Cabot), 
varying  from  60,000  to  as  much  as  1,500,000  per  c.mm.  The  increase 
involves  all  the  orders  of  white  corpuscles,  the  most  striking  increase 
being  in  the  myelocytes,  which  may  constitute  about  one-half  the  total, 
and  the  polynuclears,  constituting  almost  the  other  half.  There  is  a 
well-marked  eosinophilia  (but  this  relatively  slight  as  compared  with  the 
increases  above  noted);  an  increase  in  the  number  of  mast  cells  (coarsely 
granular  basophiles),  and  though  proportionately  they  are  greatly  dimin- 
ished, the  number  of  lymphocytes  is  actually  greater  than  that  found  in 
normal  blood. 

As  regards  the  red  cells,  until  the  final  stages,  there  is  usually  only  a 
slight  diminution  to  not  less  than  3,000,000.  Toward  the  end  the  dimi- 
nution becomes  much  greater,  and  with  this  there  may  be  poikilocytosis 
with  presence  of  nucleated  reds.  We  have  indications,  in  short,  of  a 
profound  hyperplasia,  affecting  especially  those  myeloblasts  which  give 
origin  to  the  polynuclears,  and  in  their  proliferation  are  discharged 
as  immature  myelocytes.  Simultaneously  the  other  elements  of  the 
marrow  tend  to  proliferate;  hence,  the  eosinophilia,  the  nucleated  reds, 
and  somewhat  increased  discharge  of  lymphocytes. 

This  great  increase  in  the  large  myelocytes  leads  to  accumulation  of 
the  same  in  the  organs  possessing  finer  capillaries,  and  brings  about  both 
local  malnutrition,  through  clogging  of  their  channels,  and  general  slow- 
ing of  the  circulation.  Actual  emboli  may  thus  be  formed.  The  accom- 
panying reduction  in  the  erythrocytes  also  favors  malnutrition.  These 
alone  or  combined  may  explain  the  dyspnoea,  weakness,  and  moderate 
wasting  characteristic  of  the  disease,  as  again  the  epistaxis,  retinal  and 
other  hemorrhages,  and  the  persistent  priapism  occasionally  encountered. 
But  if  there  be  increased  production  of  leukocytes,  there  are  also  well- 
marked  indications  of  increased  destruction,  foremost  among  which 
we  to-day  place  the  excessive  discharge  of  uric  acid  through  the  kidneys 
due  to  the  disintegration  of  the  nuclei  of  the  destroyed  cells.  We  know 
of  no  microbic  or  other  cause  of  the  disease,  and  to  the  liberation  of  the 
products  of  cell  disintegration  may  possibly  be  ascribed  the  fever  present 
in  practically  every  case.  The  course  of  the  disease  is  distinctly  chronic, 
lasting  for  from  six  months  to  several  years. 

LYMPHATIC  LEUKEMIA. — This  condition,  again,  is  more  frequent  in 
the  male  than  in  the  female,  and  in  young  adult  life,  though  cases  occur 


100  THE  LEUKOCYTES 

more  widely  distributed  from  infancy  to  old  age.  Unlike  the  myelogenic 
form,  this  may  exhibit  an  acute  onset  and  course,  and  we  may  broadly 
distinguish  the  two  types:  of  acute,  occurring  more  frequently  in  the 
first  thirty  years  of  life,  and  of  chronic,  in  the  latter  half.  Whereas  it 
is  the  enlarged  spleen  that  is  the  commonest  physical  sign  of  the  former 
condition,  here  enlargement  of  the  lymph  glands  is  the  marked  feature; 
weakness,  dyspnoea,  emaciation,  hemorrhages,  are  common  to  the  two 
conditions. 

The  blood  in  the  chronic  cases  shows  an  extraordinary  preponderance 
of  typical  lymphocytes — typical,  that  is,  in  form,  with  large,  deep- 
staining  nucleus  and  small  rim  of  cytoplasm,  as  again  in  size.  In  a  large 
proportion  of  the  acute  cases  we  meet  with  cells  so  atypical,  large,  with 
abundant  cytoplasm,  and  nuclei  of  irregular  shape,  and  not  so  deeply 
stained,  that  while  in  many  cases  we  distinguish  transitional  forms 
between  these  and  the  small  lymphocyte,  in  others,  doubts  arise  as  to 
whether  we  deal  truly  with  cells  of  the  lymphocyte  group — whether  we 
have  not  to  deal  with  myeloblasts,  the  non-granular  forerunners  of  the 
myelocyte.  It  is  worthy  of  note  that  it  may  be  laid  down  that  the 
more  acute  the  case,  the  greater  the  tendency  for  the  lymph  glands  to 
be  but  slightly  enlarged,  and  for  the  brunt  of  the  hyperplasia  to  be  found 
in  the  bone  marrow.  We  are  inclined  to  the  belief  that  up  to  the  present, 
adequate  means  of  distinguishing  between  the  most  immature  forms  of 
lymphoblast  and  myeloblast  have  not  been  elaborated,  and  that  in  this 
class  we  include  the  most  acute  forms  of  both  lymphatic  and  myelo- 
genic leukemia. 

There  is  in  most  cases  both  acute  and  chronic,  some  enlargement 
of  the  spleen,  though  this  in  general  is  not  so  marked  as  in  the  myelogenic 
form;  the  same  is  true  regarding  the  liver.  A  condition  peculiar  to 
this  disease  is  overgrowth  of  the  multiple  minute  lymphoid  collections 
in  the  skin,  causing  the  appearance  of  multiple  small  nodules.  The 
number  of  circulating  leukocytes  is,  as  a  rule,  not  so  great  as  in  myelo- 
genic leukemia;  the  average  is  in  the  neighborhood  of  200,000.  Counts 
between  400,000  and  1,000,000  are  not  common.  What  is  characteristic 
is  the  infrequency  of  forms  other  than  those  above  described;  the  poly- 
nuclears  are  few  in  number;  eosinophiles  and  mast  cells  may  be  wholly 
wanting;  the  basophile  myelocytes  are  either  absent  or  not  abundant. 

Lastly,  these  cases  are  characterized  by  a  more  continuous  and  some- 
times high  fever  (102°  to  104°  F.)  with  severe  sweats,  so  that  without 
blood  examination  it  is  easy  to  mistake  the  condition  for  typhoid,  acute 
tuberculosis,  hepatic  abscess,  etc.  The  condition  is  most  fatal. 

What  has  been  said  regarding  the  relationship  of  the  symptoms  to  the 
blood  changes  in  myelogenic  leukemia  applies  to  this  condition  also. 

Before  leaving  the  subject  it  is  necessary  to  call  to  mind  that  similar 
hyperplasia  of  the  lymph  glandular  system  may  present  itself  without 
excessive  discharge  of  lymphocytes  into  the  blood  ("pseudoleukemia"), 
or,  again,  that  the  blood  in  the  last  stages  of  pernicious  anemia  may  simu- 
late that  of  leukemia,  while  similarly  the  last  stages  of  myelogenic 
leukemia  may  be  accompanied  by  so  great  a  destruction  or  lack  of 


PLATE    IV 


FIG.    ! 


Chronic  Lymphatic  Leukemia  (Actual  Field).    (Cabot.) 

Twenty-nine  typical  small  lymphocytes;  D  D,  degenerating  lymphocytes;  N,  normoblast. 


FIG.  2 


Acute  Lymphatic  Leukemia  (Actual  Field).     (Cabot.) 

L,  atypical  "lymphocytes"  (Naegeli's  myeloblasts) ;  M,  neutrophilic  myelocyte;  P,  polynuclear  neutro- 
phile;  A,  "large  lymphocyte,"  with  "azur"  granules;  B,  megaloblast  (stippled);  N,  normoblast. 


THE  BLOOD  PLATELETS  101 

production  of  erythrocytes  as  to  bring  about  a  condition  much  resem- 
bling pernicious  anemia.  With  Cabot,  we  regard  one  or  other  of  these 
conditions  as  explaining  the  condition  of  leukanemia  of  Leube1  and 
other  recent  writers. 

Regarding  pseudoleukemia,  Hodgkin's  disease,  chloroma,  and  lympho- 
sarcomatosis,  the  reader  is  referred  to  our  first  volume  (pp.  675-686). 

THE  BLOOD  PLATELETS. 

If  only  because  they  play  so  important  a  part  in  the  process  of  throm- 
bosis, it  is  necessary  to  have  a  clear  understanding  regarding  the  blood 
platelets,  or,  more  accurately,  regarding  what  is  known  concerning  their 
origin.  Apart  from  this,  with  the  advent  of  the  Romanowsky  stain  and 
its  modifications,  they  have  of  late  years  come  in  for  increasing  recogni- 
tion. There  is  no  longer  any  disposition  to  regard  them  as  artefacts, 
but  there  is  still  dispute  as  to  their  exact  significance  and  as  to  their 
unity  or  duality. 

They  are  small  bodies  of  varying  size,  in  general  about  2  //  in  diameter, 
oval  or  pear-shaped,  evidently  labile,  and  varying  in  shape  with  slight 
compression  by  neighboring  cells  or  platelets,  tending  to  be  present  in 
smears  in  small  groups  (possibly  as  the  result  of  rapid  agglutination  in 
the  shed  blood).  They  are  non-nucleated,  although  containing  often 
fine,  central  granules,  which  assume  a  redder  tint  with  the  Romanow- 
sky stain  in  contrast  to  the  bluer  groundwork.  Pratt's  careful  studies 
show  that  they  are  present  in  the  normal  blood  in  greater  numbers  than 
the  leukocytes,  although  the  number  shows  wider  variation — from 
200,000  to  700,000  per  c.mm.  Since  their  discovery  and  the  early  papers 
by  Hayem,  Bizzozero,  and  Mrs.  Ernest  Hart,  there  have  been  very 
various  views  regarding  their  nature  and  mode  of  origin:  (1)  That 
they  are  precipitated  globulin  (Lowit,  Wooldridge);  (2)  that  they  are 
products  of  disintegration  of  white  corpuscles  (Lilienfeld,  Zenker,  and 
others);  (3)  that  they  are  given  off  from  disintegrating  red  corpuscles 
(Mosso,  Klebs,  Arnold,  etc.).  The  exquisite  preparations  made  by 
J.  H.  Wright,  of  Boston,2  demonstrate  without  possibility  of  doubt  that 
some  at  least  of  the  platelets,  and  those  most  typical,  are  normally  derived 
from  a  particular  order  of  cell,  namely,  from  the  giant  cells  (megacaryo- 
cytes)  of  the  bone  marrow.  These  cells  give  off  processes  projecting 
into  the  lumina  of  the  capillaries,  and  it  is  the  distal  portions  of  these 
which  become  liberated  into  the  blood  as  platelets.  As  such  they  may, 
as  Schimmelbusch  was  the  first  to  demonstrate,  retain  some  power  of 
amoeboid  movement,  but  the  mode  of  their  development  sets  at  rest  the 
debate  as  to  the  nature  of  the  central  staining  granules;  they  in  no  sense 
represent  a  nucleus. 

The  important  question  still  to  be  determined  is  whether  all  the 

1  Deutsche  Klinik,  1902,  No.  42. 

2  Unfortunately,  the  photographs  which  illustrate  his  paper  are  not  worthy  of  the 
preparations,  and  do  not  carry  conviction. 


102  DUST  BODIES— HEMOCONIA 

blood  platelets  have  this  one  mode  of  origin;  and  this  is  far  from  settled. 
That  the  leukocytes  play  any  part  in  their  production  must,  we  think, 
be  put  on  one  side,  although  certain  of  the  smaller  "dust  bodies"  to  be 
presently  referred  to  would  seem  to  have  this  origin.  Their  origin  from 
erythrocytes  cannot  be  so  easily  dismissed.  Some  would  urge  that  there 
exist  blood  platelets  proper,  and  other  bodies,  derived,  it  may  be,  from 
red  corpuscles  which  are  not  platelets.  But  this  cannot  be  seriously 
defended.  The  blood  platelets,  it  has  to  be  admitted,  are  very  variable 
—variable  in  size  from  I/*  up  to  half  the  diameter  of  a  leukocyte;  some 
have  the  central  granules  above  described,  others  show  more,  and  where 
granules  are  present  they  vary  in  amount  and  in  position;  most  contain 
no  hemoglobin,  others  have  a  hemoglobin  tint.  Now,  it  has  been  shown, 
more  particularly  by  Arnold,  that  under  conditions  of  intravascular  and 
extravascular  clotting  the  disintegration  of  the  erythrocytes  leads  to 
the  appearance  of  bodies  which  by  no  criterion  can  be  distinguished 
from  the  platelets  of  ordinary  blood.  They  may  originate  as  discharged 
endoglobular  bodies,  which  by  diffusion  soon  lose  their  hemoglobin, 
by  plasmorrhexis  (detachment  of  peripheral  nodular  projections),  or  by 
plasmoschisis  (whereby  the  whole  body  of  the  corpuscle  breaks  up 
into  oval  bodies  which  become  separate  platelets).  Appearances 
which  in  our  opinion  can  only  be  attributable  to  this  breaking  down  of 
the  erythrocytes  are  frequently  to  be  noted  in  connection  with  thrombi 
of  the  smaller  vessels  (e.  g.,  of  the  liver),  and  if  we  do  not  accept  these 
products  of  erythrocytic  disintegration  as  platelets,  then  we  are  placed 
in  the  dilemma  of  regarding  the  process  of  thrombosis  as  due  in  a 
notable  proportion  of  cases  not  to  blood  platelets  proper,  but  to  a  con- 
glutination of  bodies  which  are  not  blood  platelets,  but  simulate  them 
in  shape  and  properties.  In  short,  we  come  perilously  near  occupying 
the  position  of  the  student  who  held  that  the  Iliad  was  not  written  by 
Homer,  but  by  another  man  of  the  same  name.  It  is  simpler  to  admit 
that  the  products  of  disintegration  en  masse  of  more  than  one  order  of 
cell  afford  bodies  having  the  nature  of  blood  platelets. 

We  must  admit  that  in  pernicious  anemia  the  platelets  are  frequently 
(though  not  always)  diminished  in  number,  and  that  here  there  has 
been  observed  a  lack  of  giant  cells  in  the  bone  marrow.  In  purpura 
they  have  at  times  been  found  completely  absent.  We  know  of  no 
observations  on  the  marrow  giant  cells  in  these  cases.  They  are  dimin- 
ished also  in  typhoid — but  increased  in  myelogenic  leukemia,  and  in 
pneumonia. 

DUST  BODIES— HEMOCONIA. 

Still  smaller  bodies  or  particles  are  to  be  recognized  in  the  normal  blood 
—1  n  and  less  in  diameter.  To  these  H.  F.  Muller  has  given  the  name 
of  dust  bodies,  or  hemoconia.  The  observations  of  Nicholls1  and  others 
indicate  that  these  also  are  the  products  of  disintegration,  more  par- 
ticularly of  the  erythrocytes. 

1  Trans.  Royal  Soc.  of  Canada,  2d  series,  11,  1905,  sec.  4:1. 


CHAPTEE    V. 

THE  LYMPHATIC  SYSTEM— OEDEMA. 

RECENT  observations  have  very  materially  altered  our  conception  of 
the  finer  anatomy  of  the  lymphatic  system,  and,  with  this,  have,  of 
necessity,  modified  the  conditions  which  have  to  be  taken  into  considera- 
tion in  formulating  our  views  regarding  both  lymph  formation  and  the 
disturbances  in  the  amount  of  lymph  present  in  the  tissues  and  parts  of 
the  body.  So  recent  are  these  observations  that  time  has  not  yet  been 
afforded  for  experimental  review  of  the  older  hypotheses  in  the  light  of 
this  newer  knowledge.  It  is  impossible,  therefore,  to  write  in  other  than 
a  very  tentative  manner  about  what,  from  a  pathological  point  of  view, 
is  the  most  important  morbid  state  directly  dependent  upon  alteration  in 
lymph  production  and  lymph  discharge,  the  state,  namely,  of  oedema. 

The  older,  long-accepted  view  was  that  the  lymphatic  system  had 
its  origin  in  the  intercellular  spaces  of  the  various  tissues,  and  that 
these  "lymph  spaces"  opened  freely  into  an  arborization  of  lymph 
channels,  which  differed  from  the  spaces  in  being  of  a  definitely  tubular 
nature,  and,  like  the  blood  capillaries,  lined  by  an  endothelium.  The 
careful  studies  of,  more  particularly,  W.  G.  MacCallum  and  Florence 
Sabin  seem  to  demonstrate  conclusively  that  the  system  of  lymph 
vessels  has  arisen  by  a  process  of  budding  from  the  veins,  and  that 
it  remains  distinct  from  the  system  of  intercellular  lymph  spaces — as 
distinct,  that  is,  as  are  the  blood  capillaries.  It  follows,  therefore,  that 
in  discussing  lymph  formation  in,  and  lymph  discharge  from,  any  region, 
we  have  constantly  to  keep  in  mind  not  merely  the  mechanisms  whereby 
fluid  passes  out  of  the  bloodvessels  into  the  tissues,  but,  in  addition,  those 
controlling  the  passage  of  fluid  from  the  intercellular  lymph  spaces  into 
the  lymph  vessels.  We  have  to  recognize  thus  (1)  blood  plasma,  (2) 
intercellular  lymph,  (3)  lymphatic  lymph. 

Doing  this,  we  immediately  find  ourselves  in  an  impasse  so  far  as 
regards  the  establishment  of  hypotheses  on  the  basis  of  exact  data;  the 
ultimate  lymphatic  vessels  and  the  lymph  spaces  are  of  microscopic 
dimensions;  in  other  words,  no  sure  method  has  as  yet  been  devised 
whereby  to  collect  intercellular  lymph;  we  cannot  introduce  the  finest 
cannula  into  the  tissues  without  breaking  into  lymphatic  vessels;  and 
so  cannot  compare  lymphatic  lymph  and  unmixed  intercellular  lymph 
formed  at  the  same  time.  This  has  been  abundantly  determined,  that 
the  fluid  obtained  from  a  larger  lymph  vessel,  from  the  thoracic  duct, 
or  one  of  the  vessels  of  the  extremity  of  the  neck,  differs  materially  in  the 
relative  amounts  of  its  component  constituents  from  the  blood  plasma. 
There  is  variation,  even  if  slight,  in  the  percentage  amount  of  the  most 


104  THE  LYMPHATIC  SYSTEM— (EDEMA 

soluble  inorganic  salts.  We  have  not,  therefore,  to  deal  with  the  simple 
leakage  of  fluid  from  one  set  of  vessels  into  the  other.  But  what  is 
the  nature  of  the  process  or  processes  of  lymph  formation  is  still  a  matter 
of  keen  debate.  On  the  one  hand,  we  have  the  upholders  of  the  purely 
mechanical  theory,  first  clearly  formulated  by  Ludwig;  those  who  hold 
that  the  laws  of  filtration,  diffusion,  and  osmosis  are  adequate  to  explain 
the  variation  in  the  amount  and  composition  of  the  lymph  discharged 
from  a  part  under  varying  conditions;  on  the  other,  those  who,  with 
Heidenhain,  urge  that  while  the  known  physical  laws  in  part  determine 
the  production  and  constitution  of  lymph,  there  are  alterations  in  amount 
and  constitution  which  cannot  be  brought  into  harmony  with  the  working 
of  those  laws.  These  workers  demand  a  certain  selective  capacity  and 
activity  on  the  part  of  the  capillary  endothelium,  determining  to  some 
extent  the  amount  of  at  least  some  of  the  constituents  of  the  blood  plasma 
which  is  allowed  to  escape  from  the  capillaries.  The  most  prominent 
supporters  of  the  mechanical  theory  at  the  present  time  are  Starling1 
and  Cohnstein;  of  the  opposed  view,  are  Asher,  Hamburger,  Lazarus, 
Barlow,  Meltzer,  and  Carlson.  These  observers,  it  is  true,  differ  among 
themselves  as  to  cells  mainly  involved  in  the  process  of  lymph  formation, 
but  are  members  of  the  "vitalistic"  school  to  this  extent,  that  they  are 
unable  to  explain  lymph  formation  by  simple  physical  principles,  and  are 
compelled  to  fall  back  upon  more  elaborate  processes  occurring  within 
the  cell  as  introducing  modification  in  the  fluid  during  its  passage 
from  the  interior  of  the  blood  capillaries  to  the  interior  of  the  lymphatics. 
Here  a  word  is  necessary  regarding  the  meaning  of  " vitalism." 
There  are  those  who,  with  Haldane  and  B.  Moore,  see  evidence  of  the 
existence  of  "biotic  energy/'  of  energy  associated  with  and  determining 
the  activities  of  living  matter,  distinct  from  other  forms  of  energy. 
This  view  necessitates  the  fatalistic  attitude  that  vital  phenomena  are, 
beyond  a  certain  point,  incapable  of  explanation  by  the  ordinary  laws 
governing  matter  in  general.  With  this  view  we  have  absolutely  no 
sympathy.  All  that  Heidenhain  meant,  and  laid  down  with  precision  in 
his  opposition  to  Ludwig,  was  that  processes  are  undergone  in  the  living 
cell  which,  while  governed  by  the  ordinary  laws  of  physics  and  chemistry, 
are  nevertheless  so  complicated  that  hitherto  we  have  been  unable 
to  follow  the  successive  forces  acting  upon  the  assimilated  molecule  in 
its  passage  through  the  cell;  that  the  simpler  processes  of  diffusion, 
osmosis,  and  filtration  are  at  work,  but  are  not  everything.  According 
to  this  view,  it  is  quite  possible  that  further  research  will  throw  light  upon 
the  nature  of  the  intracellular  forces.  To  this  extent,  and  this  extent 
only,  with  Heidenhain  and  Meltzer,  we  class  ourselves  with  the  vitalists. 

1  The  clearest  statement  of  the  mechanical  theory  is  afforded  by  Starling  (Arris 
and  Gale  Lectures,  Lancet,  London:  May  9,  16,  and  23,  1906);  a  thorough  and 
impartial  criticism  of  all  the  theories  up  to  date  is  given  by  Meltzer  in  the  Harring- 
ton Lectures  on  (Edema,  American  Medicine,  8:1904:Nos.  1,  2,  4,  and  5.  This 
latter  is  the  fullest  recent  study  on  the  subject,  and  is  provided  with  a  rich  bibliog- 
raphy. 


THE  LYMPHATIC  SYSTEM— (EDEMA  105 

For  ourselves  we  cannot  accept  the  simpler  mechanical  theory,  and  this 
for  the  following  broad,  but  we  think  obvious,  reasons,  namely,  that  this 
view  presupposes  that  lymph  is  merely  the  outcome  of  the  discharge 
through  a  single  endothelial  membrane,  the  blood  capillary,  into  the 
lymph  channels,  takes  no  adequate  account  of  the  influence  of  the  tissue 
cells  in  its  composition,  and,  lastly,  fails  to  explain  how  the  interstitial 
lymph  makes  its  way  into  the  lymph  vessels.  The  problem  of  lymph 
formation  in  reality  consists  of  three  parts:  (1)  What  is  the  mechanism 
by  which  certain  constituents  of  the  blood  plasma  pass  through  the  capil- 
lary wall?  (2)  How  and  to  what  extent  is  the  interstitial  lymph  thus 
produced  acted  upon  by  the  tissue  cells  which  it  bathes?  (3)  By  what 
mechanism  does  the  interstitial  lymph  gain  entrance  into  the  lymphatic 
vessels?  If  for  the  moment  we  admit,  with  Starling,  that  the  first  of 
these  steps  is  purely  a  mechanical  process  determined  by  the  interaction 
of  two  factors,  the  intracapillary  blood  pressure  and  the  permeability 
of  the  capillary  wall,  we  still  have  the  other  two  problems  to  answer. 
Now,  as  regards  the  one  of  these,  we  are  altogether  too  apt  to  repeat, 
parrot-like,  that  the  tissues  are  nourished  by  the  blood.  As  a  matter 
of  fact,  save  for  the  vascular  endothelial  cells,  and  one  or  two  rare  excep- 
tions, like  the  Kupfer  cells  in  the  liver,  in  which  tissue  cells  impinge 
directly  upon  the  blood  stream,  the  tissue  cells  are  not  nourished  directly 
by  the  blood,  but  by  the  interstitial  lymph;  the  nutritive  fluid  has  to  pass 
out  of  the  capillaries  into  the  lymph  spaces  surrounding  the  individual 
tissue  cells  before  those  cells  can  abstract  from  it  the  particular  food- 
stuffs needed  by  them.  This  demands  that  the  different  orders  of 
cells  abstract  from  the  interstitial  lymph  different  orders  of  substances, 
as  also  that  they  excrete  or  discharge  into  it  very  varying  products 
of  metabolism;  in  short,  demands  conditions  of  give  and  take  of  so  com- 
plex a  nature  that  even  if  broadly,  under  certain  conditions  of  experiment, 
thoracic  duct  lymph  has  the  character  of  a  filtrate  through  a  semi- 
permeable  membrane,  it  cannot,  in  its  finer  analysis,  conform  to  a  fluid 
of  that  nature,  and  under  normal  conditions  of  moderate  flow,  must 
inevitably  depart  widely  from  this  type  of  fluid. 

And  as  regards  the  last  of  these  problems,  there  are  again,  it  seems 
to  us,  insuperable  difficulties  in  regarding  the  eventual  lymph  of  the 
lymph  vessels  as  a  filtrate  or  product  of  diffusion.  The  ultimate  lymph 
vessels  are  so  delicate  that  we  fail  to  recognize  their  existence  in  ordinary 
sections,  even  under  high  powers.  We  utterly  fail  to  conceive  how  the 
result  of  increased  accumulation  of  lymph  in  the  tissue  spaces  can  result 
in  an  increased  filtration  of  that  lymph  into  these  delicate  vessels.  On 
the  contrary,  the  greater  the  interstitial  pressure,  the  greater  the  tendency 
for  these  delicate  channels  to  become  collapsed  and  obliterated.  And, 
as  a  matter  of  fact,  the  extreme  tension  of  the  tissues  in  cases  of  advanced 
anasarca  of  the  lower  limbs,  for  example — a  tension  so  great  that  the 
lymph  is  apt  to  ooze  through  the  deeper  layers  of  the  cutis  and  form 
"blebs" — indicates  that  this  actually  happens.  But  in  other  cases,  as, 
for  example,  in  inflammatory  cedema,  we  obtain  a  marked  increase 
in  the  discharge  of  lymph  through  the  lymph  vessels  coming  from  the 


106  ANASARCA 

inflamed  area.  The  simplest  and  most  rational  conclusion  to  reach 
is,  that  ordinarily  the  lymphatic  endothelium  actively  absorbs  and 
secretes  the  interstitial  lymph  into  the  vessels,  and  that  under  certain 
conditions  this  secretion  is  increased.  Only  in  this  way  can  we  imagine 
an  active  flow  becoming  set  up  within  them.  And  if  we  are  forced  to 
predicate  such  powers  for  the  lymphatic  endothelium,  then,  by  analogy, 
we  must  suppose  that  the  capillary  endothelium  has  powers  of  a  like  order, 
and,  with  Heidenhain,  must  endow  this  with  a  certain  grade  of  selective 
secretory  activity.  Indeed,  the  conception  of  the  tissue  cells  as  nourished 
not  directly  by  the  blood,  but  by  the  lymph,  would  seem  to  demand 
that  the  capillary  endothelium  of  the  various  tissues  abstracts  particular 
substances  from  the  circulating  blood  necessary  for  the  specific  metabo- 
lism of  these  tissues,  and  passes  these  into  the  interstitial  lymph.  We 
cannot,  for  example,  comprehend  the  extraordinary  passage  of  fats  into 
the  milk,  unless  the  capillaries  of  the  mammary  gland  in  the  first  place 
possess  a  selective  power. 

Under  the  term  oedema  we  include  all  abnormal  accumulations  of 
fluid  approximating  in  its  constitution  to  that  of  lymph,  and  occurring 
in  the  tissue  spaces  and  serous  cavities  of  the  body.  To  these  accumula- 
tions in  different  regions  special  names  have  been  given,  and  with  this 
newer  knowledge  we  can  divide  them  into  distinct  classes: 

1.  Anasarca,  or  interstitial  cedema,  as  of   the  limbs  and  body  wall. 
Ghemosis  is  the  name  given  to  serous  infiltration  of  the  subconjunctival 
tissue.     (Edema  glottidis,  to  anasarca  of  the  upper  portion  of  the  larynx. 

2.  Accumulations  in  serous  cavities,  including  ascites,  involving  the 
peritoneal    cavity;    hydrothorax    (the    pleural);    hydrocele    (the   tunica 
vaginalis);  hydrocephalus,  internal  and  external,  involving  the  ventricles 
of  the  brain  and  the  pia-arachnoid  spaces. 

3.  Accumulations  of  albuminous  fluids  which,  strictly  speaking,  are 
outside  the  body,  i.  e.,  affect  surfaces  in  direct  communication  with  the 
exterior.     The  important  example  of  this  form  is  pulmonary  oedema. 

A  little  consideration  shows  that  in  these  three  classes  we  have  three 
different  orders  of  accumulations: 


ANASARGA. 

The  essential  feature  of  the  anasarcous  state  is  the  excessive 
accumulation  of  intercellular  lymph.  In  other  words,  the  discharge 
of  this  lymph  into  the  lymphatic  vessels  has  not  kept  pace  with  the 
formation  of  lymph  by  passage  of  fluid  through  the  capillary  walls. 
Examination  of  dropsical  tissues  under  the  microscope  shows  the  indi- 
vidual cells  composing  the  tissue  widely  separated,  in  consequence  of 
the  intercellular  accumulation.  What  is  characteristic  is  that  the  lym- 
phatic vessels  are  not  obvious.  On  the  contrary,  the  absence  of  distended 
lymph  vessels  suggests  that  the  interstitial  pressure  due  to  fluid  accumula- 
tion has  brought  about  a  relative,  or,  it  may  be,  a  complete  occlusion  of 
the  delicate  lymphatic  channels,  and  that  one  of  the  factors  in  the  con- 


ASCITES  AND  ALLIED  CONDITIONS  107 

tinuous  and  progressive  intensity  of  anasarca  is  this  local  obliteration 
of  these  vessels.  A  comparison  between  sections  from  simple  ana- 
sarca and  those  from  elephantiasis  or  macroglossia,  due  to  acquired  or 
inherited  obstruction  of  the  lymph  vessels  of  a  part,  shows  that  we  have 
to  deal  with  two  distinct  conditions,  namely,  of  (1)  interstitial  tissue 
accumulation  of  lymph,  and  (2)  lymphangiectasis,  or  distension  of  the 
lymph  vessels.  Even  prior  to  a  knowledge  of  the  barrier  separating  the 
lymph  spaces  from  the  lymph  vessels,  it  had  become  recognized  that 
this  latter  condition  was  a  separate  entity,  although  it  had  been  in  gen- 
eral neglected  in  the  discussion  of  cedema.  In  the  existence  of  these 
two  pathological  states  we  possess  the  confirmation  of  the  conclusion 
reached  by  MacCallum  and  the  anatomists. 


ASGITES  AND  ALLIED  CONDITIONS. 

The  serous  cavities  are  lined  throughout  by  an  endothelium.  It 
follows  thus  that  serous  fluid  accumulations  within  them  have  passed 
through  not  one  but  two  endothelial  layers  in  the  process  of  production. 
To  this  extent  they  correspond  with  lymph-vascular  lymph.  Here 
again  we  note  that  there  may  be  pronounced  (interstitial)  oadema  of 
the  intestines,  with  little  or  no  ascites,  and  vice  versa.  As  to  how  the 
ascitic  and  pleural  fluid  gain  entrance  into  the  efferent  lymph  vessels 
proper  is  still  a  matter  of  debate.  The  more  recent  teaching  is,  that 
under  normal  conditions  the  peritoneal  cavity  is  closed  off  from  the 
underlying  efferent  lymphatic  channels,  and  that  an  endothelial  layer 
covers  over  the  apparent  ostia.  We  find  it  difficult  to  harmonize  this 
teaching  with  the  abundant  injection  of  the  diaphragmatic  lymph 
channels  with  red  blood  corpuscles  which  rapidly  follows  the  introduction 
of  blood  into  the  peritoneal  cavity.  Such  abundant  passage  can  only, 
we  hold,  be  due  to  the  existence  of  actual  stomata,  or  channels  of  direct 
communication,  and  we  have  explained  the  curious  minute  hemispherical 
pits  occasionally  observable  in  plastic  exudates  covering  the  dome  of 
the  liver  as  caused  by  eddies  opposite  to  those  stomata  in  the  diaphragm. 
While  saying  this,  we  cannot  pass  over  the  evidence  adduced  by  Mac- 
Callum and  others  that  sections  through  the  diaphragm  demonstrate 
the  presence  of  a  distinct  membrane  separating  the  diaphragmatic 
lymphatics  from  the  peritoneal  cavity.  The  only  satisfactory  com- 
promise would  seem  to  consist  either  in  concluding  that  under  certain 
conditions,  by  retraction  or  contraction  of  the  endothelial  cells  constitut- 
ing or  affording  the  membrane,  what  had  been  an  intact  membrane 
becomes  provided  with  a  central  stoma  or  passage  of  direct  communica- 
tion, or  that  there  exists  normally  a  combination  of  intact  membranes 
and  occasional  scattered  stomata.  We  confess  that  the  latter  view 
does  not  appeal  to  us ;  the  existence  of  lining  membranes  would  seem  to 
predicate  a  certain  selective  function  and  control  of  the  composition  of 
the  efferent  lymph;  that  of  coarse  pores  or  stomata  would  be  diametri- 
cally opposed  to  any  such  selective  action.  On  the  other  hand,  the  exist- 


108  PULMONARY  (EDEMA 

ence  of  potential  stomata,  which  act  as  weirs,  closed  under  normal 
conditions,  but  permitting  the  free  passage  of  fluid  and  free  particles 
when  the  intraperitoneal  pressure  becomes  excessive,  would  seem  not 
irrational.  We  bring  these  matters  forward  at  this  point  in  order  to 
indicate  that,  according  to  the  one  view,  accumulations  in  serous  cavities 
are  distinct  from  lymph-vascular  lymph;  according  to  the  other,  the 
normal  existence  of  a  dividing  membrane  would  indicate  that  under 
more  natural  conditions  there  may  be  essential  differences  in  the  com- 
position of  the  two  fluids,  although,  under  abnormal  conditions,  the 
contents  of  the  serous  cavities  may  pass  unchanged  into  the  efferent 
lymphatics.  We  regret  that  in  the  present  state  of  our  knowledge 
it  is  impossible  to  afford  dogmatic  teaching  on  this  point.  The  fact 
that  in  a  hydrocele,  or  in  a  case  of  ascites,  the  fluid  may  accumulate 
until  there  is  very  high  pressure  is  not  to  be  brought  forward  on  one  side 
or  the  other;  there  might  be  abundant  stomata,  and  nevertheless  the 
pressure  be  such  as  to  obliterate  the  underlying  network  of  delicate  lymph 
channels,  and  thereby  arrest  the  outflow.  There  is,  however,  one 
striking  feature  in  connection  with  simple  transudates  (as  distinct  from 
inflammatory  exudates)  into  the  serous  cavities,  namely,  that  they  con- 
tain much  less  solids  than  either  serum  or  ordinary  lymph,  the  reduction 
being  specially  marked  in  albuminous  matter.  This  in  itself  may  justify 
us  in  considering  them  as  constituting  a  class  apart. 


PULMONARY  (EDEMA. 

There  can,  however,  be  no  doubt  as  to  the  necessity  of  regarding 
pulmonary  oedema  as  belonging  to  a  distinct  class.  That  fluid  is  always 
pouring  from  the  pulmonary  capillaries  into  the  alveoli  is  clearly  shown 
by  the  abundant  moisture  contained  in  the  expired  air;  the  accumulation 
of  serous  fluid  in  the  alveoli  is,  therefore,  only  to  be  regarded  as  an  exag- 
geration of  a  normal  process.  What  is  distinctive  is  that  here  the  accu- 
mulation of  fluid  is  not  interstitial,  but  is,  strictly  speaking,  external 
to  the  body;  the  discharge  is  on  to  surfaces  communicating  with  the  ex- 
terior. It  is  not,  therefore,  determined  by  any  force  acting  on  the 
capillaries  from  without;  neither  diffusion  nor  osmosis  can  be  called  into 
play  to  determine  the  discharge.  What  is  more,  the  delicate  alveolar 
epithelium  is  so  directly  applied  over  the  capillary  network  that  we 
appear  to  have  a  relationship  similar  to  that  seen  in  the  glomeruli  of  the 
kidneys,  with  a  practical  absence  of  intervening  lymph  space  between 
vessel  and  epithelium.  Here,  then,  again,  we  have  a  different  order  of 
conditions. 

Briefly,  while  it  is  impossible  not  to  be  impressed  by  Professor  Star- 
ling's valiant  support  of  Ludwig's  mechanical  hypothesis,  a  study,  more 
particularly,  of  actual  clinical  cases  of  oedema  cannot  but  convince  us 
that  this  hypothesis  does  not  satisfy.  Pressure  plus  variation  in  per- 
meability alone  will  not  explain  the  great  varieties  of  cases  in  which  we 
encounter  the  cedematous  state,  nor  the  variations  in  the  constitution  of 


CONGESTIVE  (EDEMA  109 

the  oedema  fluid.  It  will  not,  for  example,  explain  Leber's  experiment,1 
in  which  he  showed  that  the  cornea  with  intact  epithelium  of  the  mem- 
brane of  Descemet  will  stand  a  pressure  of  200  mm.  of  mercury,  whereas, 
once  that  epithelium  is  removed,  solutions  readily  filter  through;  or 
the  observations  of  Tigerstedt  and  Santesson  that  the  freshly  removed 
lung  of  a  frog  filled  with  0.6  per  cent.  NaCl  solution  will  stand  a  pressure 
of  14  mm.  of  mercury  for  several  hours  without  any  escape  of  the  con- 
tained fluid,  whereas  the  same  lung  killed  by  slight  heat,  or  by  pouring 
in  distilled  water  or  (frog's)  bile,  at  once  allows  filtration. 

Something  is  necessary  to  explain  the  sudden  change  in  the  porosity 
of  these  membranes  over  and  above  the  ordinary  physical  laws  deter- 
mining the  rate  of  filtration  through  dead  membranes. 

We  are  apt  to  regard  the  endothelial  cells  of  the  vessels  and  capillaries 
as  of  an  extraordinarily  low  type,  as  flattened  plates  of  cytoplasm  and 
little  more;  on  the  other  hand,  we  admit  freely  that  bacteria,  organisms 
much  more  minute,  and  of  a  much  lower  type  of  structure,  possess  selec- 
tive assimilative  powers.  The  position  is  irrational.  These  endothelial 
cells  are  nucleated;  they  are  actively  phagocytic;  can  proliferate  actively; 
and,  as  observed  in  inflammatory  states,  are  acutely  sensitive  to  changes 
in  their  environment.  While  admitting  that,  other  things  being  equal, 
they  permit  a  more  active  passage  of  plasma  under  a  higher  pressure, 
and  conform  in  many  respects  to  the  laws  governing  filtration,  diffusion, 
and  osmosis,  it  must,  we  think,  be  concluded  that  certain  substances 
are  taken  up  by  them  selectively,  while  at  times  other  substances  of  equal 
solubility  are  not  taken  up.  We  feel  some  compunction  in  dealing 
thus  so  largely  in  generalities.  The  subject  is  most  complicated,  and 
to  analyze  conscientiously  the  data  at  our  disposal  would  consume 
more  space  than  we  can  afford.  At  most,  keeping  these  views  in  mind, 
we  believe  that  the  different  forms  of  oedema,  and  the  variations  seen  in 
these,  become  more  comprehensible.  These  forms  are: 

1.  Congestive  (Edema. — This  is  the  commonest  clinical  type,  and 
is  seen  in  cases  of  obstruction  to  the  venous  onflow,  either  local  or 
general.  The  most  extensive  cases  are  seen  in  obstructive  heart  disease. 
In  such  cases  there  is  (a)  increased  venous  pressure,  (b)  increase  in  the 
total  amount  of  blood  within  the  vessels,  (c)  dilatation  of  the  capillaries 
and  increased  capillary  pressure,  (d)  slowing  of  the  blood  stream,  (e) 
increased  venosity  of  the  capillary  blood.  The  increased  capillary 
pressure  is  here  not  the  sole  factor.  Thus,  oedema  does  not  ensue  if 
the  main  vein  of  a  limb  be  ligatured  in  a  healthy  animal.  Again,  the 
variation  in  albuminous  contents  is  too  great  to  be  ascribed  to  mere 
difference  in  the  permeability  of  the  capillary  wall.  As  shown  by  Reuss, 
pleura!  transudations  on  the  average  contain  four  times  as  much  albumin 
as  do  those  from  the  subcutaneous  tissues,  and  twice  as  much  as  does 
ascitic  fluid.  We  are  forced  to  the  conclusion  that  the  cells  in  these 
different  regions  have  a  varying  sensibility,  and  are  affected  differently 

1  Leber,  Archiv  f.  Ophthalm.,  19: 1873: 125.  Tigerstedt  and  Santesson,  Bijhang 
till  K.  Svensk.  vet.  Akad.  Stockholm,  11: 1886:  No.  2. 


HO  (EDEMA 

by  the  increased  venosity  of  the  blood.  It  may  further  be  noted  that 
there  are  pronounced  individual  differences  in  the  reaction  to  one  and 
the  same  lesion.  Thus,  long-continued  mitral  stenosis  commonly 
results  in  pulmonary  oedema;  we  have,  nevertheless,  encountered  two 
cases  of  this  disease  in  which,  with  extreme  anasarca  and  ascites,  there 
has  been  inconsiderable  hydrothorax,  and  the  lungs  have  been  devoid 
of  serous  effusion.  Cases  of  advanced  anasarca  with  little  or  no  ascites, 
and  of  the  converse  condition,  are  far  from  infrequent. 

2.  Lymphatic  Obstruction. — The  accumulation  of  fluid  in  the  spaces 
of  the  body  is  dependent  on  the  interaction  of  two  factors — the  rate 
of  discharge  of  fluid  from  the  bloodvessels  and  the  rate  of  removal. 
If  the  latter  be  less  than  the  former,  then  an  cedematous  condition  must 
develop.  We  should  then  expect  a  priori  to  find  that  lymphatic  obstruc- 
tion is  a  potent  cause  of  oedema.  But  this  is  not  the  case.  The  main 
lymphatics  of  a  part  may  be  ligatured  or  compressed,  and  yet,  as  a  rule, 
no  cedema  occurs.  Even  when  the  thoracic  duct  is  ligatured,  ascites 
may  develop,  though  slowly,  yet,  as  Cohnheim  showed,  oedema  does 
not  result.  Two  factors  are  responsible,  namely,  the  existence,  in  many 
cases,  of  abundant  collateral  channels,  and  the  reabsorption  of  the 
tissue  lymph  into  the  bloodvessels.  And  the  latter  would  seem  the  more 
important — so  important,  in  fact,  that  we  seem  justified  in  regarding 
the  lymph  channels  not  as  the  prime,  but  as  an  accessory  factor  in  tissue 
drainage,  with  the  additional  function  of  removing  selectively  certain 
products  of  metabolism.  Even  during  the  process  of  bleeding  an  animal 
to  death,  the  last  portions  of  blood  are  much  more  watery  than  the  first, 
a  fact  which  can  only  be  explained  by  the  passage  of  tissue  fluid  into  the 
circulation  through  the  capillary  walls.  And  as  shown  by  Roy  and  Lloyd 
Jones,  after  less  extreme  hemorrhage,  the  specific  gravity  of  the  tissues 
becomes  rapidly  raised,  that  of  the  blood  diminished.  The  exchange 
of  fluid  between  the  surrounding  tissues  and  the  blood  may,  nay,  must, 
be  most  considerable.  This,  first  demonstrated  by  Magendie,  has  been 
convincingly  shown  by  Starling  and  Tubby.1 

Methylene  blue  or  indigo  carmine  injected  into  the  pleural  cavity 
appeared  in  the  urine  within  five  minutes,  whereas  the  lymph  presented 
no  trace  of  coloration  for  another  twenty  minutes,  or  it  might  be  two 
hours.  Lymphatic  obstruction  alone  is  thus  little  likely  to  cause  oedema. 
It  is  true  that  occasionally  we  meet  with  this  condition  following  obstruc- 
tion; thus,  secondary  cancer  of  the  axillary  glands,  with  the  not  infrequent 
extension  of  the  malignant  growth  along  the  lymph  channels,  or  extensive 
removal  of  the  axillary  lymphatic  chain,  may  be  followed  by  oedema  of 
the  arm.  But  this  is  not  a  necessary  outcome,  and  where  it  occurs  we 
must  conclude  that  hydremia,  or  a  toxic  condition  of  the  blood,  with 
altered  state  of  the  capillary  endothelium,  is  superadded. 

As  already  noted,  where  there  is  lymphatic  obstruction  there  the 
vessels  behind  the  obstruction  are  apt  to  become  dilated;  and  from  these 
dilated  vessels  it  is  evident  that  fluid  may  escape  into  the  tissues  and 

1  Jour,  of  Physiol.,  16: 1894: 140. 


INFLAMMATORY  (EDEMA  HI 

spaces  of  the  body.  This  is  often  to  be  determined  where  there  has  been 
cancerous  or  tuberculous  growth  involving  the  region  of  the  receptaculum 
chyli.  Radiating  from  the  affected  area  are  distended  lymph  vessels, 
or  white  streaks,  filled  with  semisolid  inspissated  lymph,  consisting  of 
fat  droplets  and  cell  debris.  This,  it  may  be  noted,  only  in  the  upper 
abdominal  area;  to  our  knowledge,  this  inspissation  is  never  encountered 
in  cases  of  lymphangiectasis  of  the  limbs  or  face — a  further  evidence  of 
the  selective  activity  of  the  lymph-vascular  endothelium. 

Chylous  Ascites. — Did  the  lymph  vessels  communicate  freely  with 
the  tissue  spaces,  we  should  expect  ascitic  fluid  to  approximate  in 
composition  to  the  chyle  in  the  abdominal  lymphatics;  but  this  is 
notoriously  not  the  case  under  ordinary  circumstances.  Under  abnormal 
conditions  we  obtain  this  approximation.  A  condition  of  true  chylous 
ascites  is  found  in  cases  of  rupture  of  the  receptaculum  chyli,  the  fluid 
affording,  upon  analysis,  a  relatively  high  percentage  of  proteins  and 
fat,  the  latter,  though  small  in  amount,  giving  it  a  milky  appearance. 
Rupture  of  the  thoracic  duct  may  similarly  lead  to  chylous  hydro- 
thorax  (of  the  renal  or  bladder  lymphatics  in  filariasis  and  other  states, 
to  chyluria).  The  thoracic  and  pleural  accumulations  may  occur  not 
only  through  trauma,  but  through  inflammatory  erosion,  or  sometimes 
as  the  result  of  obstruction  and  dilatation. 

Chyliform  Ascites. — The  above  condition  is  with  some  difficulty  to 
be  distinguished  from  one  seen  occasionally  in  low  forms  of  serous 
peritonitis,  most  frequently  due  to  abdominal  carcinoma  or  tuberculosis, 
in  which  the  fluid  becomes  milky  from  fat  liberated  by  the  breaking  down 
of  leukocytes  and  endothelial  cells  which  have  undergone  fatty  degenera- 
tion. The  percentage  of  fat  is  in  general  high  (as  high  as  6  per  cent, 
in  some  cases,  compared  with  0.5  per  cent,  and  less  in  true  chylous 
ascites).  Sugar  and  diastatic  enzymes,  frequently  to  be  detected  in 
the  former  condition,  are  absent  in  this. 

Pseudochylous  Ascites. — More  frequent  than  either  of  these  states 
is  that  termed  pseudochylous  ascites,  in  which  the  milky  fluid  simulating 
chyle  is  found  to  be  free  from  fats.  There  has  been  much  obscurity 
as  to  the  cause  of  the  turbidity;  in  some  cases  it  is  apparently  due  to 
the  presence  of  mucoid  substances;1  in  others,  it  would  seem,  to  partly 
dissolved  proteins.2  Joachim  ascribes  it  to  a  combination  of  lecithin  and 
pseudoglobulin.3 

3.  Inflammatory  (Edema. — In  this  group  of  cedemas  either  we  can 
recognize  under  the  microscope  changes  in  the  endothelial  lining  of  the 
capillaries,  to  which  we  ascribe  largely  the  increased  transudate  (exudate), 
or  by  analogy  we  hold  that  changes  of  like  order  must  obtain.  The  type 
example  is  to  be  seen  in  acute  inflammation.  In  this,  in  addition  to 
arterial  and  capillary  dilatation,  the  capillary  endothelium  is  swollen 
and  more  prominent,  and  there  are  other  indications  of  alteration  in  the 

1  Gourand  and  Corset,  Compt.  Rend.  Soc.  Biol.,  60:1906:23. 
2Poljakoff,  Fortschr.  d.  Med.,  21:1903:1081. 
3  Joachim,  Biochem.  Centralbl,  1 : 1903 : 437. 


112  (EDEMA 

state  of  these  cells,  such  as  adhesion  of  the  leukocytes  (suggesting, 
as  Wells  has  pointed  out,  an  altered  surface  tension),  and  nuclear 
enlargement  and  proliferation.  Associated  with  these  various  changes 
we  find  increased  pouring  of  fluid  from  the  tissues  along  the  lymphatics, 
higher  proteid  content  of  the  discharged  lymph,  and  increased  cell 
contents,  both  leukocytes  and,  it  may  be,  red  corpuscles.  Such  modified 
transudate  is  termed  an  exudate.  In  constitution  it  approaches  more 
nearly  to  the  blood  plasma  than  does  congestive  oedema  fluid.  It  has, 
however,  to  be  admitted  that  in  different  inflammations  we  find  every 
transition  from  fluid  of  the  one  type  to  the  other,  and  that  in  practice 
it  is  at  times  practically  impossible  to  state  with  precision  whether  we 
deal  with  a  hydrothorax,  for  example,  or  a  mild  form  of  serous  pleurisy. 

There  can  be  little  doubt  regarding  physical  changes  in  the  capillaries 
being  responsible,  to  a  very  large  extent,  for  the  increased  transudate:  the 
increased  intracapillary  pressure,  the  dilatation  and  thinning  of  the 
capillary  walls,  the  dilatation  of  the  stigmata  of  the  vessels,  or  formation 
of  stomata  or  passages  where  the  leukocytes  and  red  corpuscles  pass  out. 
Such  gross  openings  in  the  capillary  wall,  when  present,  must  necessarily 
cause  the  exudate  to  approximate  in  composition  to  the  blood  plasma. 
At  the  same  time,  the  indications  of  reactive  change  in  the  endothelial 
cells  at  least  suggest  the  co-existence  of  some  amount  of  selective  activity 
on  the  part  of  the  endothelial  membrane,  while,  at  the  same  time,  the 
enlargement,  nuclear  and  cytoplasmic  changes  in  the  tissue  cells  of 
the  inflamed  area  suggest  an  altered  interchange  between  them  and  the 
tissue  lymph,  and  that  the  resultant  lymph-vascular  lymph  is  much  more 
than  the  result  of  mere  filtration,  diffusion,  and  osmosis. 

4.  Toxic  (Edema. — Intimately  allied  to  this  last  is  the  group  of 
toxic  cedemas.  As  Heidenhain  demonstrated,  there  is  a  class  of  sub- 
stances which  act  as  lymphagogues,  i.  e.,  circulating  in  the  blood,  set 
up  increased  lymph  formation  in  sundry  areas.  Some  of  these  set  up 
alterations  in  blood  pressure  which  may  be  an  important  factor,  but 
others  (curare,  extract  of  mussels,  crab,  etc.)  induce  lymph  flow  in  the 
absence  of  noticeable  change  in  the  circulation.  The  logical  conclusion 
is  that  they  act  directly  on  the  vascular  endothelium  of  certain  areas, 
modifying  its  properties.  It  is  the  custom  to  describe  these  bodies  as 
having  a  toxic  action;  the  difficulty  is  where  to  draw  the  line  between 
stimulation  and  irritation.  Thus,  it  may  be  laid  down  as  a  rule  that 
increased  glandular  activity  is  accompanied  by  marked  increase  in  lymph 
flow.  This  is  notably  the  case  in  the  liver.  It  is  scarcely  to  be  imagined 
that  when  in  the  course  of  its  normal  function  the  liver  removes  dele- 
terious agents  from  the  portal  blood,  we  are  dealing  with  a  diseased,  toxic 
state  of  the  capillary  endothelium.  Rather  we  must  conclude  that  that 
possesses  a  selective  capacity;  that  certain  substances  contained  in  the 
portal  blood  stimulate  it,  and  that  only  when  these  are  in  excess  is 
there  an  overs timulation  and  toxic  state  of  the  cells  induced.  While 
saying  this,  we  have  also  to  recognize  that  lowered  vitality  of  the  endo- 
thelium is  accompanied  by  increased  passage  out  of  fluid.  We  need  only 
recall  the  difference  in  tra.nsudation  between  living  and  dead  membranes, 


NEUROPATHIC  (EDEMA  113 

A  well-marked  example  of  this  order  of  toxic  oedema  is  seen  in  the 
so-called  ischemic  oedema  supervening  after  prolonged  lowering  of 
the  blood  supply  of  a  part  after  ligature,  compression  (as  by  Esmarch's 
bandage),  or  frostbite.  In  these  cases,  clearly,  the  inadequate  blood 
supply  has  deleteriously  affected  the  endothelium,  and  now,  upon  the 
circulation  being  resumed,  the  escape  of  fluid  into  the  tissues  is  extreme. 
But,  admittedly,  it  is  difficult  to  determine  in  every  case  which  of  these 
two  conditions  we  have  to  deal  with.  Thus,  we  are  still  undetermined 
where  to  place  one  of  the  most  common  examples  of  widespread  oedema 
and  anasarca,  namely,  the  oedema  of  acute  parenchymatous  nephritis. 
This  differs  from  the  oedema  of  heart  disease  in  its  relatively  rapid  onset 
and  in  its  primary  distribution.  Thus,  a  favorite  seat  for  its  early  appear- 
ance is  in  the  loose  tissue  of  the  orbit,  resulting  in  a  puffiness  around  the 
eyes.  Congestion  and  alteration  in  blood  pressure  cannot  explain  its 
development,  and  the  experiments  of  Cohnheim  and  others  prove 
definitely  that  mere  hydremia  will  not  reproduce  the  condition.  We 
can  only  conclude  that  in  consequence  of  the  renal  incompetence  certain 
toxic  substances  circulating  in  the  blood  have  a  more  or  less  specific 
action  upon  the  vascular  endothelium  of  certain  areas.  But  what  is 
the  nature  of  this  action  we  do  not  know — whether  depressant,  lowering 
the  vitality,  or,  on  the  contrary,  stimulant,  leading  to  the  active  absorption 
and  removal  of  fluid  plus  toxic  substances  from  the  blood  stream. 
The  matter  has  to  be  left  sub  judice. 

5.  Neuropathic  (Edema. — Here,  in  connection  with  oedema,  as 
with  inflammation  and  so  many  other  processes,  we  find  that  nervous 
influences  alone  can  set  up  disturbances  of  the  same  type  as  those  due 
to  local  irritation.  We  may  encounter  (1),  as  we  have  already  pointed 
out  (vol.  i,  p.  410),  a  collateral  or  sympathetic  oedema  in  the  areas  inner- 
vated from  the  same  region  of  the  brain  or  cord  as  that  controlling  a 
focus  of  acute  inflammation,  or  (2),  in  association  with  definite  nerve 
lesions,  may  find  oedema  of  particular  fields  of  •  nerve  supply,  as  in 
herpes  zoster.  But  in  addition  to  these,  and  unassociated  with  any 
apparent  inflammatory  disturbance,  we  meet  with  a  remarkable  series 
of  angioneurotic  oedemas  exhibiting  sudden  and  acute  pouring  out  of 
fluid  into  particular  areas,  for  which  we  can  ascribe  no  cause  other  than 
nervous  influence  upon  the  vessels  of  the  part.  It  is  true  that  the  line 
distinguishing  these  from  the  urticarias  of  cases  of  idiosyncrasy  is  not 
always  easy  to  draw.  In  the  latter  the  exhibition  of  certain  foods,  even 
in  minute  amounts,  leads  to  local  vascular  disturbances  with  infiltration 
and  subcutaneous  or  submucous  swelling.  Underlying  this  there 
would  seem  to  be  a  neurotic  influence,  though  the  suggestion  has  of 
late  received  credence  that,  as  in  serum  sickness,  some  substance 
present  in  the  circulating  blood  in  minute  quantities  has  a  specific 
Lymphagogue  action  upon  the  endothelium  of  the  vessels  of  particular 
regions.  But  as  in  hay  fever,  and  in  those  severely  affected  by  the  mere 
presence  of  a  cat  in  their  neighborhood,  acute  local  oedematous  condi- 
tions may  be  induced  by  influences  acting  through  the  respiratory 
system  which  it  is  difficult  to  attribute  to  any  but  pure  reflex  nervous 
8 


114  (EDEMA 

influence,  with  vascular  instability  of  particular  regions.  An  important 
group  of  spasmodic  asthmas  would  seem  to  come  into  this  category, 
at  least  in  part,  due  not  merely  to  constriction  of  the  bronchial  muscula- 
ture, but  also  to  sudden  congestion  and  oedema  of  the  bronchial  mucous 
membrane.  It  would  thus  seem  necessary  to  distinguish  a  class  of  pure 
angioneurotic  cedemas  from  that  of  the  idiosyncratic  cedemas  of  what, 
failing  a  better  term,  we  must  still  refer  to  as  toxic  in  origin. 

FIG.  14 


Urticaria  factitia  (angioneurosis).      (Hyde  and  Ormsby.) 

6.  Hydrops  ex  Vacuo. — Lastly,  reference  must  be  made  to  the 
condition  in  which,  with  atrophy  of  tissues,  there  is  replacement  by 
lymphatic  fluid.  Practically  the  only  region  in  which  this  is  recognizable 
is  in  the  brain  case  and  vertebral  canal  following  upon  shrinkage  of  the 
brain  and  cord.  Here  clearly  the  lack  of  pressure  must  be  the  main 
reason  why  the  extravasated  cerebrospinal  fluid  fails  to  be  discharged 
into  the  lymph  vessels,  and  as  a  consequence  accumulates.  Allied  to 
this  is  what  we  may  term  the  replacement  hy  drops,  seen  in  the  develop- 
ment of  necrotic  and  hemorrhagic  cysts  (vol.  i,  p.  795). 

From  the  above  recital  it  is  obvious  that  (1)  the  time  has  not  yet 
arrived  to  lay  down  any  broadly  simple  laws  regarding  the  nature  of 
oedema,  and  that  (2)  we  must  recognize  a  varying  interaction  of  several 
factors — blood  pressure  and  filtration,  diffusion,  osmosis,  the  selective 
activity  of  the  vascular  endothelium,  the  tissue  cells,  and,  it  may  well  be, 
the  endothelium  of  the  lymph  vessels.  Much  has  still  to  be  done  before 
we  can  speak  with  precision  of  the  relative  value  of  these  different 
agencies. 


CHAPTER    VI. 

THE  CARDIO-VASCULAR  SYSTEM. 
CARDIAC  FUNCTION  AND  ITS  DISTURBANCES. 

WE  must  take  for  granted  a  knowledge  of  the  gross  anatomy  of  the 
heart,  here  merely  recalling  (1)  that  the  weak  muscular  tissue  of  the 
auricles  is  independent  of  the  strong  musculature  of  the  ventricles  save 
for  a  small  connecting  bundle  first  noted  by  Stanley  Kent,  and  later  by 
the  younger  His.  As  we  shall  see,  this  bundle  plays  an  important  part 
in  the  regulation  of  the  heart  beat;  (2)  that  the  musculature  of  the 
ventricles  is  not  entirely  independent,  there  being  an  extensive  crossing 
of,  more  particularly,  the  outer  bundles  from  the  one  ventricle  to  the 
other,  whereby  it  comes  to  pass  that  the  work  of  the  two  ventricles  must 
be  largely  synchronous,  and  (3)  that  we  have  evidence  of  the  existence 
of  both  motor  and  sensory  nerves  in  association  with  this  organ.  Fibers 
pass  to  the  root  of  the  heart  from  both  vagi,  others  again  from  the  upper 
dorsal  sympathetic  ganglia;  and  by  physiological  methods  we  have 
determined  the  existence  and  some  of  the  functions  of  nerve  bundles  of 
different  orders. 

As  bearing  upon  disturbed  function,  certain  considerations  regarding 
the  physiology  of  the  heart  demand  somewhat  fuller  treatment.  As 
a  mechanism  for  the  propulsion  of  the  blood,  we  observe  in  that  mechan- 
ism (1)  chambers  for  the  collection  of  the  blood;  (2)  the  motor  apparatus 
proper  for  the  propulsion  of  the  blood;  (3)  valves  to  determine  that  the 
blood  is  discharged  in  a  particular  direction,  so  that  the  heart  being  inter- 
polated in  a  system  of  closed  vessels,  a  circulation,  and  not  merely  a  flow 
and  ebb  of  the  blood,  is  assured. 


1.  THE  CARDIAC  CHAMBERS. 

The  heart  is  a  double  pump,  each  moiety  consisting  of  two  chambers, 
or  more  accurately,  of  an  auricular  antechamber  leading  into  the  ven- 
tricular chamber,  or  pump  proper.  Undoubtedly,  the  auricles,  through 
the  muscle  in  their  walls,  do  act  as  pumps  propelling  the  blood;  nay, 
more,  we  possess  ample  evidence  that  the  normal  cardiac  contraction 
originates  at  the  venous  ostia,  where  the  venae  cavse  open  into  the  right 
ventricle,  and  that  the  auricular  muscle  is  essential  for  the  due  convey- 
ance of  the  wave  of  contraction  from  this  region  to  the  ventricles. 
Further,  there  is  evidence  that  in  certain  states  the  auricular  pulse 
wave  is  demonstrable  in  the  arterial  pulse,  or,  otherwise,  the  effect  of 


116 


THE  CARDIAC  CHAMBERS 


the  contraction  of  the  auricular  muscle  is  then  sufficiently  powerful 
to  be  conveyed  through  the  ventricular  blood  into  the  column  of  aortic 
blood.  But  admitting  this,  it  has  also  to  be  admitted  that  the  mus- 
cular contraction  of  the  auricular  walls  is  not  the  dominant  force  lead- 
ing to  the  filling  of  the  ventricles.  It  is  but  necessary  to  observe  the 
auricles  of  the  exposed  mammalian  heart  to  note  that  their  contraction 
is  incomplete;  that  it  does  not  empty  the  auricles  at  each  beat.  What 
is  more,  we  have  accumulated  increasing  evidence  (see  p.  123)  that 

FIG.  15 


Thrombus  completely  filling  the  left  auricle  except  for  two  passages;  1  and  3  represent 
the  channels  from  the  right  and  left  pulmonary  veins. 

the  ventricular  expansion  is  active,  and  that  a  large  proportion  of  the 
blood  filling  the  ventricles  enters  it  before  the  auricular  systole  occurs; 
in  other  words,  that  systole  is  not  absolutely  essential  for  the  onward 
passage  of  the  blood;  at  most,  when  through  the  progressive  filling  and 
expansion  of  the  ventricle  the  active  diastole  is  becoming  weak,  the 
auricular  contraction  acts  as  a  final  but  auxiliary  force  whereby  the  intra- 
ventricular  blood  pressure  is  raised,  and  thereby  (we  would  hold)  the  auto- 
matic or  idiomuscular  contraction  of  the  ventricular  muscle  is  stimulated, 
or  perhaps,  more  accurately,  the  muscle  fibers  are  placed  in  that  tejnse 


ABNORMALITIES  1 17 

state  which  favors  contraction  following  upon  a  minimal  stimulus. 
There  are,  indeed,  cases  in  which  obviously  the  auricles  are  incapable  of 
exciting  any  propulsive  action,  and,  nevertheless,  the  circulation  continues. 
Thus  we  have  seen  the  left  auricle  completely  filled  and  distended  with 
blood  clot  save  for  two  passages  leading  from  the  right  and  left  pul- 
monary veins  respectively  to  the  ventral  orifice.  In  this  case,  judging 
from  the  appearance  of  the  thrombus,  the  auricle  must  have  been  out 
of  action  for  days  rather  than  hours;  nevertheless,  the  circulation  was 
not  noticeably  impeded.  And,  as  Hoover  points  out,  there  are  not  a  few 
cases  recorded  by  veterinarians  in  which  the  auricles  in  the  horse  have 
been  found  completely  rigid  from  universal  calcification.  Thus  we  are 
inclined  to  regard  the  auricles  as  essentially  antechambers  for  the 
gradual  collection  of  blood  from  the  veins  prior  to  the  active  diastole  of 
the  ventricles.  Did  these  antechambers  not  exist;  did  the  thin-walled 
and  collapsible  veins  open  directly  into  the  actively  expanding  ventricles, 
then  the  suction  force  exerted  by  the  ventricles  would  tend  to  approxi- 
mate the  venous  walls,  and  arrest  the  circulation  into  the  ventricles. 
These  collecting  chambers  prevent  any  such  catastrophe. 

Use  of  the  Ventricles. — -These  are  essentially  the  pumping  apparatus 
of  the  circulating  system,  and  as  such  their  functions  are  best  considered 
under  the  next  heading.  Here  we  would  only  note  that  their  walls  are 
far  from  rigid,  but  elastic,  and  that,  just  as  if  we  attach  a  weight  to  a 
resting  band  of  muscle,  such  as  the  gastrocnemius  of  a  frog,  we  find  that 
the  band  undergoes  progressive  elongation,  so,  similarly,  if  through  posi- 
tive pressure  increasing  volumes  of  fluid  seek  to  gain  unimpeded  entrance 
into  the  ventricles,  or,  to  express  it  otherwise,  if  a  column  of  blood  of 
increasing  length  becomes  connected  with  the  ventricular  cavity,  this  has 
the  same  result — the  resting  muscle  expands  and  the  chamber  undergoes 
distension.  We  shall  have  more  to  say  regarding  this  when  we  come 
to  consider  the  subjects  of  hypertrophy  and  dilatation. 

Abnormalities. — The  most  serious  disturbances  of  the  heart,  con- 
sidered as  a  series  of  chambers,  occur  in  connection  with  certain  congenital 
vices  of  development.  Although  the  heart  develops  into  the  two  distinct 
pumping  systems,  right  and  left,  already  noted,  it  must  be  recalled  that 
at  an  early  stage  it  exists  in  the  embryo  as  a  two-chambered  organ  with 
a  single  auricle  and  a  single  ventricle.  By  a  very  complicated  series  of 
outgrowths  there  develop  septa  dividing  each  of  those  primary  chambers 
into  two;  so  that  the  normal  state  after  birth  is  that  the  right  heart 
is  completely  closed  off  from  the  left.  Up  to  the  time  of  birth  the  sepa- 
ration between  the  two  auricles  is  wanting  to  this  extent,  that  there 
exists  a  passage  or  foramen  (the  foramen  ovale)  whereby  the  blood  from 
the  inferior  vena  cava  (more  particularly)  crosses  the  right  auricle 
and  enters  the  left.  In  quite  a  large  proportion  of  cases  this  undergoes 
incomplete  postnatal  closure;  in  a  few  cases  it  remains  widely  open; 
or  there  may  be  yet  more  incomplete  formation  of  the  interauricular 
septum.  In  all  these  cases  there  is  the  possibility  of  mingling  of  the  venous 
blood  entering  the  right  heart,  with  the  oxygenated  blood  entering  the 
left.  The  same  is  liable  to  happen  when  the  interventricular  septum 


118  THE  MOTOR  APPARATUS 

is  imperfect.  Yet  graver  disturbances  ensue  when,  through  abnormal- 
ities in  the  division  of  the  originally  single  vessel  of  discharge,  we  gain 
a  long  series  of  anomalies,  from  mere  narrowing — congenital  stenosis — 
of  either  pulmonary  artery  or  aorta  to  conditions  of  complete  "Rechts- 
lage,"  in  which  the  systemic  arteries  are  supplied  with  blood  from 
the  right  heart,  the  pulmonary  arteries  with  blood  from  the  left.  These 
abnormalities  in  the  growth  of  the  arterial  septum  downward  are  very 
frequently  associated  with  incomplete  development  of  the  interventricular 
septum  upward,  so  that  there  is  combined  free  communication  between 
the  two  ventricles,  with  consequent  admixture  of  venous  and  arterial 
blood. 

2.  THE  MOTOR  APPARATUS. 

Here  we  deal  essentially  with  the  ventricles,  and  of  these,  the  left 
ventricle  more  particularly  concerns  us. 

Arrangement  of  the  Muscle  Fibers.— In  the  first  place,  it  must  be 
noted  that  the  ventricle  does  not  contract  as  a  sphere  and  thus  become 
narrowed  in  every  direction.  The  successive  layers  of  muscle  fibers 
are  so  arranged  that,  with  contraction,  the  length  of  the  left  ventricle 
is  practically  unaltered.  The  form  of  this  ventricle  and  mode  of  con- 
traction are  such  that  in  systole  singularly  little  internal  pressure  is 
exerted  upon  the  apex.  It  is  a  fact  which  we  think  is  not  generally 
known  that  the  total  thickness  of  the  ventricular  wall  at  the  apex  of 
the  heart  is  little  more  than  one-eighth  of  an  inch.  The  arrangement 
of  the  fibers  and  the  mode  of  their  contraction  is  such  that  the  walls 
of  the  apical  half  are  brought  together  and  the  blood  propelled  upward, 
i.  e.,  toward  the  aorta.  Another  fact  not  generally  recognized  is  that 
the  left  ventricle  never  becomes  completely  emptied;  there  is  always 
left  some  of  what  Roy  and  Adami  have  termed  residual  blood.  As 
may  be  determined  by  inserting  the  little  finger  through  a  small  slit 
in  the  apex  of  a  large  animal  that  has  been  curarized,  the  lower  half  of 
the  ventricle  contracts  tightly  round  it  in  systole,  but  above  the  level  of 
the  apices  of  the  papillary  muscles  there  is  a  persistent  chamber. 

Two  accessory  muscle  systems  are  to  be  noted,  the  papillary  (Albrecht), 
whose  function  is  to  keep  taut  the  mitral  valve  and  prevent  this  from 
becoming  everted  into  the  auricle;  the  fibers  from  this  pass  downward, 
then  laterally  and  forward  and  to  the  surface  of  the  septum;  and  the 
ring  musculature  (Krehl),  controlling  the  upper  orifice  of  the  ventricle. 

The  contraction  of  the  right  ventricle  is  not  so  complete;  the  shape 
on  transverse  section  is  that  of  a  crescent  applied  to  the  more  circular 
left  ventricle.  The  result  of  contraction  is  that  the  outer  wall  becomes 
approximated  to  the  inner,  a  process  aided  by  the  bands  of  muscle  which 
pass  across  the  cavity. 

Nature  of  the  Cardiac  Contraction:  Systole. — Our  conception 
of  muscular  contraction  is  naturally  based  upon  what  we  know  regarding 
the  contraction  of  the  skeletal  muscles.  In  them  we  know  that  a 
nervous  stimulus  is,  under  ordinary  conditions,  the  originator  of  the 


THE  MYOGEN1C  THEORY  119 

contraction,  and  we  are  apt  to  neglect  Sherrington's  observation  on  the 
nature  of  the  patellar  and  other  reflexes,  that  these  occur  so  rapidly 
after  the  blow  that  induces  them  that  they  can  only  be  explained  as  the 
direct  response  of  the  muscle  fibers  to  a  sudden  strain,  and  cannot  be 
of  nervous  origin.  Thus  it  is  that  the  early  view  of  the  cardiac  contrac- 
tion was  that  each  systole  was  the  result  of  a  nervous  impulse. 

The  Myogenic  Theory. — We  owe  more  particularly  to  Gaskell 
(1883)1  the  view  which  prevails  to-day — that  the  cardiac  contraction  is 
automatic,  or,  more  accurately,  is  myogenic  rather  than  neurogenic, 
the  muscle  directly  responding  to  stimuli,  the  contraction  stimulus 
travelling  not  by  the  nerves,  but  along  the  muscle  cells.  Thus,  in  the 
embryo,  the  primitive  heart  is  the  earliest  organ  to  present  active  function, 
and  that  long  before  there  is  any  sign  of  the  development  of  the  peripheral 
nerves.  We  see  it  there  as  a  tube  bent  upon  itself,  and  undergoing  rhyth- 
mical contraction,  contractions  which  begin  at  the  one  end,  and  continue 
as  a  peristaltic  wave  to  the  other.  And  still,  in  the  fully  developed  higher 
animal,  it  is  seen  that  the  wave  of  contraction  begins  at  the  region  corre- 
sponding with  the  sinus  venosus,  or  first  portion  of  the  more  tubular 
heart  of  the  embryo  and  of  lower  forms  of  life.  The  earlier  difficulty 
in  understanding  the  conveyance  of  the  wave  from  one  chamber  to  the 
other  in  the  apparent  absence  of  any  muscular  band  of  connection  be- 
tween auricles  and  ventricles  has  been  solved  by  the  important  discoveries 
of  late  years  with  which  one  associates  the  names  of  Stanley  Kent,2 
the  younger  His,3  Aschoff  and  Tawara,4  Erlanger,5  and  Keith  and  Flack.6 
Such  band  of  connection  does  exist,  and  is  of  a  very  remarkable  character. 
Thus,  in  1893  the  first  two  of  these  observers  demonstrated  the  exist- 
ence of  a  peculiar  band  of  fibers  of  muscular  type,  which,  beginning 
apparently  in  a  small  node  in  the  wall  of  the  right  auricle  near  the  coro- 
nary sinus,  continues  downward  into  the  ventricles.  The  course  of  these 
fibers  has  been  very  thoroughly  studied  by  Tawara,  working  under  Aschoff, 
who  has  found  that  forming  a  node  above  the  auriculoventricular  junction, 
the  band  divides  into  two  main  branches,  one  for  each  ventricle,  and  each, 
becoming  superficial,  branches  in  the  subendothelial  tissue,  important 
branches  passing  to  each  papillary  muscle.  The  cells  forming  these  sub- 
endothelial fibers  had  hitherto  been  known  as  Purkinje  cells,  and  had 
been  regarded  as  a  layer  of  vegetative  muscle  cells  (Fig.  15).  Whether 
there  exist  two  orders  of  cells  in  this  position,  those  of  the  conducting 
system,  and  myoblasts,  has  still  to  be  determined.  The  cells  of  this 
system,  it  may  be  added,  are  not  ordinary  muscle  cells — they  are  rela- 

1  German  writers  are  apt  to  give  the  credit  to  Engelmann,  whose  work  was  later, 
as  also,  it  may  here  be  noted,  they  credit  His,  Jr.,  rather  than  the  earlier  English 
observer  Kent,  with  the  discovery  of  the  auriculoventricular  bundle. 

2  Journal  of  Physiology,  14:  1893:  229. 

3  Arbeiten  a.  d.  med.  Klinik  zu  Leipzig,  1883:21. 

4  Das  Reizleitung  system  der  Saugethiere,  Jena,  Fischer,  1906. 

5  Jour,  of  Exp.  Med.,  7:1905;  see  also  Erlanger  and  Blackman,  Amer.  Jour,  of 
Physiol,  19:  1907. 

6  Journ.  Anat.  and  Physiol.  (Proc.  Anat.  Soc.  Great  Britain),  37:  1903. 


120  THE  MOTOR  APPARATUS 

lively  large  and  clear,  with  only  occasional  sarcous  elements;  they  recall, 
in  fact,  cells  of  a  more  embryonic  type.  More  recently  Keith  and  Flack 
have  demonstrated  a  sino-auricular  ring  or  node  of  cells  of  similar  order 
situated  at  the  opening  of  the  superior  vena  cava  into  the  right  auricle. 
The  connection  of  these  with  the  auriculoventricular  node  has  still 
to  be  worked  out. 

The  significance  of  these  observations  is  shown  by  experiments  in 
which  (a)  the  sinus  region  is  by  pressure  cut  off  from  the  auricle, 
or  (6)  the  auricles  separated  from  the  ventricles.  The  result  in  either 
case  is,  at  first,  arrest  of  the  heart  wave  beyond  the  point  of  stricture. 
After  a  certain  period,  in  the  first  experiment  the  auricles  begin  to  beat 
again,  but  at  a  rate  slower  than  that  of  the  sinus,  and  in  the  second,  the 
ventricles  begin  contracting,  but  at  a  rate  slower  than  that  of  the  auricles, 
or  otherwise  the  individual  parts  of  the  heart  are  capable  of  exhibiting 
automatic  contraction,  but  ordinarily  the  stimulus  to  contraction,  origi- 

FIG.  16 


Purkinje's  fibers  from  a  sheep's  heart:   n,  nuclei;   c,  protoplasm;   f,  striated  muscular  substance. 
(After  Ranvier,  Legons  d' Anatomic  Ge'ne'rale  sur  le  Systeme  Musculaire,  Paris,  1880,  p.  300.) 

nating  in  the  sino-auricular  node,  is  conducted  to  the  auricular  walls, 
thence  to  the  auriculo-ventricular  node,  and  thence  to  the  ventricular 
muscles. 

Before  considering  the  significance  of  these  observations,  other  charac- 
teristics of  the  cardiac  contraction  have  to  be  briefly  referred  to.  In  the 
first  place  the  heart  muscle  differs  from  the  skeletal  in  this,  that  whereas 
the  latter  can  be  tetanized,  the  former  cannot.  That  is  to  say,  with 
ordinary  muscle  the  state  of  contraction  does  not  prevent  a  nervous 
electric  stimulus  still  influencing  the  fibers,  so  that  if  stimuli  be  repeated 
with  sufficient  frequency,  the  muscle  passes  into  a  state  of  persistent 
contraction.  The  heart  muscle,  on  the  other  hand,  exhibits  a  refractory 
stage  (Marey),  whereby  we  mean  that  when  once  in  the  state  of  con- 
traction it  is  refractory  to,  or  uninfluenced  by,  stimuli,  and  this  state 
persists  for  a  certain  period.  Associated  evidently  with  this  is  the  further 
characteristic  known  as  the  law  of  maximal  contraction,  that  the  weakest 
stimulus  adequate  to  produce  a  contraction  evokes  as  powerful  a  con- 


THE  NEUROGENIC  THEORY  121 

traction  as  the  strongest  (Bowditch).  This,  we  would  emphasize,  is 
not  the  same  as  saying  that  every  contraction  of,  say,  the  left  ventricle, 
is  of  equal  force.  There  are,  as  we  shall  point  out,  conditions  under 
which  the  refractory  period  may  be  prolonged — in  which,  therefore,  the 
amount  of  "  contractile  material"  becomes  increased,  and  the  subsequent 
contraction  is  therefore  more  powerful.  It  only  signifies  that  this  sub- 
sequent contraction  will  be  equally  powerful,  whether  incited  by  a  minimal 
or  a  maximal  stimulation;  that  the  explosion  of  the  keg  of  gunpowder 
will  be  equally  forceful,  whether  caused  by  the  scarce  glowing  stump  of 
a  match  or  a  2000  volt  electric  current;  the  amount  of  gunpowder  in 
the  keg  will,  however,  modify  matters.  According,  therefore,  to  the 
myogenic  theory,  the  "  explosion"  of  the  cells  of  the  sino-auricular  node 
— and  of  the  heart  muscle  fibers  in  succession — is  followed  by  a  period 
of  resuscitation  of  the  contractile  matter,  and  with  this  accumulation 
the  cells  become  more  and  more  excitable  until  some  stimulus,  not 
nervous — it  may  be  the  strain  put  upon  the  cells  by  the  accumulation 
and  presence  of  the  blood  in  the  auricle — induces  another  explosion. 
Or,  again,  it  may  be  that  the  very  excitability  of  the  elements  which  go  to 
form  the  contractile  material,  leads,  as  they  become  stored  up,  to  an 
active  rearrangement  of  these  elements.  This  is  still  undetermined. 

The  Neurogenic  Theory.— What  part,  then,  is  played  by  the  nervous 
mechanism  of  the  heart,  for  this,  as  we  have  pointed  out,  definitely  exists, 
both  nerves  coming  from  without,  and  intrinsic  ganglion  cells  in  the 
auricular  septum  and  elsewhere.  The  vagus  fibers,  we  know,  can  com- 
pletely arrest  the  heart,  but  with  more  moderate  power  of  stimulation 
they  slow  the  heart  beat,  increase  the  refractory  period,  so  that  the 
individual  contractions  are  fewer  in  number,  but  each  individual  beat 
more  powerful,  although  in  a  given  period  the  main  result  is  that  the  work 
done  by  the  heart  is  lessened.  The  accelerator  fibers,  on  the  other 
hand,  reduce  the  refractory  period,  so  that  the  beats  succeed  each  other 
with  greater  rapidity.  At  the  same  time  they  appear  to  stimulate  the 
increased  formation  of  the  contractile  material,  as  shown  by  the  fact  that 
the  work  accomplished  by  the  heart  in  a  given  time  is  increased.  For 
this  reason  they  have  also  been  described  as  augmentor  fibers.1 

The  more  recent  work  of  Engelmann2  indicates  that  those  nerves  may 
control  the  heart  work  by  various  means.  Thus,  he  distinguishes  be- 
tween inotrope  influences  (causing  change  in  the  force  of  the  heart  beats), 
chronotrope  (causing  change  in  the  rate),  dromotrope  (causing  changes 
in  the  rate  with  which  the  wave  of  contraction  is  conveyed  from  one 
segment  of  the  heart  to  the  other),  and  bathmotrope  (causing  modifications 
in  excitability).  We  would  add  that  changes  other  than  nervous  may 
also  affect  the  heart  in  one  or  other  of  these  directions. 

As  already  indicated,  the  myogenic  theory  here  put  forward  is  still 
strongly  opposed  by  not  a  few  physiologists;  more  particularly 

1  Roy  and  Adami,  Phil.  Trans.  Roy.  Soc.,  Lond.,  183  B;  1892:199. 

2  Arch.  f.  Anat.  Physiol  (Physiol.  Abth.),  1900:  315,  and  Deutsche  Klinik,  4:  1903: 
215. 


122  THE  MOTOR  APPARATUS 

Kronecker  and  his  school  have  brought  forward  data  which  are  diffi- 
cult to  reconcile  completely  with  the  theory  of  muscular  convey- 
ance of  the  cardiac  wave.  Thus,  Kronecker  and  Imchanitzky  have 
shown  that  the  bundle  of  His  can  be  ligatured  without  disturbing  co- 
ordination between  the  auricles  and  ventricles,  and  Paukul  has  demon- 
strated the  existence  of  nerve  plexuses  accompanying  the  bundle,1  and 
has  found  that  if  these  be  injured,  incoordination  is  induced.  It  is  not, 
therefore,  according  to  this  school,  the  muscle  bundle  itself,  but  the 
accompanying  nerves  that  constitute  the  coordinating  mechanism. 

There  is  a  remarkable  condition  of  irregular,  independent  contraction 
of  the  heart  muscle  fibers  which  may  be  brought  about  in  various  ways 
in  animals  of  the  laboratory.  This  is  known  as  fibrillation.  If,  for 
example,  the  anterior  coronary  artery  of  the  dog's  heart  be  ligatured,  or 
puncture  made  into  a  particular  spot  in  the  interventricular  septum 
(Kronecker),  the  heart  passes  into  this  state,  and  from  regular  contractions 
takes  on  the  appearance  of  a  thin  bag  filled  with  actively  wriggling  worms. 
In  general  this  state  is  not  recovered  from,  and  from  arrest  of  circulation, 
death  ensues.  If,  now,  the  heart  be  treated  by  a  strong  fixative  agent 
when  in  this  state  of  fibrillation,  Imchanitzky2  has  shown  that  the  state 
of  striation  in  adjoining  cells  may  be  sharply  contrasted;  at  one  side 
of  the  dividing  line  between  two  cells  the  striae  may  be  widely  apart, 
at  the  other  close  together;  the  one  cell  in  a  state  of  expansion,  the 
other  of  tense  contraction.  The  appearances  are  certainly  not  those 
we  would  expect  to  find  were  there  the  conduction  of  the  contractile 
wave  from  one  cell  to  the  next  in  series.  This  argument,  however,  does 
not  strike  us  as  absolutely  convincing,  for  she  admits  that  there  may  be 
the  same  sudden  change  of  striation  in  the  course  of  a  single  cell.3 
On  the  other  hand,  the  earlier  observations  point  strongly  in  the  direction 
of  the  fine  plexus  of  nerves  interspersed  through  the  heart  musculature 
serving  as  the  main  coordinating  mechanism. 

To  reconcile  these  divergent  views,  we  would  again  have  recourse  to 
the  parable  of  the  coach  and  its  horses  and  the  driver  (vol.  i,  p.  449). 
We  cannot  but  think  that  under  normal  conditions  the  fibers  contract 
under  the  influence  of  the  "strain"  to  which  they  are  subjected,  although 
constantly  under  the  controlling  influence  of  the  nerve  plexus,  which  are 
to  the  cells  what  the  reins  and  bit  are  to  the  horses. 

Meltzer4  points  out  a  possible  means  of  reconciling  the  myogenic  and 
neurogenic  theories.  Tawara  has  described  in  the  atrioventricular 
bundle  the  existence  of  a  peculiar  mass  of  net-like  structure  containing 
both  nerve  cells  and  nerve  fibers  (Tawara's  node),  while  Keith  and 

1  It  deserves  note  that  Tawara  had  described  the  co-existence  of  nerve  fibers  in 
the  bundles. 

2  Arch.  Internat.  de  Physiol.,  4:  1906:  1.     This  article  gives  a  resume  of  the  liter- 
ature on  this  subject. 

3  The  same  phenomenon  has  been  observed  in  human  hearts  exhibiting  fragmenta- 
tion; it  may  be  the  indication  that  in  these  cases  death  has  been  due  to  the  heart 
assuming  the  state  of  fibrillation. 

4  Medical  Record,  75:  1909:  873.     An  excellent  resume  of  recent  studies. 


DIASTOLE  123 

Flack  have  discovered  a  similar  body  at  the  junction  of  the  superior 
vena  cava  and  auricle  (Keith's  node).  We  may  suppose  that  the  normal 
wave  of  cardiac  contractions  originates  in  Keith's  node,  and  that  under 
exceptional  circumstances  Tawara's  node  can  automatically  set  up  the 
ventricular  contractions  independent  of  the  more  proximal  sino-auricular 
node,  or  otherwise  the  contractions  originate  usually  in  the  heart  itself, 
but  through  the  agency  of  these  nerve-containing  centres. 

Parenthetically,  in  this  connection,  it  must  be  recalled  that  the  heart 
possesses  also  efferent  nerves.  These  are  not  sensory  nerves  proper, 
in  the  sense  that  we  normally  perceive  the  effects  of  their  stimulation. 
Normally,  that  is,  like  other  visceral  nerves,  stimuli  do  not  extend  beyond 
the  cord,  or  at  most  the  medulla,  there  setting  up  reflexes.  Only  in 
cases  of  more  severe  stimulation  of  certain  orders  do  we  in  lower  degree 
obtain  a  vague  sense  of  discomfort  that  cannot  be  accurately  localized, 
in  the  higher  degree  acute  pain,  which,  again,  is  not  sharply  localized, 
but  is  recognized  more  definitely  to  be  in  the  cardiac  region.  What  is 
more  marked  in  these  cases — in  angina  pectoris,  for  example — is  the 
"referred  pain;"  pain  referred  to  the  areas  innervated  by  the  first  and 
second  dorsal  nerves,  to  the  skin  over  the  upper  part  of  the  thorax, 
down  the  inner  side  of  the  left  arm  as  far  as  the  elbow,  more  rarely  down 
both  arms,  or  to  the  little  finger  or  fingers.  As  pointed  out  by  Sir  Lauder 
Brunton,  Harvey  knew  that  the  outer  wall  of  the  heart  is  insensitive 
to  touch.  It  is  either  anemia  of  the  ventricular  muscle,  or  distension 
from  within  that  induces  cardiac  pain.  The  referred  pain  is  an  irradia- 
tion effect.  Stimulation  of  the  specific  ganglion  cells  in  the  first  dorsal 
region,  if  extreme,  causes  an  overflow  to  neighboring  cells,  those  associ- 
ated with  tactile  and  other  cutaneous  sensations  of  the  first  dorsal  area; 
and  as  these  have  cerebral  connections,  stimuli  proceeding  along  them 
to  the  brain  are  referred  to  the  regions  they  innervate.  At  most,  efferent 
cardiac  stimuli  reach  normally  the  medulla.  It  may  be  questioned 
whether  in  cases  of  severe  stimulation  we  here  again  deal  with  irradiation 
effects,  or  whether  there  is  thence  a  normal  direct  path  to  the  cerebrum, 
which,  being  little  used,  does  not,  when  stimuli  pass  along  it,  convey  to 
us  an  accurate  sense  of  the  locality  of  the  primary  stimulation.  The 
majority  of  patients,  for  example,  cannot  surely  determine  whether  they 
experience  cardiac  or  gastric  pain.  These  considerations  apply  not  only 
to  cardiac  pain,  but  to  visceral  pain  in  general. 

Diastole. — Although,  as  we  have  pointed  out,  the  properties  of  the 
cardiac  muscle  fibers  differ  in  important  particulars  from  those  of  ordinary 
striated  muscle,  it  is,  nevertheless,  natural  that  our  conceptions  of  their 
mode  of  action  are  based  upon  our  knowledge  of  that  of  voluntary  muscle. 
Hence,  as  it  is  firmly  fixed  in  our  minds  that  muscular  contraction  alone 
is  an  active  process,  relaxation  being  passive,  for  long  the  tendency  has 
been  to  regard  diastole  as  passive,  as  a  mere  act  of  relaxation.  For 
long  there  have  been  observations  known  opposing  this  view,  such  as  the 
evidently  forcible  rounding  of  the  ventricles  in  diastole,  when  the  living 
and  beating  heart  of  an  animal  is  held  between  finger  and  thumb,  an 
expansion  too  forcible  to  be  explained  by  the  internal  blood  pressure 


124  THE  MOTOR  APPARATUS 

or  by  elastic  recoil.  So  long  ago  as  1885  Pawlow  demonstrated  that 
in  the  fresh  water  mussel  there  exists  a  muscle  which  expands  on  stimu- 
lation, contracting  when  that  stimulation  is  removed.  It  has,  however, 
been  difficult  if  not  impossible  to  demonstrate  the  active  nature  of 
muscular  expansion  in  warm-blooded  animals.  And  so  it  follows  that 
the  negative  pressure  which  can  often  be  determined  in  the  ventricular 
cavities  of  the  mammalian  heart  under  experimental  conditions  has  been 
ascribed  to  the  elasticity  of  the  heart  wall,  the  general  negative  pressure 
of  the  thoracic  cavity  during  inspiration  acting  as  an  adjuvant. 

Under  ordinary  conditions  of  experiment — e.  g.,  in  Holies  ton's  valuable 
studies  upon  the  intraventricular  pressure — it  is  difficult  to  demonstrate 
this  negative  pressure  as  constantly  existing  during  the  diastolic  phase, 
and  that  because,  coincident  with  the  expansion  of  the  ventricles,  the 
blood  pours  into  the  ventricles  under  definite  positive  pressure;  it  is 
only  when  this  flow  becomes  slowed  that,  prior  to  systole,  a  brief  period 
of  negative  pressure  shows  itself  on  the  curves.  Recently,  Stefani,1 
working  under  Luciani,  has  placed  the  existence  of  active  diastole  beyond 
reasonable  doubt.  Enclosing  the  dog's  heart  in  a  cardiometer  or  box, 
such  as  that  employed  by  Roy  and  Adami,  he  found  that  the  heart 
was  still  able  to  propel  the  blood  into  the  aorta  when  the  pericardial 
pressure  (i.  e.,  the  pressure  of  the  fluid  within  the  box  enclosing  the  heart) 
was  25  cm.  H2O  higher  than  within  the  cava,  i.  e.,  within  the  right  ven- 
tricle. Further,  when  the  pressure  acting  upon  the  heart  from  without 
was  so  increased  that  no  blood  entered  the  heart,  and  none,  therefore, 
passed  into  the  aorta,  stimulation  of  the  peripheral  end  of  the  cut  vagus 
led  to  the  appearance  of  an  aortic  wave.  Evidently,  therefore,  vagus 
stimulation  increased  the  active  expansion  of  the  ventricles,  permitting 
blood  to  enter  their  cavities,  which  blood  became  expelled  in  systole. 
The  heart,  therefore,  is  not  merely  a  force  pump,  but  is  also  a  suction 
pump,  and  in  both  ways  brings  about  the  circulation  of  the  blood. 
From  a  pathological  point  of  view  these  observations  are  of  importance 
as  throwing  light  upon  its  action  in  cases  of  circulatory  obstruction. 
The  hypertrophy  that  occurs  in  these  cases,  it  is  suggested,  is  not  only  due 
to  increased  work  in  propulsion,  but  also  to  increased  suction  in  diastole. 
We  thus  gain  an  explanation,  hitherto  wanting,  for  the  not  infrequent 
cases  in  which  uncomplicated  stenosis  of  the  mitral  valve  is  accompanied 
by  hypertrophy  of  the  left  ventricle.  If  systolic  effort  alone  induced 
overgrowth,  then,  less  blood  reaching  it  through  the  narrow  valve,  that 
chamber  should  remain  small,  but  as  above  noted,  that  frequently  is 
not  the  case. 

Here  must  be  called  to  mind  the  similar  indication  of  active  dilatation 
of  the  muscles  of  the  arterial  wall.  The  well  ascertained  existence  of 
vasodilator  nerves,  which  on  stimulation  cause  enlargement  of  the 
arterial  lumen,  in  contrast  to  the  vasoconstrictor  fibers,  can  only  mean 
that  dilatation  of  the  muscle  fibers  is  an  active  process.  Here,  it  is  true, 
we  deal  with  non-striated  muscle  fibers,  but  in  their  relationships  these 

1  Luciani,  Physiologie  der  Menschen,  1 :  1905. 


ARRHYTHMIA  125 

are  strictly  homologous  with  the  cardiac  muscle,  which  we  must  regard 
as  a  highly  differentiated  development  of  the  muscle  layer  of  one  part 
of  the  hemal  tube.  With  Sir  Lauder  Brunton1  we  may  assume  the 
existence  of  two  orders  of  contraction  on  the  part  of  these  muscles,  (a) 
longitudinal  contraction,  leading  to  a  shortening  of  the  long  diameter, 
and  (6)  transverse  leading  to  a  lengthening  of  the  same. 

Arrhythmia. — For  us  the  main  significance  of  these  recent  develop- 
ments of  the  myogenic  theory  is  the  light  they  throw  upon  various 
orders  of  cardiac  irregularity.  It  cannot  be  said  that  we  as  yet  have 
fully  solved  by  any  means  all  the  problems  which  these  cardiac  irregular- 
ities present.  There  are  still  those  inclined  to  uphold  the  pure  neuro- 
genic  or  the  pure  myogenic  theory  of  origin  of  many  of  these  states. 
But  if  what  has  been  laid  down  in  the  previous  volume  be  kept  steadily 
in  mind,  namely,  that  analogous  series  of  reactive  phenomena  may 
present  themselves  set  up  by  the  direct  action  of  noxse  upon  the  tissues 
and  by  nervous  influences  respectively,  as  demonstrated  by  the  existence 
of  neurogenic  and  "referred"  inflammatory  disturbances,  and  of  neuro- 
genic  hyperpyrexia,  then,  applying  the  same  consideration  to  the  heart 
action,  we  can  harmonize  what  appear  to  be  absolutely  contradictory 
findings.  Of  these  arrhythmias  six  types  are  to  be  recognized : 

(1)  Respiratory. — It  is  a  very  old  observation  that  the  rate  of  the 
pulse  is  accelerated  with  inspiration,  slowed  with  expiration.     At  times, 
in  neurasthenia,  those  recovering  from  acute  infections,  etc.,  this  differ- 
ence in  rate  is  greatly  exaggerated,  the  pulse  during  inspiration  becoming 
so  rapid  and  the  heart  beats  so  small  as  to  be  scarcely  recognizable. 
Mackenzie2  ascribes  this  to  pressure  influences  acting  upon  the  sinus,  or 
as  we  may  express  it,  to  excitability  of  Keith's  node. 

(2)  "Extrasystolic." — Experimentally  upon  the  hearts  of  animals  of  the 
laboratory,  working  regularly,  it  is  possible  to  interpolate  extra  contrac- 
tions or  systoles,  and  where  this  is  done  by  ventricular  stimulation  it  is  found 
that  (1)  this  interpolated  beat  is  smaller  than  normal;  (2)  that  it  is  followed 
by  a  longer  diastole,  and  this  (3)  by  a  systole  more  powerful  than  usual 
in  such  a  way  that  the  diastole  before  the  extra  beat  and  the  compara- 
tively lengthened  post-systolic  period  together  equal  in  length  two  dias- 
toles, while  it  may  be  suggested  that  the  small  extra  systole  and  the 
following  delayed  systole  together  in  effectiveness  correspond  to  two  ordi- 
nary beats.     In  the  conditions  of  pulsus  bigeminus,  pulsus  trigeminus, 
etc.,  we  have  indications  in  man  of  the  existence  of  this  extra  systole, 
and  this  is  the  most  common  type  of  cardiac  irregularity.     What  is  the 
meaning  or  the  cause  of  this  extra  systole  is  still  an  open  question.    For 
ourselves,  studying  a  large  series  of  tracings,  we  cannot  but  be  impressed 
with  their  resemblance  to  interference  curves,  namely,  to  the  interfer- 
ence between  two  series  of  waves  of  different  length  and  rate. 

The  character  of  the  pulse  tracings  in  this  series  of  cases  is,  we  would 

1  Therapeutics  of  the  Circulation,  London,  J.  Murray,  1908:  43. 

2  Diseases  of  the  Heart,   London,  1908:  the  fullest  study  upon  this  and   allied 
phenomena  that  has  yet  been  published. 


126  THE  MOTOR  APPARATUS 

emphasize,  that  of  "interference  curves;"  it  corresponds,  that  is,  with  the 
orders  of  tracings  that  can  be  obtained  by  the  interference  of  two  sets  of 
periodic  waves  of  differing  wave  lengths,  with  these  distinctions  (1)  that 
owing  to  the  law  of  maximal  contraction,  where  the  waves  augment 
each  other,  i.  e.,  when  the  upstrokes  of  the  two  coincide,  there  is  no 
summation;  and  (2)  that  the  existence  of  a  refractory  period  following 
the  wave  of  the  one  order  prevents  the  appearance  of  waves  of  the  other 
order  timed  to  show  themselves  during  that  refractory  period.  We  have 
in  the  previous  paragraphs  suggested  that  these  two  orders  of  waves  are 
the  nervous  stimuli  and  the  automatic  contractions  respectively.  There 
are,  however,  difficulties  in  accepting  this  view.  If  the  assumption  of 
the  physiologists  be  correct,  that  the  law  of  maximal  contraction  demands 
that  each  contraction  of  the  heart  muscle  necessitates  the  explosion  or 
using  up  of  all  the  contractile  material  accumulated  during  the  previous 
refractory  period,  it  must  follow  that  where  stimuli  of  two  rates  of  peri- 
odic incidence  act  on  the  ventricular  muscle,  the  waves  of  greater  fre- 
quency alone  will  be  effective,  the  other  order  of  waves  will  make  no 
impression  upon  the  curves.  The  very  existence,  however,  of  these 
irregular  curves  of  heart  beat,  in  which  it  by  no  means  necessarily 
follows  that  the  longer  the  interval  between  the  beats  the  greater  the 
size  of  the  subsequent  wave,  shows  that  our  ordinary  conception  of  the 
significance  of  the  refractory  phase  is  not  wholly  satisfactory.  It  is  at 
least  worthy  of  suggestion  that  the  refractory  phase  corresponds  with 
the  stage  of  active  expansion  of  the  heart  fibers,  already  noted  (p.  123). 
If  we  assume  that  active  contraction  and  active  expansion  are  under 
the  control  of  different  nerves  (e.  g.,  the  accelerators  and  the  vagi, 
respectively),  then  were  the  stimuli  to  pass  down  these  nerves  to  the 
ventricular  muscle  at  different  rates,  we  would,  it  may  be  suggested, 
obtain  interference  curves  of  the  nature  of  those  observed  in  this  order 
of  cases. 

(3)  Due  to  Disturbance  in  Conduction. — Great  interest  has  of  late  been 
manifested  in  cases  of  what  is  termed  the  Stokes-Adams  syndrome,  or 
"heart-block."     In  this  there  is  a  striking  bradycardia,  or  slowing  of  the 
pulse.    By  fluoroscopic  examination,  or  by  simultaneous  register  of  arterial 
and  venous  (jugular)  pulses,  it  is  found  that  the  ventricular  beat  occurs 
only  with  every  other  auricular  contraction,  or,  it  may  be,  with  every 
third  or  fourth.     Along  with  this  there  may  be  Cheyne-Stokes  breathing, 
attacks  of  syncope  or  of  epilepsy,  or  even  apoplectiform  seizures.     Since 
the  publication  of  Tawara's  paper  autopsies  upon  quite  a  series  of  suf- 
ferers from  this  syndrome  have  recorded  the  existence  of  disease  affecting 
the  region  of  the  atrioventricular  node,  degeneration  of  the  myocardium 
and   necrosis   involving  the  region,    fibrosis   and  gummas,    leading  to 
partial  or  complete  destruction  of  the  same:  conditions,  that  is,  which 
evidently  have  obstructed  or  completely  destroyed  the  band  of  communi- 
cation between  auricle  and  ventricle.     They  amply  explain  the  lack 
of  coordination  between  the  two  sets  of  chambers. 

(4)  Of  Central  Origin. — But  there  are  other  cases  on  record  in  which 
anemia  of  the  medulla  or  vagus  irritation  can  alone  be  invoked.    Brady- 


ARRHYTHMIA  127 

cardia,  or  abnormal  slowing  of  the  heart  beat,  and  tachycardia,  or  abnor- 
mal rapidity,  may  both  experimentally  be  produced  by  nervous  influences 
alone.  It  is  possible  that  efferent  impulses  alone  may  so  depress  the 
excitability  of  ventricular  muscle  that,  instead  of  there  being  (as  in 
some  cases)  simple  bradycardia,  auricle  and  ventricle  becoming  equally 
slowed,  arrhythmia  may  be  produced  of  such  a  nature  that  only  every 
other,  or  it  may  be  only  every  third  or  fourth,  auricular  contraction  may 
be  followed  by  a  ventricular  systole.  With  Erlanger1  we  may  explain 
this  as  due  not  to  absence  of  stimulus  conveyed  from  auricle  to  ventricle, 
along  the  "  bundle  of  His,"  but  as  due  to  the  fact  that,  following  a  ven- 
tricular beat,  the  contractile  material  is  so  slowly  accumulated  (or  the 
muscular  excitability  so  depressed)  that  the  next  stimulus  passing  from 
the  auricles  fails  to  arouse  a  contraction.  Only  with  further  accumula- 
tion of  the  contractile  material  will  a  second  or  later  stimulus,  of  like 
strength,  become  effective. 

Here,  the  opposite  condition  must  also  be  noted.  Of  this  we  only 
know  one  instance,  observed  in  our  laboratory  at  the  Royal  Victoria 
Hospital  by  Dr.  Klotz  in  the  heart  of  a  late  patient  of  Dr.  C.  F.  Martin 
—the  condition  of  complete  interruption  of  the  bundle  of  His  or  con- 
ducting system,  with,  nevertheless,  no  sign  of  cardiac  irregularity  or  heart- 
block.  In  this  case  there  was  complete  replacement  of  all  the  tissues 
in  the  region  of  the  auriculo-ventricular  node  by  a  very  extensive  sarco- 
matous  infiltration.  At  most,  some  scattered  and  greatly  degenerated 
fibers  were  to  be  detected,  which  might  possibly  represent  isolated  cells 
of  the  system.  The  patient  had  for  long  been  bedridden,  and  as  the 
Stokes-Adams  syndrome  is  peculiarly  apt  to  manifest  itself  after  some 
act  of  exertion,  it  may  well  be  that  in  this  case  the  ventricles  were  beating 
automatically,  i.  e.,  had  assumed  their  own  independent  rhythm  of  con- 
traction— just  as  happens  experimentally  and  eventually  after  compres- 
sion or  destruction  of  the  bundle  of  His,  and,  doing  this,  were  able  to 
fulfil  all  the  needs  of  the  organism. 

While  everything  indicates  that  the  heart  automatically  may  take  up 
an  independent  rhythm  which  results  in  irregular  action,  i.  e.,  that 
direct  stimuli  acting  on  the  ventricular  muscle  may  stimulate  the 
production  of  independent  contractions,  nevertheless,  the  indications 
are  that  very  often  vagus  action  is  responsible  for  cardiac  arrhythmia. 
The  many  years  of  study  of  the  mammalian  heart  by  Roy  and  one  of  us2 
led  to  the  conclusion  that  the  main  function  of  the  vagus  is  to  protect  the 
heart,  even,  if  need  be,  at  the  expense  of  the  body  in  general.  Moderate 
vagus  stimulation  slows  the  heart  beat,  the  individual  beats  becoming 
stronger,  but  undoubtedly  the  work  accomplished  by  the  heart  is  lessened 
— the  output  of  the  ventricles  in  a  given  time  is  reduced.  Stronger  vagus 
stimulation  actually  stops  the  auricles,  and  experimentally  we  can  pro- 

1  Journ.  of  Exp.  Medicine,   7:  1905,  and  Bull,   of  the  Johns  Hopkins  Hosp.,  16: 
1905:234. 

2  Roy  and  Adami,  Contributions  to  the  Physiology  and  Pathology  of  the  Mam- 
malian Heart,  Phil.  Trans.  Roy.  Soc.,  London,  183  B:  1892:  199  to  298. 


128  .'  THE  MOTOR  APPARATUS 

duce  the  various  grades  of  (1)  temporary  complete  stoppage  of  the 
ventricles,  and  so  of  the  pulse;  (2)  assumption  by  the  ventricles  of  an 
independent  rhythm,  the  auricles  still  being  arrested;  and  (3)  various 
grades  of  arrhythmia.  It  is  not  that  the  vagus  directly  stimulates  the 
ventricle  or  auricle  to  contract;  on  the  contrary,  we  have  evidence  that 
this  wave  lowers  the  excitability  of  both  the  auricular  and  the  ventricular 
muscle.  Rather  along  the  lines  suggested  above,  we  may  regard  the 
vagus  as  the  anabolic  nerve,  or,  at  least,  as  the  nerve  favoring  the 
active  expansion  of  the  heart  muscle.  Thus,  if  stimuli  conveyed  down 
this  nerve  be  sufficiently  powerful  they  may  neutralize  and  overcome 
those  passing  down  the  atrioventricular  bundle  and  favoring  contrac- 
tion. And  now,  when  through  the  action  of  vagus  stimuli  there  has 
been  increased  accumulation  of  the  contractile  or  expansive  material 
either  a  minimal  nervous  stimulus  to  contraction  may  result  in  a 
maximal  contraction,  or  other  stimuli,  not  of  nervous  origin,  may  be 
directly  effective  in  causing  contraction.  In  other  words,  according  to 
the  conditions  acting  upon  the  heart,  so  may  there  be  either  nervous  or 
automatic  contraction  of  the  ventricles  manifesting  itself.  We  may  thus 
lay  down  that  when  the  ventricles  are  overworked,  as  is  the  case  in 
beginning  failure  of  compensation,  then  for  self-protection,  afferent 
impulses  from  the  heart  call  the  vagus  heart  centres  into  activity.  It  is 
under  these  conditions  that  arrhythmia  shows  itself.  Or  otherwise, 
arrhythmia  is  frequently  an  indication  that  the  ventricles  are  working 
at  the  limit  of  their  reserve  force,  and  need  vagus  assistance  in  order  to 
prevent  complete  cardiac  exhaustion.  Further,  other  reflex  arcs  may 
stimulate  the  vagus  centres.  These  centres  may  be  acted  upon  by 
emotional  and  psychic  influences,  or  influences  reaching  them  from  the 
gastric  or  splanchnic  areas  and  from  sensory  surfaces.  The  analysis 
and  determination  of  the  cause  in  any  particular  case  of  cardiac 
arrhythmia  demands,  therefore,  a  wide  survey  of  conditions  through- 
out the  organism. 

(5)  Pulsus  Alternans. — Of  this  and  the  next  form  of  irregularity  of 
heart  beat  less  has  been  determined.     In  the  pulsus  alternans  a  strong 
ventricular  beat  alternates  with  a  weak,  with  equal  intervals  between 
the  beats.     It  occurs  with  indications  of  great  cardiac  weakness.     We 
have  already  indicated  that  in  "extra  systole"  the  lengthened  diastole  is 
associated  with  ventricular  disturbance.     The  want  of  such  lengthening 
in  these  cases  suggests  that  the  disturbance  originates  in  the  auricle  or 
at  Keith's  node. 

(6)  Aperiodic  Irregularity  (pulsus  irregularis  perpetuus). — Markedly 
irregular  irregularity  is  encountered  in  cases  of  advanced  mitral  and 
tricuspid  stenosis  and  incompetence.      Mackenzie  assumes  that  there 
is  some  break  in  continuity  of  the  conducting  paths  between  the  sino- 
auricular  and  the  atrioventricular  mode.     It  cannot,  however,  be  said 
that  we  have  adequate  anatomical  or  other  data  upon  which  to  base  a 
conclusion. 

The  Filling  of  the  Ventricles,  and  its  Effect  on  the  Muscle 
Fibers. — Distension,   Hypertrophy,   and    Dilatation. — The   work  accom- 


THE  FILLING  OF  THE  VENTRICLES  129 

plished  by  the  heart  in  a  given  time  is  determined  by  the  amount  of  blood 
propelled  in  that  time,  and  the  external  (arterial)  pressure  against  which 
it  is  discharged.  And  the  amount  propelled — or  expelled — is  the  product 
of  the  amount  discharged  per  heart  beat  into  the  number  of  heart 
beats.  The  pressure  remaining  the  same,  the  like  amount  of  work  is 
accomplished  by  a  rapidly  beating  heart  discharging  a  small  quantity 
of  blood  at  each  systole,  as  by  a  heart  beating  at  half  the  rate,  discharging 
each  time  twice  the  quantity.  It  will  be  realized  that  in  those  two  cases 
the  conditions  within  the  ventricles  may  be  very  different,  to  the  extent 
that  the  diastolic  expansion  in  the  latter  case  will,  in  the  normally  acting 
heart,  be  roughly  twice  that  seen  in  the  former,  or  otherwise,  the  muscle 
fibers  in  the  latter  case  have  to  contract  against  twice  the  load,  and,  in 
diastole,  expand  to  a  greater  extent  under  this  increased  load. 

The  physics  connected  with  this  aspect  of  cardiac  work  are  not  a 
little  interesting,  inasmuch  as  they  give  a  clearer  understanding  of  what 
happens  in  the  not  infrequent  cases  of  obstruction  to  the  outflow  of  blood 
from  the  heart.  Here  we  may,  we  think,  employ  safely  our  knowledge 
of  the  laws  of  contraction  of  voluntary  muscle.  The  ventricle,  that  is, 
under  its  load  of  blood  to  be  expelled,  may  be  compared  with  the  familiar 
gastrocnemius  muscle  of  the  frog.  If  a  series  of  weights  be  attached  to 
such  a  muscle  hanging  at  rest,  it  is  to  be  noted  that  some  weights  are 
so  small  that  the  length  of  the  muscle  is  not  altered;  the  natural  tonus  of 
the  muscle  is  greater  than  the  expanding  force  exerted  by  those  weights. 
With  progressively  increasing  weights,  however,  the  resting  muscle 
becomes  more  and  more  stretched,  at  first  with  relatively  rapid  increases 
in  length,  later  as  the  limit  of  elasticity  is  reached,  with  lessened  increment 
in  length.  The  application  of  these  facts  to  the  ventricle  is  that,  with 
increase  in  load,  even  within  normal  limits,  the  ventricles  show  distension, 
and  that  increase  in  work  per  individual  systole  is  accompanied  by  a 
natural  distension  of  the  ventricle.  In  other  words,  within  normal 
limits  increased  work  of  the  heart  is  followed  by  increased  size  of  the 
organ  in  diastole.  There  is  a  natural  distension  as  distinguishable  from 
a  pathological  dilatation. 

On  the  other  hand,  still  considering  the  gastrocnemius,  if  we  record 
the  excursion  of  this  muscle  when  stimulated  with  the  like  strength  of 
electric  current,  but  when  bearing  a  succession  of  increasing  loads, 
we  find  that  the  work  accomplished  by  the  muscle,  i.  e.,  the  weight  raised 
multiplied  by  the  distance  to  which  it  is  raised,  is  far  from  being  at  its 
maximum  with  the  smallest  weight.  The  work  done  when  progressively 
increasing  weights  are  attached  undergoes  increase  up  to  a  certain  point, 
or,  otherwise,  there  is  an  optimum  load  which  with  a  given  muscle  and 
given  strength  of  stimulus  leads  to  the  accomplishment  of  the  greatest 
amount  of  work.  Here  the  application  is  that  the  heart  accomplishes 
most  work  not  when  the  arterial  pressure  is  lowest,  but  under  a  certain 
mean  arterial  pressure,  which  of  necessity  varies  with  different  in- 
dividuals and  different  states  of  nutrition  of  the  ventricular  muscle. 
So  also  it  would  seem  that  a  certain  amount  of  diastolic  stretch  of  the 
muscle  fibers,  or  distension  of  the  ventricles,  results  in  more  effective 
9 


130  THE  MOTOR  APPARATUS 

contractions,  i.  e.,  in  the  expulsion  of  the  greater  amount  of  blood  as  the 
result  of  individual  contraction. 

Or  we  can  approach  this  subject  from  another  point  of  view.  If,  as 
shown  by  Roy  and  Adami,1  two  points  be  taken  upon  the  surface  of  the 
left  ventricle  of  a  dog,  and  by  a  proper  instrument  the  distance  between 
these  points  under  various  conditions  be  recorded,  it  is  found  that  upon 
increasing  the  intraventricular  pressure,  as  by  narrowing  the  aortic 
arch  by  means  of  a  ligature,  the  heart  becomes  more  rilled  in  diastole, 
and  the  two  points  become  farther  apart,  while  in  systole,  the  points  do 
not  approximate  to  nearly  the  same  extent  as  when  there  is  less  resist- 
ance. Similar  results  are  obtainable  if,  instead  of  primarily  increasing 
the  pressure  in  the  arterial  system,  the  heart  is  given  more  work  to  do 
by  increasing  the  amount  of  blood  supplied  to  it.  This  can  be  accom- 
plished, temporarily,  by  pressure  upon  the  abdomen,  or  over  longer 
periods,  by  injecting  into  the  venous  circulation  some  few  hundred 
cubic  centimeters  of  defibrinated  blood.  Again,  there  is  the  same 
filling  in  diastole,  and  relatively  slighter  approximation  of  the  points 
in  systole.  With  increased  work  of  the  heart  accompanying  the  dis- 
tension in  diastole,  there  is  a  dilatation  in  systole  also;  the  fibers  do  not 
shorten  to  the  same  extent.  There  is  of  necessity  residual  blood  in  the 
ventricular  chambers.  The  significance  of  this  is  grasped  if  we  consider 
the  ventricular  chambers  as  a  sphere.2  There  is  this  to  be  noted  con- 
cerning the  relationship  between  the  circumference  of  a  sphere  and  its 
contents,  namely,  that  as  a  sphere  expands,  its  cubic  contents  increase 
out  of  all  proportion  to  its  increase  in  circumference,  or,  more  accurately, 
the  ratio  of  increase  is  not  an  arithmetical  ratio,  but  is  such  that  if  the 
circumference  be  taken  as  abscissae,  the  corresponding  volumes  as 
ordinates,  the  curve  of  successive  values  is  what  is  known  as  a  cubical 
parabola  (Fig.  16).  From  this  it  follows  that  a  degree  of  shortening 
of  the  fibers  of  the  heart  wall  sufficient,  let  us  say,  to  reduce  the  circum- 
ference of  the  ventricle  an  inch,  will  cause  a  greater  diminution  in 
volume  (or  greater  output)  the  more  distended  or  dilated  the  ventricle 
is  at  the  beginning  of  its  contraction.  For  example,  a  diminution  of  the 
circumference  by  an  inch  of  a  sphere  of  ten  inches'  circumference  causes 
a  diminution  of  volume,  or  an  output,  equal  to  4.5  cubic  inches,  when  a 
diminution  by  one  inch  in  the  circumference  of  a  sphere  five  inches 
round  causes  an  output  of  only  1.027  cubic  inches,  although  in  the  first 
case  the  circumference  was  reduced  only  one-tenth,  in  the  second  one- 
fifth.  That  is  to  say,  with  moderate  distension  or  dilatation  of  the  heart, 
the  fibers  will  need  to  contract  a  very  small  amount  in  order  to  expel  a 
given  amount  of  blood  compared  with  the  amount  of  their  contraction 
in  a  normal  undilated  heart.  It  is  thus  very  possible  that,  in  a  hard- 
working heart,  a  certain  grade  of  distension  is  economical,  and  that  the 
presence  of  residual  blood  by  diminishing  the  extent  to  which  each  fiber 

1  Practitioner,  52-  1894:81. 

2  This  is  the  nearest  geometrical  figure  that  we  can  employ  here  for  purposes  of 
illustration. 


THE  FILLING  OF  THE  VENTRICLES 


131 


is  called  upon  to  contract,  may  be  a  saving  to  the  heart  and  to  the 
organism  as  a  whole. 

From  these  considerations  and  observations  it  follows  that  hyper- 
trophy is  never  primary;  distension  (or,  as  it  is  usually  termed,  dilatation) 
always  precedes  hypertrophy.  The  only  distinction — and  perhaps  it 
is  a  necessary  distinction — that  we  can  recognize  between  these  two 


FIG.  17 


I' 

1 

s 


Circumferences   in   inches. 

Curve  representing  the  relationship  between  the  circumference  of  a  sphere  and  its  volume,  with 
successive  unit  increments  of  circumference.  Ordinate — volume  in  cubic  inches.  Abscissae — 
circumference  in  inches. 

terms — distension  and  dilatation — is  that  the  former  is  a  temporary 
state  which  disappears  as  soon  as  the  heart  is  relieved,  the  latter  is  a 
more  permanent  condition,  brought  about  by  disease,  and  still  persist- 
ing when  the  cause  ceases  to  be  effective.  Possibly,  we  may  add  that 
dilatation  proper  should  only  be  regarded  as  present  when  there  is  actual 
incompetence  of  the  heart  muscle  and  incapacity  to  contract  to  an 


132  THE  HEART  VALVES 

extent  commensurate  with  the  load  borne  by  the  muscle.  If  we  deal 
simply  with  distension,  and  the  heart  muscle  be  well  nourished,  we  have 
the  inevitable  sequel  that  continuance  of  increased  work  within  reasonable 
limits  leads  to  hypertrophy — either  hypertrophy  proper,  or  hyperplasia, 
or  often  a  combination  of  the  two  (vol.  i,  p.  540).  Such  hypertrophy 
or  hyperplasia  will  either  relatively  or  absolutely  lessen  the  load  of  each 
individual  muscle  fiber.  As  a  result,  with  lessened  load,  each  fiber  will 
contract  more  completely,  and  the  dilatation  will  tend  to  disappear. 
In  such  cases  we  deal  with  simple  hypertrophy. 

There  can  be  no  doubt  that  where  there  is  ample  reserve  force  and  good 
compensation,  this  simple  hypertrophy  exists  and  may  persist  for  years, 
though  it  is  the  exception  rather  than  the  rule  upon  the  postmortem 
table.  Where  there  is  persistent  cause  for  increased  heart  work  (as  in 
aortic  stenosis  or  incompetence),  then  more  often  we  find  that  the 
reserve  force  of  the  ventricular  muscle  becomes  diminished  or  exhausted, 
and  eccentric  hypertrophy  supervenes;  that  is  to  say,  we  have  combined 
a  pathological  dilatation  with  hypertrophy.  Not  a  few  authorities  still 
describe  a  concentric  hypertrophy.  In  our  opinion  this  is  non-existent. 
It  is  true  that  now  and  again  we  encounter  an  apparent,  a  false  concentric 
hypertrophy  upon  the  postmortem  table,  in  which  what  is  striking  is 
the  tensely  knit  ventricular  muscle,  greatly  increased  in  amount,  with 
practically  no  lumen  to  the  ventricle.  All  the  cases  we  have  seen  of  this 
condition  have  been  from  cases  examined  within  the  first  six  hours  or 
so  after  death.  They  represent  rigor  mortis,  the  heart  muscle  passing 
into  this  state  in  one  hour  after  death.  Seen  next  day,  such  hearts 
present  well-marked  hypertrophy  with  dilatation.  There  is  no  such 
thing  as  true  concentric  hypertrophy;  it  implies  that  the  ventricle,  in 
contracting,  expends  a  large  part  of  its  energy  in  compressing  the  more 
internal  fibers,  a  most  unnatural  lack  of  economy  in  the  work  of  the 
organ.  The  causes  of  hypertrophy  and  dilatation  will  be  dealt  with 
seriatim  on  pp.  155  and  156. 

3.  THE  HEART  VALVES. 

The  mechanism  of  the  action  of  the  heart  valves  is,  or  should  be,  so 
well  known  that  little  need  be  said  regarding  their  physiology,  although 
in  discussing  their  pathology  certain  less  known  aspects  of  their  normal 
mode  of  function  will  have  to  be  dwelt  upon.  We  have  to  consider  the 
results  of  their  imperfect  closure  and  imperfect  opening :  of  incompetence 
and  stenosis.  Practically  all  the  disturbances  we  have  to  consider, 
whether  congenital  or  of  postnatal  origin,  come  under  these  two  headings. 

Incompetence. — This  may  be  relative,  due  to  no  disease  of  the 
valves  themselves,  but  to  a  giving  way  and  expansion  of  the  ring  of  tissue 
to  which  the  valves  are  attached,  so  that  their  cusps  do  not  meet  and 
close  the  aperture ;  or  actual,  due  to  disease  or  injury  to  the  cusps,  pro- 
ducing the  like  result.  As  a  consequence,  at  the  period  of  the  cardiac 
cycle,  when  the  valves  should  be  closed,  there  is  regurgitation  of  blood, 
and  passage  back  of  the  same  into  chambers  from  which  it  had  previously 


INCOMPETENCE  133 

been  discharged.  The  blood  thus  regurgitated  constitutes  an  additional 
load  for  these  chambers  to  propel  at  their  next  systole  in  addition  to  the 
normal  load  reaching  them  from  the  normal  source  or  sources.  To 
accommodate  this  additional  blood  the  affected  chambers  undergo  dis- 
tension (or  physiological  dilatation);  to  cope  with  the  increased  work 
they  are  called  upon  to  perform,  they  exhibit  hypertrophy. 

The  study  of  the  heart  post  mortem  suggests  that  incompetence  is 
a  more  frequent  condition  than  is  recognized  clinically;  or  otherwise, 
that  frequently  regurgitation  exists  without  the  existence  of  murmurs 
calling  attention  to  its  presence.  Notably  is  this  the  case  in  connection 
with  the  tricuspid;  the  shape  and  the  relative  weakness  of  the  right  ven- 
tricle may  almost  be  said  to  favor  incompetence  and  regurgitation  with 
even  a  moderate  grade  of  distension  of  the  ventricle.  Such  distension 
is  the  outcome  of  either  (1)  incompetence  or  stenosis  of  the  pulmonary 
valve,  (2)  obstructive  disease  of  the  lungs  and  pulmonary  circulation, 
or  (3)  obstruction  to  outflow  of  the  blood  through  the  left  heart.  The 
result  is  distension  of  the  right  auricle,  and,  as  there  are  no  adequate 
valves  at  the  entrance  of  the  venae  cavas,  with  contraction  of  the  ventricle 
a  reverse  wave  of  blood  is  propelled  into  the  larger  systemic  veins,  and 
these  become  distended  at  a  period  when  normally  the  blood  should 
be  pouring  from  them  into  the  right  auricle.  The  result  is  a  marked 
obstruction  to  the  venous  circulation.  The  greatly  distended  right 
auricle  undergoes  some  hypertrophy,  but  very  soon  the  compensation 
is  incomplete,  with  the  result  that  the  blood  accumulates  or  is  dammed 
back  on  the  venous  side  of  the  heart,  passive  congestion  showing  itself 
in  the  liver  and  other  organs,  and  with  the  other  consequences  already 
discussed  on  p.  106  et  seq. 

A  similar  relative  incompetence  is  not  infrequent  at  the  mitral  valve, 
whether  as  the  result  of  high  blood  pressure  or  obstructive  aortic  valve 
disease,  or  again  through  acute  or  terminal  dilatation  of  the  left  ventricle ; 
acute  dilatation  being  brought  on  by  the  action  of  toxemia,  alcohol  and 
other  drugs ;  terminal,  being  due  to  failure  of  compensation  after  long- 
continued  hypertrophy  or  progressive  malnutrition.  Nor  is  this  relative 
incompetence  unknown  in  connection  with  the  pulmonary  and  aortic 
orifices;  the  fact  that  in  the  last  three  years  we  have  encountered  at 
autopsy  no  less  than  three  cases  of  the  last  condition,  makes  us  think 
that  it  is  more  common  than  is  generally  suspected.  In  one  of  these 
three  the  regurgitation  had  evidently  been  of  long  continuance,  for  the 
corpora  Arantii  and  free  edge  of  those  portions  of  the  cusps  which  did 
not  meet  at  the  centre  had  been  thickened  and  rounded.  Such  relative 
incompetence  of  the  pulmonary  and  aortic  valves  would  seem  to  be 
brought  about  in  part  by  dilatation  of  the  origins  of  the  pulmonary 
artery  and  of  the  aorta;  in  part  by  a  giving  way  of  the  muscular  ring 
immediately  beneath  the  valves. 

Actual  or  organic  incompetence  of  the  heart  valves  will  be  discussed  in 
the  chapters  devoted  to  the  morbid  anatomy  of  the  heart.  Here,  it  is  only 
necessary  to  call  attention  to  the  fact  that  incompetence  most  frequently 
co-exists  when  there"  is  stenosis.  The  narrowing  of  the  cardiac  orifices  is 


134  THE  HEART  VALVES 

rarely  of  such  a  nature  as  to  permit  complete  apposition  of  the  diseased 
components  of  the  valve. 

The  results  of  incompetence,  whether  relative  or  actual,  are  of  the 
same  order,  whichever  valve  is  involved,  namely:  regurgitation,  over- 
loading of  the  chambers  into  which  the  blood  regurgitatates,  distension 
of  the  same,  followed  by  compensatory  hypertrophy.  The  overfilling 
of  the  chamber  behind  the  incompetent  valve  sooner  or  later  brings 
about  further  damming  back  of  the  blood  toward  the  venous  side  of  the 
heart,  until  eventually,  in  aortic  incompetence,  for  example,  we  obtain 
mitral  incompetence,  pulmonary  congestion,  tricuspid  incompetence,  and 
general  passive,  venous  congestion  of  the  organs. 


CHAPTER    VII. 

THE  HEART:  PATHOLOGICAL  ANATOMY  AND  HISTOLOGY. 

FOR  purposes  of  description  it  is  convenient  to  regard  the  heart  as 
consisting  of  three  portions :  the  pericardium,  the  myocardium,  and  the 
endocardium.  It  should  be  borne  in  mind,  however,  that  no  serious 
affection  of  any  one  of  these  structures  can  exist  without  involving  the 
others  to  a  greater  or  less  extent. 

Being  a  hollow  viscus  that  contains  a  constantly  moving  fluid  tissue, 
the  bulk  of  which  is  constantly  altering,  and,  moreover,  being  subject 
to  various  peripheral  impressions,  the  heart  is  in  a  state  of  physiological 
unrest  and  is  consequently  proportionately  liable  to  be  affected  by  disease 
processes. 

The  average  weight  of  the  heart  in  the  adult  male  is  300  grams;  in 
the  female,  250  grams. 

THE  PERICARDIUM. 

The  pericardium  is  a  serous  sac  composed  of  a  connective-tissue 
membrane  lined  with  endothelium.  Unlike  the  other  large  lymph 
spaces  of  the  body,  it  usually  contains  a  relatively  large  amount  of  fluid, 
viz.,  from  30  to  50  to  100  c.cm.,  even  in  the  absence  of  any  pathological 
condition.  No  doubt,  the  presence  of  the  larger  quantities  is  to  be  re- 
garded as  an  agonal  manifestation.  From  the  fact  that  the  pericardial 
fluid  is  rich  in  albumin,  we  must  conclude  that  the  vessels  of  the  peri- 
cardium have  a  physiologically  greater  permeability  to  their  fluid  con- 
tents than  have  those  of  the  other  serous  cavities.  This  will  explain 
the  greater  susceptibility  of  the  pericardium  to  exudative  processes  and 
the  formation  of  considerable  amounts  of  fibrin. 

The  close  relationship  of  the  pericardium  to  the  heart,  lungs,  and 
pleural  cavity  renders  it  also  especially  liable  to  secondary  invasions, 
and  the  free  movement  of  its  two  layers,  one  upon  the  other,  explains 
the  rapid  propagation  of  the  various  inflammatory  processes  to  which 
it  is  subject. 

DEVELOPMENTAL    ANOMALIES. 

In  acardiac  monsters  the  pericardium  is  more  or  less  imperfectly 
developed. 

Complete  or  partial  defects  occur  in  rare  cases,  generally  associated  with 
other  malformations.  The  sac  may  be  quite  absent  or  represented  by 


136  THE  PERICARDIUM 

a  few  fringes  at  the  base  of  the  heart.  More  commonly  there  is  a  partial 
loss  of  substance  over  the  left  ventricle,  through  which  the  heart  may 
protrude  into  the  pleural  cavity. 

Hernise  of  the  serosa  through  the  outer  fibrous  layer  are  very  rare. 


CIRCULATORY  DISTURBANCES. 

Anemia. — This  may  affect  the  pericardium  in  common  with  the  rest 
of  the  body. 

Hyper emia. — Active  hyperemia  is  met  with  in  cases  of  death  from 
pressure  on  the  base  of  the  brain,  and  in  commencing  inflammation. 
Passive  hyperemia  occurs  from  the  same  causes  as  it  does  elsewhere, 
and  in  death  from  suffocation.  It  may  lead  to  rupture  of  the  vessels 
and  the  formation  in  their  neighborhood  of  small  ecchymoses  or  sub- 
serous  hemorrhages,  which  are  most  commonly  present  about  the  base 
of  the  heart.  Petechial  spots  are  so  common  at  autopsy  that  it  is  prob- 
able that  they  are  frequently  produced  during  the  death  agony. 

Similar  extravasations  are  the  effect  of  poisons  (e.  g.,  phosphorus), 
septicemia,  morbus  Werlhofii,  pernicious  anemia,  leukemia,  and  the 
various  infections. 

Hematopericardium. — Hematopericardium  is  the  condition  in  which 
blood  is  found  in  the  pericardium.  This  is  due  to  wounds  of  the 
heart,  rupture  of  the  heart  wall,  of  an  aortic  aneurism,  or  of  the  aorta, 
pulmonary  artery,  or  coronary  vessels. 

Hydropericardium. — Hydropericardium,  or  hydrops  pericardii,  may 
be  part  of  a  general  anasarca.  The  amount  of  fluid  sometimes  reaches 
as  high  as  one  liter.  The  fluid  is  pale  amber  in  color,  clear,  without 
flocculi,  and  poor  in  albumin.  The  condition  is  important,  as  it  leads  to 
stretching  ,of  the  pericardium,  pressure  upon  and  even  atrophy  of  the 
heart,  and  pressure  upon  neighboring  structures. 

PNEUMATOSIS. 

Pneumopericardium. — Air  in  the  pericardium  may  be  due  to  fistulous 
communication  between  the  hollow  viscera,  such  as  the  oesophagus  and 
stomach,  and  the  pericardial  sac;  to  subdiaphragmatic  abscess,  or  to 
fracture  of  the  ribs  and  traumatic  perforation;  or,  again,  to  the  presence 
of  certain  bacteria  like  the  Bacillus  Welchii  and  the  B.  coli.  In  the  last 
contingency  the  sac  is  often  empty  and  the  surface  of  the  heart  resembles 
meat  dried  in  the  sun.  A  very  striking  example  is  recorded  by  one  of 
us,1  where,  in  a  case  of  perforative  appendicitis,  pneumopericardium 
developed  during  life  with  a  distinct  musical  sound  over  the  precordium, 
which  could  be  heard  at  a  considerable  distance.  At  the  autopsy 
there  was  a  subdiaphragmatic  pus  collection,  but  the  continuity  of  the 
pericardium  was  intact.  The  B.  Welchii  was  found  in  all  the  organs. 

1  A.  G.  Nicholls,  Brit.  Med.  Jour.,  11:1907:1844. 


PERICARDITIS  137 


INFLAMMATIONS. 

Pericarditis. — The  most  important  disease  of  the  pericardium  is 
inflammation,  which  may  present  a  variety  of  characteristics. 

Primary  idiopathic  pericarditis,  so  called,  probably  does  not  exist,  except 
in  those  cases  due  to  wounds  of  the  pericardium.  The  cases  recorded 
in  children,  while  they  may  be  "  idiopathic''  clinically,  almost  certainly 
are  due  to  some  mediastinal  lesion. 

Secondary  pericarditis  is  a  term  applied  to  those  forms  due  to  extension 
of  disease  from  other  parts.  It  is  less  misleading,  however,  to  divide  the 
cases  into  (1)  hematogenic,  in  which  the  irritants  reach  the  pericardium 
by  way  of  the  blood,  as,  for  instance,  miliary  tuberculosis  and  those 
forms  that  complicate  acute  rheumatism,  smallpox,  influenza,  nephritis, 
diabetes,  septicemia,  and  cerebrospinal  meningitis,  and  (2)  those  arising 
per  extensionem.  In  the  latter  class  the  disease  may  arise  from  a  great 
variety  of  causes,  among  which  may  be  mentioned  pneumonia,  chronic 
pulmonary  tuberculosis,  acute  pleurisy,  empyema,  aortic  aneurisms, 
inflammation  of  mediastinal  and  peribronchial  glands,  acute  and  chronic 
endocarditis,  and  many  abdominal  conditions,  such  as  peritonitis,  appen- 
dicitis, abscess  of  the  liver  or  pancreas,  and  ulcer  of  the  stomach.  In 
91  cases  of  which  we  have  notes,  occurring  in  the  postmortem  practice  of 
the  Royal  Victoria  Hospitals,  58  per  cent,  arose  by  extension,  of  which 
three-quarters  were  due  to  preexisting  disease  of  the  lungs  or  pleura. 

While  it  is  true  that  in  some  cases  cultures  have  failed  to  show  the 
presence  of  microorganisms,  it  is  almost  certain 'that  all  exudative,  as 
distinguished  from  transudative  processes,  are  due  to  their  action.  The 
organisms  which  are  usually  found  are  the  Diplococcus  lanceolatus, 
B.  coli,  Staphylococcus  pyogenes,  streptococcus,  B.  tuberculosis,  Fried- 
lander's  pneumobacillus,  M.  meningitidis  intracellularis,  and  B.  Welchii. 
One  case  has  come  under  our  notice  where  the  B.  pyocyaneus  was  present. 
Mixed  infections  are  not  uncommon,  and  the  B.  tuberculosis  is  at  fault 
in  more  cases  than  are  usually  suspected. 

The  relative  frequency  with  which  pericarditis  complicates  endocarditis, 
particularly  of  the  aortic  valves,  is  readily  explained  when  we  remember 
the  close  anatomical  relationship  of  the  pericardial  reflexion  to  the 
aortic  ring.  That  the  inflammation  can  extend  through  the  vessel  wall 
at  this  point  is  beyond  a  doubt,  and  not  only  acute,  but  chronic  endo- 
carditis may  provide  a  starting  point. 

When  pleurisy  exists,  it  is  very  common  for  the  outside  of  the  peri- 
cardial sac  to  be  affected  (pericarditis  externa).  Often  in  such  cases 
the  pericardial  fluid  is  increased  in  amount,  but  without  flakes  (inflam- 
matory hy drops),  while  the  superficial  vessels  of  the  pericardium  are 
congested  and  the  surface  of  the  heart  may  show  a  rosy  flush.  Though 
the  endothelium  may,  in  general,  be  quite  smooth,  even  at  this  early 
stage  bacteria  may  be  present.  The  condition  is  sometimes  called 
pericarditis  serosa.  This  form  rapidly  passes  over  into  a  serofibrinous 
inflammation,  in  which  the  sac  becomes  distended  with  a  fluid  exudate  of 


138  THE  PERICARDIUM 

a  yellowish  turbid  appearance,  sometimes  stained  with  blood,  in  which 
float  flakes  of  fibrin.  The  amount  of  the  exudate  may  be  small  or  may 
reach  a  liter  or  more.  The  character  of  the  exudate  varies  considerably, 
depending  on  the  nature  of  the  infection. 

The  fibrin  may  take  the  form  of  a  granular  deposit,  rendering  the 
fluid  turbid,  or  it  may  exist  in  large  flakes,  or  again  may  be  gelatinous. 
If  pus-producing  organisms  be  present,  such  as  the  staphylococcus  and, 
in  certain  cases,  the  pneumococcus,  the  exudate  is  purulent  (jpyoperi- 
cardium).  The  amount  of  fluid  may  be  so  small  that  the  fibrinous 
deposit  is  quite  thick  and  dry  (pericarditis  fibrinos a  sicca).  This  condition 
may  alternate  with  the  serous  outpouring,  or  may  be  due  to  absorption 
of  the  fluid  during  the  later  stages  of  the  affection.  The  deposit 

FIG.  18 


Fibrinous  pericarditis.      (From  the  Pathological  Museum  of  McGill  University.) 

does  not  form  a  layer  of  even  thickness  upon  the  epicardium,  but, 
owing  to  the  movements  of  the  heart,  tends  to  collect  in  little  clumps. 
The  condition  is  aptly  described  by  Laennec,  who  compared  it  to  the 
appearance  produced  by  the  rapid  separation  of  two  slices  of  bread  and 
butter.  In  some  cases  the  fibrin  over  the  left  ventricle  is  arranged  as 
a  raised  network,  while  over  the  right  it  takes  the  form  of  transverse 
parallel  bands.  When  the  deposit  is  thick  and  in  the  form  of  long  tags, 
the  condition  is  known  as  the  cor  villosum.  The  serous  fluid  may 
contain  so  much  blood  that  a  characteristic  appearance  is  presented, 
known  as  pericarditis  hemorrhagica.  In  such  cases  there  may  be  relatively 
little  fibrin.  This  form  occurs  in  debilitated  persons  and  alcoholics,  or 
as  a  manifestation  of  a  hemorrhagic  diathesis,  for  example,  in  scurvy 


PERICARDITIS  139 

and  morbus  maculosus.  Tuberculous  and  carcinomatous  disease  of 
the  pericardium  also  at  times  produce  this  appearance.  In  the  case  of 
drunkards,  the  condition  is  analogous  to  pachymeningitis  hemorrhagica 
interna.  In  cases  of  some  standing  one  sees  the  process  of  organization 
beginning.  Small  vessels  are  seen  to  be  springing  from  the  deeper  layers 
of  the  pericardium  and  making  their  way  into  the  exudate,  which  now 
shows  signs  of  disintegration. 

If  the  heart  in  a  case  of  fibrinous  pericarditis  in  the  early  stages  be 
examined  microscopically,  it  will  be  seen  that  the  flattened  endothelial 
cells  lining  the  sac  are  swollen  and  desquamating,  lying  free  in  little 
masses  upon  the  surface.  The  vessels  of  the  pericardium  are  congested 
and  there  is  beginning  extravasation  of  leukocytes.  The  pericardial 

FIG.  19 


Organizing  pericarditis.        Section  shows    newly-formed    capillaries    in  the  pericardial    exudate. 
Zeiss  obj.  DD.,  ocular  No.  I.      (From  the  Pathological  Laboratory  of  McGfll  University.) 

connective  tissue  and  the  superficial  layers  of  the  heart  muscle  are  oedema- 
tous.  In  parts  where  the  process  is  somewhat  more  advanced,  there  is 
upon  the  surface  a  distinct  layer  of  fibrin,  forming  a  meshwork  mixed 
with  leukocytes  and  containing  numerous  bacteria.  This  frequently 
stains  rather  badly,  as  if  undergoing  disorganization.  In  some  instances 
the  fibrin  melts  together  and  forms  hyaline  clumps.  In  severe  cases  the 
underlying  heart  muscle  shows  marked  cloudy  swelling  and  congestion. 
When  the  exudation  into  the  sac  is  marked  it  leads  to  pressure  upon 
the  heart  and  great  vessels,  causing  more  or  less  stasis  of  the  circulation. 
The  lung  often  becomes,  to  some  extent,  atelectatic.  Acute  pericarditis 
may  extend  to  the  pleura  and  peritoneum.  When  it  heals,  but  few 
traces  may  be  left  of  its  occurrence. 


140  THE  PERICARDIUM 

Pericarditis  Chronica. — Acute  pericarditis  may  eventually  become 
chronic,  in  that,  owing  to  repeated  relapses  with  partial  absorption  of 
the  exudate,  the  process  may  extend  over  weeks  and  result  in  thickening 
of  the  pericardium  and  fibrous  adhesion  between  the  two  leaves  of  the 
sac.  Many  cases  which  start  clinically  as  acute  are  really  chronic  in 
their  nature.  Chronic  pericarditis  may  also  start  insidiously.  The 
disease  is  perhaps  most  common  in  young  persons.  In  developing 
cases,  where  pericardial  thickening  is  going  on  and  more  or  less  adhesion 
of  the  surfaces  has  taken  place,  one  can  see  remains  of  the  exudation 
in  the  form  of  disorganized,  granular,  rather  inspissated,  masses  of  fibrin. 
The  adhesions  are  partial  or  complete,  and  in  some  cases  calcification 
of  the  fibrinous  material  takes  place. 

An  important  type  is  the  indurative  mediastinopericarditis,  where  not 
only  are  the  pericardial  layers  united,  but  the  connective  tissue  of  the 
mediastinum  is  thickened,  so  that  the  heart  may  be  firmly  united  to 
the  chest  wall,  the  lungs,  and  the  diaphragm.  The  etiology  of  this  form 
is  somewhat  obscure.  The  most  potent  cause  appears  to  be  acute  peri- 
carditis, either  rheumatic  or  following  some  of  the  infectious  fevers.  A 
peculiar  form  of  chronic  pericarditis  is  that  in  which  the  pericardial  sac 
\  is  obliterated  and  the  two  layers  are  converted  into  a  thick  hyaline  mem- 
brane of  gristly  consistence.  This  form  is  nearly  always  seen  in  associa- 
tion with  similar  disease  of  the  pleura  and  peritoneum,  but  Eichorst1  has 
recorded  a  case  in  which  the  pericardium  alone  was  affected. 

The  most  important  chronic  conditions  are  tuberculosis  and  syphilis. 

Tuberculous  Pericarditis. — Tuberculous  pericarditis  may  be  hema- 
togenous  or  extend  from  the  mediastinal  and  peribronchial  glands,  or 
from  the  lungs  and  pleura.  It  begins  with  the  formation  of  small  grayish 
tubercles  on  the  inner  surface  of  the  sac,  which  are  surrounded  by  a 
hyperemic  zone  and  often  capped  with  granulation  tissue.  As  the 
condition  progresses,  exudation  takes  place,  the  tubercles  enlarge  arid 
coalesce,  forming  ultimately  caseous  nodules.  The  exudate  may  be 
serofibrinous,  fibrinous,  purulent,  or  hemorrhagic.  It  is  usually  moder- 
ate in  amount,  and  much  productive  change  is  going  on.  The  disease 
at  times  assumes  the  guise  of  a  simple  serofibrinous  inflammation,  or  the 
two  walls  of  the  pericardium  may  be  completely  and  firmly  united  by 
a  thick  layer  of  newly  formed  connective  tissue  containing  caseous  foci. 
This  layer  is  often  very  thick  and  may  have  a  semitranslucent,  structure- 
less appearance  resembling  partially  solidified  celloidin. 

Syphilitic  Pericarditis. — Syphilitic  pericarditis  is  rare,  generally  depend- 
ing upon  syphilis  of  the  heart  wall.  It  leads  to  adhesions. 

Actinomycosis. — Actinomycosis  of  the  lungs,  mediastinum,  oesopha- 
gus, and  peritoneal  cavity  occasionally  extends  to  the  pericardium. 

The  results  of  pericarditis,  when  healing  takes  place,  may  be  that  the 
exudate  is  absorbed  and  there  is  complete  return  to  the  normal  condition. 
Or  the  fluid  portion  may  be  absorbed,  leaving  a  granular,  fatty,  and 
caseous-looking  detritus  between  the  layers  of  an  adherent  pericardium. 

1  Eichorst,  Zuckergussherz,  Deutsche  med.  Woch.,  28:1902:293. 


TUMORS  141 

Such  deposits  may  even  calcify.  Milk  spots  (maculae  tendinese),  which 
are  so  common1  on  the  front  of  the  right  ventricle,  are  by  some  thought 
to  be  due  to  preexisting  pericarditis.  Most  often  they  would  seem  to  be 
of  the  nature  of  "corns"  of  the  serosa,  the  result  of  intermittent  press- 
ure. A  more  important  sequel  is  the  formation  of  fibrous  adhesions 
between  the  two  layers  of  the  pericardium.  These  occur  in  about  3.5 
per  cent,  of  all  postmortems.  Fine  fibrous  bands  near  the  aortic  ring 
are  very  common,  and  are  due  to  extension  of  inflammation  from  the 
valves.  Local  adhesions  by  veil-like  processes  may  be  present  (partial 
synechia),  or  the  two  layers  may  everywhere  be  in  such  close  and  firm 
contact  that  it  is  impossible  to  separate  them  (total  synechia).  The 
latter  form  leads  to  dilatation  of  the  heart  chambers.  On  the  other 
hand,  great  thickening  of  the  pericardium  may  lead  to  atrophy  of  the 
heart.  Such  a  condition  is  often  spoken  of  under  the  term  "chronic 
adhesive  pericarditis,"  but  since  it  is  a  result  and  not  a  process,  it  would 
be  more  correct  to  call  it  pericarditis  adhesiva  obsolescens. 

Parasites. — Trichinae,  cysticerci,  and  echinococci  have  been  found  in 
the  cavity. 

Foreign  Bodies. — Foreign  bodies  may  enter  from  without  or  from 
ulcerative  processes.  It  is  peculiarly  frequent  in  cattle  to  have  needles, 
wire,  etc.,  which  have  passed  through  the  walls  of  the  first  stomach, 
find  their  way  into  the  pericardium  and  lead  to  sudden  death  by 
puncture  of  the  heart.  Analogous  cases  occasionally  occur  in  man. 


RETROGRESSIVE  METAMORPHOSES. 

Serous  Atrophy. — Serous  atrophy  of  the  pericardial  fat  occurs  in 
effusions.-  The  fat  is  yellow  and  gelatinous  in  appearance,  and,  micro- 
scopically, shows  swelling  and  hydropic  degeneration  of  the  fat  cells. 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — Primary  Tumors. — Primary  tumors  are  very  rare.  Car- 
cinoma ( ?)  and  endothelioma  are  recorded,  as  are  also  a  few  instances  of 
primary  sarcoma.2 

Secondary  Tumors. — Secondary  growths  may  be  extensions  from  tumors 
of  the  mediastinum,  lung,  oesophagus,  and  stomach,  or  may  be  metas- 
tatic.  Of  the  first,  carcinoma  and  lymphosarcoma  may  be  mentioned, 
and  of  the  second,  carcinoma  and  melanotic  sarcoma. 

Among  benign  tumors  lipomas  (polypi)  and  fibromas  occur. 

1  According  to  our  Montreal  statistics  they  are  to  be  found  in  more  than  14  per 
cent,  of  all  autopsies. 

2  J.  C.  Williams,  New  York  Med,  Jour.,  71 : 1900:  537. 


142  THE  MYOCARDIUM 

THE  MYOCARDIUM. 

CONGENITAL  ANOMALIES. 

Congenital  peculiarities  are  said  to  be  more  frequent  in  the  male 
than  in  the  female  sex.  In  general,  they  are  due  to  a  retardation  or  an 
actual  vitium  in  the  development  of  certain  parts.  More  rarely,  they 
are  to  be  attributed  to  inflammatory  processes  occurring  during  foetal 
life.  Many  of  them  are  of  great  practical  importance,  inasmuch  as 
their  presence  may  prevent  an  independent  existence  on  the  part  of  the 
offspring,  or  if  life  be  possible,  grave  circulatory  disturbances  may  be 
the  consequence,  with  abnormal  susceptibility  to  disorders  of  related 
parts  and  all  that  this  implies. 

The  number  of  the  cardiac  anomalies  of  this  class  is  legion,  and  we 
cannot,  for  lack  of  space,  do  more  here  than  indicate  in  a  sketchy  fashion 
the  more  important  forms. 

In  a  general  way  the  developmental  anomalies  of  the  heart  and  its 
sinuses  may  be  summarized  as  follows: 

1.  Numerical  variations. 

2.  Displacements. 

3.  Anomalies  of  the  heart  as  a  whole. 

4.  Anomalies  and  defects  of  the  various  septa. 

5.  Anomalies  of  the  lumina  of  the  various  ostia. 

6.  Anomalies  of  the  semilunar  cusps  and  auriculoventricular  valves. 

7.  Patency  and  other  anomalies  of  the  ductus  arteriosus. 

8.  Anomalies  of  the  vessels  in  immediate  relationship  to  the  heart. 
Under  the  category  of  numerical  variations  we  note  the  condition  of 

total  absence  of  the  heart,  as,  for  example,  in  the  monstrous  birth  known 
as  acardiacus.  This  is  associated  with  other  grave  anomalies  of  develop- 
ment. One  case  is  on  record,  also,  of  a  double  heart.  Verocay  has  de- 
scribed and  figured  a  case  of  seven  hearts  in  a  series  found  in  a  chicken. 

The  heart  may  be  displaced  in  various  ways  as  a  result  of  errors 
of  development  or  acquired  disease.  Thus,  it  may  be  rotated  upon 
its  vertical  or  anteroposterior  axis.  Under  the  term  dextrocardia  are 
recognized  two  conditions:  one,  in  which  the  heart  occupies  a  position 
on  the  right  side  of  the  thorax;  and  the  other,  in  which,  while  the  heart 
is  found  at  its  normal  site,  its  various  chambers  and  sinuses  are  simply 
reversed  (true  dextrocardia).  The  latter  form  is,  as  a  rule,  though  not 
invariably,  found  associated  with  transposition  of  other  viscera.  Ectopia 
cordis  is  a  condition  in  which  the  heart  is  found  outside  the  thoracic  cavity. 
Should  the  organ  remain  high  up  in  the  neck,  we  have  ectopia  cervicalis; 
should  it  enter  the  abdomen  through  a  slit  in  the  diaphragm,  it  is  called 
ectopia  abdominalis;  should  it  appear  beneath  the  skin  of  the  thorax 
through  a  defect  of  the  sternum,  it  is  known  as  ectopia  pectoralis.  In 
such  cases  the  pericardium  is  sometimes  wanting. 

The  chief  anomalies  of  the  heart  as  a  whole  are  bifid  apex,  diverticulum, 
primary  hypertrophy,  and  hypoplasia.  Hypoplasia  is  not  uncommon, 


CONGENITAL  ANOMALIES 


143 


and  is  usually  found  in  the  vessels  as  well  as  the  heart.  The  heart  is 
either  small  at  birth  or  the  whole  vascular  system  gradually  lags  behind 
in  the  general  bodily  development.  In  long-standing  cases,  cardiac 
insufficiency  and  dilatation  come  on.  Rokitansky  has  noted  the  occa- 
sional association  of  the  condition  with  defects  of  the  external  genitalia. 
Bamberger  and,  later,  Virchow  pointed  out  the  relationship  of  cardio- 

FIG.  20 


Heart  showing  (A)  defect  of  interauricular  septum  below  (persistent  ostium  prim  urn),  with  (B 
cleavage  of  right  anterior  segment  of  mitral  valve.  (C)  Interauricular  septum  above  showing 
closed  foramen  ovale.  (D)  Left  posterior  mitral  segment.  From  a  woman,  aged  thirty-two 
years,  without  cardiac  symptoms,  dying  of  perforative  appendicitis.  (From  a  specimen  in  the 
Pathological  Museum,  McGill  University.) 

vascular  hypoplasia  to  chlorosis.  Ortner  and  others  hold  that  the 
condition  predisposes  to  infection.  We,  ourselves,  have  noticed,  post 
mortem,  the  great  frequency  with  which  the  condition  is  present  in 
cases  of  tuberculosis,  especially  in  young  people.  The  vessels  are 
thin-walled  and  show  a  tendency  to  fatty  change. 

The  commonest  defect  of  the  septa  is  the  patent  foramen  ovale.  This 
is  so  frequent  that  it  can  hardly  be  called  an  anomaly,  unless  it  is  so 


144 


THE  MYOCARDIUM 


widely  open  as  to  allow  free  communication  between  the  auricles.  In 
1500  autopsies  at  the  Royal  Victoria  Hospital,  the  condition  occurred 
220  times,  or  in  14.7  per  cent.  When  patent,  the  opening  may  be 
merely  of  pin-point  size  or  may  admit  a  finger.  Defects  of  other  parts 
of  the  auricular  septum  may  also  occur.  These  defects  are  often  asso- 
ciated with  other  abnormalities,  such  as  coloboma.  or  mental  degra- 
dation. 

The  interventricular  septum  may  be  partially  or  totally  wanting. 
Defect  of  this  kind  is  usually  associated  with  pulmonary  stenosis  and 
rechtslage  of  the  aorta,  and  constitutes  one  of  the  commonest  forms  of 
congenital  heart  disease.  When  the  septa  are  completely  wanting,  we 
have  a  two-chambered  heart  (cor  biloculare).  Absence  of  the  ventricular 

FIG.  21 


Defect  of  the  interventricular  septum  at  undefended  space.      Heart  of  infant.      No  other 
anomaly.      (From  a  specimen  in  the  McGill  Pathological  Museum.) 

septum,  with  presence  of  the  auricular,  constitutes  a  three-chambered 
heart  (cor  biatriatum  triloculare).  A  remarkable  instance  of  this  latter 
form  is  to  be  found  in  the  Pathological  Museum  of  McGill  University, 
the  gift  of  Dr.  A.  F.  Holmes,  one  of  the  original  founders  of  the 
Medical  Faculty.1 

The  aortic  septum  may  be  partially  or  completely  defective. 

Pulmonary  stenosis  is  one  of  the  commonest  of  cardiac  anomalies. 
Both  it  and  the  severer  grade  of  atresia  are  commonly  associated  with 
defects  of  the  septum. 

Aortic  stenosis  and  atresia  are  rare. 

The  ductus  arteriosus  Botalli  may  be  absent,  patent,  or  anomalous  in  its 


1  Trans.  Medico-Chir.  Soc.  of  Edin.,  1824.     Reprinted  by  Abbott,  Montreal  Med. 
Jour.,  30:1901:  p.  524. 


MYOMALACIA  CORDIS  145 

course.  Patency  of  the  duct  is  sometimes  associated  with  stenosis  of  the 
pulmonary  artery,  or  of  the  aortic  isthmus.  It  occurred  6  times  in  1500 
autopsies  at  the  Royal  Victoria  Hospital. 

The  coronary  arteries  may  be  increased  in  number,  may  have  an 
abnormal  origin,  or  may  take  an  abnormal  course. 

Aberrant  chordae  tendinese  are  not  uncommon :  a  case,  giving  rise  to  a 
remarkable  musical  murmur,  has  been  recorded  by  W.  F.  Hamilton.1 

Those  desiring  more  detailed  information  than  that  given  here  are 
referred  to  Dr.  Maude  Abbott's  admirable  article  in  Osier's  Modern 
Medicine  (vol.  iv,  1908,  p.  323),  an  article  that  will  be  for  long  the 
"last  word"  on  this  most  important  and  interesting  subject. 


CIRCULATORY  DISTURBANCES. 

Anemia. — This  may  be  part  and  parcel  of  a  general  anemic  condi- 
tion, or  may  be  a  local  condition  due  to  a  narrowing  or  obliteration  of 
the  branches  of  the  coronary  arteries.  A  pericardial  exudate  or  hydro- 
pericardium  also  produces  a  local  anemia  by  pressure.  The  affected 
muscle  has  a  pale  grayish-brown  color.  As  sudden  death  is  frequent 
from  acute  anemia,  the  coronary  vessels  should  always  be  examined 
for  sclerosis,  thrombi,  or  emboli. 

Hyperemia.— Active  Hyperemia. — Active  hyperemia  occurs  in  acute 
infections,  in  abnormal  nervous  conditions,  and  in  death  from  respiratory 
failure. 

Passive  Hyperemia. — Passive  hyperemia  is  found  under  the  same 
conditions  as  elsewhere. 

Hemorrhage. — Interstitial  hemorrhage  is  often  due  to  obliteration 
of  the  arteries,  as  in  hemorrhagic  infarction,  or  to  spontaneous  rupture 
of  the  venules  in  the  state  of  passive  congestion.  Apart  from  these 
conditions  it  is  not  common,  but  is  sometimes  present  in  certain  dys- 
crasias,  as  in  the  various  infective  diseases,  the  hemorrhagic  diatheses, 
leukemia,  pernicious  anemia,  and  in  poisoning  with  phosphorus,  arsenic, 
and  morphine.  In  some  cases  it  is  due  to  the  death  agony. 

Myomalacia  Cordis. — By  this  is  understood  a  degeneration  of  the 
heart  muscle  resulting  from  arterial  anemia.  Atheroma  of  the  coronary 
vessels,  with  its  attendant  thrombosis,  is  the  most  common  cause.  Embo- 
lism in  the  coronaries  is  a  less  frequent  cause,  inasmuch  as  these  vessels 
are  so  placed  that  foreign  bodies  cannot  readily  enter.  The  t-ssence  of 
the  process  is  that  it  is  a  rapid  one,  bringing  about  what  is  practically 
a  white  infarct,  with  subsequent  softening  and  degeneration  of  the 
muscle.2 

The  affected  area,  according  to  its  age  and  vascular  relationship, 
varies  in  appearance  at  different  times.  At  first,  it  is  still  firm  and 
of  a  dull  yellow  color.  Later,  the  patch  becomes  yellowish  white  and 

1  Montreal  Medical  Journal,  28 :  1899 :  508. 

2  See  R.  Marie,  L'infarctus  du  Myocarde  et  ses  consequences,  Paris,  1897. 
10 


146  THE  MYOCARDIUM 

friable.  If  the  neighboring  capillaries  rupture,  a  red  infarct  is  the 
result,  presenting  a  dull,  reddish  appearance.  Later  still,  the  color 
changes  to  a  rusty  brown  and,  as  fibrosis  occurs,  to  a  dull  gray.  Micro- 
scopically, the  affected  muscle  fibers  appear  to  be  swollen,  hyaline, 
and  have  lost  their  characteristic  structure.  If  they  take  the  stain,  it 
is  with  a  diffuse  glassy  appearance.  As  in  other  infarctous  conditions, 
an  inflammatory  reaction  shows  itself  at  the  periphery  of  the  necrobiotic 
area,  when  of  any  standing.  The  site  of  election  for  this  process  is  in  the 
wall  of  the  left  ventricle,  at  or  below  the  junction  of  the  lower  and  middle 
thirds,  at  the  tip  of  the  papillary  muscles,  sometimes  in  the  right  ventricle, 
more  rarely  in  an  auricle. 

Such  localized  areas  of  degeneration  may  produce  a  rupture  of  the 
heart  wall  leading  to  hematopericardium  and  sudden  death,  or,  if 
fibrosis  has  had  time  to  develop,  may  result  in  the  formation  of  a  partial 
aneurism  in  the  wall.  This  is  most  frequent  in  the  anterior  wall  of 
the  left  ventricle,  next,  in  the  posterior,  and  occasionally  in  the  septum. 
Such  aneurisms  may  lead  to  an  imperfect  discharge  of  the  heart's 
contents  and  to  the  formation  of  parietal  thrombi  with  their  attendant 
dangers.  Lazarus-Barlow1  has  described  a  seemingly  unique  case  of 
dissecting  aneurism  of  the  right  heart  wall. 

Fibrosis  of  the  Myocardium. — This  condition  has  much  in  common 
with  the  last  mentioned,  but  differs  from  it  in  that  it  is  a  fibrous  degenera- 
tion of  the  heart  muscle,  which  is  very  slowly  produced.  It  is  often 
called  "chronic  interstitial  myocarditis,"  but  incorrectly,  since  it  is 
primarily  a  degenerative  process.  Owing  to  sclerosis  of  the  coronary 
vessels,  which  leads  to  a  narrowing  of  their  caliber  and  consequently 
impoverished  blood  supply,  the  muscle  bundles  in  the  affected  tract 
undergo  starvation  atrophy.  Associated  with  this  is  proliferation  of 
the  preexisting  interstitial  connective  tissue  which  gradually  invades 
the  degenerating  part — a  true  replacement  fibrosis — and  is  reparative 
in  its  result.  This  is  the  pure  type.  It  cannot  be  denied,  however, 
that  other  cases  exist  in  which,  in  addition  to  the  "replacement  fibrosis," 
there  is  the  production  of  a  cellular  granulation  tissue  that  may  either 
develop  into  scar  tissue  or  may,  in  its  turn,  participate  in  the  degenerative 
change.  Such  inflammatory  changes  must,  we  think,  Le  regarded  as 
due  to  the  irritation  produced,  and  secondary  in  nature.  It  would 
consequently  seem  more  rational  to  include  this  condition  among  the 
degenerations  rather  than  the  inflammations. 

The  scar  tissue  may  be  fairly  diffused  throughout  the  organ,  giving 
it  a  tough  feel  like  leather,  Much  more  commonly,  the  change  is  local- 
ized to  certain  parts.  On  section,  the  muscle  shows  grayish  streaks 
running  parallel  to  the  muscle  bundles,  particularly  in  the  wall  of  the 
left  ventricle  and  at  the  tip  of  the  papillary  muscles.  If  the  condition 
be  more  extreme,  one  sees  irregular  areas  of  a  semitranslucent  appear- 

1  Brit.  Med.  Jour.,  ii:  1899:  1344.  For  dissecting  aneurisms,  see  also  Vestberg, 
Om  dissekerande  hjartaneurismer,  Nordiskt  Med.  Arkiv.,  Ny  Foljd,  7  :  Nos.  26  and 
30  :  1897, 


INFLAMMATIONS  147 

ance,  and  of  a  grayish  or  grayish-white  color,  sharply  defined  from  the 
healthy  muscle,  which  are  sunken  below  the  general  level  of  the  cut 
surface.  These  have  the  appearance  of  tendon.  Besides  the  favorite 
site  in  the  wall  of  the  left  ventricle,  similar  areas  may  be  found  in  the 
columnar  carneae,  the  septum,  or  even  in  the  right  ventricle. 

Microscopically,  the  condition  is  characterized  by  the  presence  among 
the  muscle  bundles  of  larger  or  smaller  fibrous  patches  often  presenting 
a  stellate  appearance.  The  muscle  fibers  about  the  margin  show  definite 

FIG.  22 


Myocardial  fibrosis  from  disease  of  coronary  arteries.      Leitz,  obj.  No.  7  without  oculan 
(From  the  collection  of  Dr.  A.  G.  Nicholls.) 

evidence  of  atrophy,  and  melt  away  into  the  fibrous  mass.  Sometimes 
in  the  centre  of  the  patch  can  be  seen  a  few  isolated  muscle  bands, 
atrophied  by  compression.  The  scar  tissue  is  generally  very  poor  in 
nuclear  elements,  but  toward  the  outer  margin  is  more  cellular.  New- 
formed  capillaries  can  be  made  out,  with  perhaps  small  areas  of  inflam- 
matory infiltration.  The  more  healthy  muscle  fibers  usually  show 
marked  hypertrophy,  as  evidenced  by  the  fact  that  the  nuclei  are  relatively 
large,  stain  deeply,  and  are  blunted  at  the  ends. 


INFLAMMATIONS. 

Acute  Inflammations. — Inflammation  of  the  heart  muscle,  apart 
from  that  which  sometimes  accompanies  ischemic  necrosis,  is  mostly 
due  to  infection  or  intoxication.  The  various  agents  may  reach  the 


148  THE  MYOCARDIUM 

muscle  from  the  pericardium,  or  by  means  of  the  blood  stream.  Wounds 
also  may  be  a  cause. 

A  diffuse  form  of  myocarditis  occurs  in  the  course  of  many  infective 
fevers,  notably  typhoid,  diphtheria,  scarlatina,  rheumatism,  septicemia, 
pyemia,  and  other  similar  conditions.  The  changes  in  the  heart 
muscle  here  must  be  regarded,  we  think,  as  to  some  extent  degenerative. 
The  first  obvious  change  is  that  of  cloudy  swelling,  and  Virchow  is  prob- 
ably correct  in  regarding  this  as  the  first  stage  in  an  inflammatory 
process.  But  besides  this  myocarditis  parenchymatosa,  in  many  cases 
there  is  evidence  of  an  interstitial  process  as  well,  for  we  see  collections 
of  inflammatory  leukocytes  between  the  muscle  fibers,  together  with 
connective  tissue  proliferation.  This  form  is  called  by  Orth  myocarditis 
degenerative^.  The  affected  muscle  is  pale,  soft,  and  friable,  and  often 
cedematous.  Hemorrhagic  points  may  often  be  seen  through  its  sub- 
stance. The  cavities,  particularly  the  left  ventricle,  are  dilated. 

Microscopically,  the  fibers  are  swollen,  the  striation  faint,  and  the 
nuclei  indistinct  (cloudy  swelling).  Vacuolation  is  sometimes  seen, 
and  fragmentation.  If  the  process  continue,  the  nuclei  swell  and  divide 
and  the  fibers  show  fatty  or  hyaline  degeneration.  The  vessels  are 
usually  congested  and  may  show  proliferative  changes.  Collections  of 
inflammatory  leukocytes  in  many  cases  are  seen  between  the  muscle 
fibers,  and  in  the  later  stages  proliferation  of  the  connective  tissue,  with 
the  formation  of  fibroblasts.  In  this  form,  suppuration  does  not  occur. 
If  the  patient  survive,  the  inflammation,  if  slight,  may  entirely  resolve. 
In  other  cases  a  diffuse  fibrosis  of  the  heart  muscle  is  the  result.  In  this 
connection  it  might  be  mentioned  that  Aschoff  and  Tawara1  have 
lately  drawn  attention  to  a  special  form  of  acute  myocarditis  occurring 
in  acute  rheumatism,  in  which  scattered  foci  of  leukocytic  infiltration 
are  found  along  the  course  of  the  vessels,  in  type  suggesting  an  infective 
origin,  but  not  going  on  to  suppuration. 

Purulent  Myocarditis. — Purulent  myocarditis  can  arise  in  various 
ways.  Apart  from  those  cases  which  are  due  to  a  direct  extension  from 
a  purulent  pericarditis  or  an  ulcerative  endocarditis,  an  embolic  infection 
is  the  commonest  cause.  The  so-called  idiopathic  heart  abscess,  where 
there  is  usually  a  single  pus  collection  of  some  size,  is  distinctly  rare. 
Formerly,  many  cases  of  myomalacia  were  erroneously  classed  under 
the  term  abscess.  Most  cases  are  simple  expressions  of  a  general  pyemia. 

On  examination,  the  affected  heart  is  seen  to  be  riddled  with  opaque 
grayish  or  grayish-yellow  dots  the  size  of  a  pin-head  or  larger.  Many 
of  these  are  surrounded  by  a  hemorrhagic  zone.  Small  hemorrhages 
are  often  seen,  and  all  grades  occur  from  this  to  true  pus  collections. 
The  heart  may  be  dilated,  and  show  cloudy  or  fatty  change. 

Microscopically,  these  areas  are  seen  to  consist  of  pus  cells  with 
cell  detritus.  The  vessels  are  often  found  to  be  plugged  with  micro- 
organisms. In  many  cases  the  bacteria  have  penetrated  the  vessel 

1  Die  heutige  Lehre  von  den  pathologischen-anatomischen  Grundlage  der  Herz- 
schwache,  Gustav  Fischer,  1906. 


CHRONIC  INFLAMMATIONS  149 

walls  and  appear  within  the  leukocytes  or  are  lying  free.  The  muscular 
fibers  show  cloudy  swelling  or  fatty  degeneration.  The  vessels  in  the 
neighborhood  are  greatly  congested. 

In  the  event  of  recovery,  the  detritus  may  be  absorbed  and  a  fibrous 
scar  be  produced,  or  the  area  may  become  calcified.  More  often  the 
patient  dies,  but  if  he  survive  for  a  time  the  abscesses  may  coalesce, 
and  rupture  into  the  pericardium  or  into  a  cavity  may  occur.  Thus 
communication  can  be  opened  up  between  two  cavities,  or  a  partial  heart 
aneurism  can  result. 

Acute  Miliary  Tuberculosis. — Acute  miliary  tuberculosis  is  somewhat 
rare,  only  one  case  having  come  under  our  observation.  It  is  a  part  of  a 
disseminated  miliary  process. 

Chronic  Inflammations.— Tuberculosis.1— Most  frequently  the  tuber- 
culous affection  is  an  extension  from  a  tuberculous  pericardium,  especially 
from  the  chronic  caseating  form.  It  is  rarer  to  find  smaller  or  larger 
caseous  foci  in  the  heart  wall.  The  differential  diagnosis  between  this 
condition  and  syphilitic  gumma  is  very  difficult,  and  often  can  only  be 
made  after  a  general  survey  of  the  whole  case,  or  from  the  discovery  of 
the  B.  tuberculosis  in  the  affected  area.  Gummas,  however,  are  apt  to 
be  enclosed  in  a  dense  mass  of  hyperplastic  connective  tissue. 

Syphilis. — Syphilis  is  an  infrequent  cardiac  affection.  According 
to  H.  P.  Loomis,2  syphilis  of  the  myocardium  takes  the  form  of  (1) 
gummatous  foci,  almost  invariably  in  the  wall  of  the  left  ventricle; 
(2)  fibroid  induration,  either  localized  or  diffuse;  (3)  amyloid  infiltration; 
(4)  endarteritis  obliterans,  often  causing  infarctions.  Indurative  inflam- 
mation is  perhaps  the  most  common. 

Solitary  gummas  may  reach  the  size  of  a  pigeon's  egg.  They  are 
rare  in  inherited  syphilis.  Multiple  miliary  gummas  are  also  described. 
Fatty  degeneration  of  the  muscle  is  a  more  frequent  manifestation  of 
the  specific  virus.  Rupture  of  the  wall  or  a  partial  heart  aneurism 
may  result. 

Actinomycosis. — Actinomycosis  is  very  rare,  and  is  secondary  to  actino- 
mycosis  of  the  mediastinum,  lungs,  and  pericardium.  It  takes  the 
form  of  small  granulomas  of  a  grayish  or  yellowish-white  color,  often 
suppurative  in  character. 

Trauma. — Solution  of  the  continuity  of  the  heart  muscle  arises  in 
various  ways.  Wounds  inflicted  by  instruments  or  bullets,  either  wholly 
or  partially  penetrating  the  wall  from  without,  are  not  uncommon.  An 
ulcer  of  the  stomach  has  been  known  to  penetrate  the  wall. 

Foreign  Bodies. — Foreign  bodies  are  sometimes  found  in  the 
heart  wall.  These  may  come  not  only  from  without,  but  from|the 
oesophagus  and  stomach,  and  are  usually  needles,  bullets,  fish  bones, 
knife  blades  and  the  like.  The  condition  may  be  latent  or,  on  the  other 
hand,  lead  to  sudden  death. 

1  For  a  full  description  of  Tuberculosis   of   the   Myocardium,  see  Anders,  Jour. 
Amer.  Med.  Assoc.,  1902:  1081. 

2  American  Journal  of  the  Medical  Sciences,  110:  1895:  389. 


OF    THE 

UNIV/FDCITV 


150  THE  MYOCARDIUM 

Parasites. — Parasites  are  extremely  rare.  Echinococci,  cysticerci, 
'and  pentastoma  have  been  found.  Trichinae  are  said  not  to  be  found. 
Echinococcus  cysts  may  burst  into  a  cavity,  causing  a  general  infection 
'or  sudden  death  from  pulmonary  embolism. 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Simple  Atrophy. — Simple  atrophy  of  the  heart  is  charac- 
terized by  a  general  diminution  in  the  size  and  weight  of  the  organ. 
The  arteries  do  not  partake  so  much  in  the  process,  consequently, 
owing  to  the  shrinkage  of  the  muscle,  they  become  tortuous.  The  epi- 
cardium  and  the  endocardium  alike  from  the  same  cause  present  a 
rumpled  appearance  and  often  secondary  thickening.  The  muscle  is 
firm  and  tough,  and  may  be  of  a  dull,  dark  brown  color — brown  atrophy. 

Microscopically,  the  fibers  are  thinner  than  normal.  The  outline 
of  the  cells  is  well  marked,  owing  to  an  actual  separation  of  the  muscle 
bands.  This  is  regarded  as  being  due  to  a  disintegration  of  the  fibers 
in  consequence  of  impaired  nutrition  of  the  cement  substance.  In  brown 
atrophy,  in  addition,  there  is  a  great  increase  in  the  amount  of  the  pig- 
ment which  is  usually  situated  about  the  poles  of  the  nuclei,  and  the 
pigment  takes  a  dark  brown  color.  In  severe  cases  brown  granules  are 
iseen  to  be  scattered  throughout  the  cell. 

Whether  the  affection  can  occur  as  a  purely  senile  change  has  been 
doubted;  nevertheless,  routine  examination  of  sections  from  the  post- 
mortem room  shows  that  after  middle  age  some  degree  of  brown  atrophy 
is  very  common.  More  certain  causes  are  marasmus,  malignant  disease, 
and  tuberculosis  of  the  lungs.  Some  cases  are  due  to  pressure,  as  from 
mediastinal  lesions  or  from  progressive  relapsing  pericarditis. 

Local  Atrophy. — Local  atrophy  is  due  to  pressure  or  to  sclerosis  of 
small  branches  of  the  coronary  arteries.  Atrophy  of  the  columnse 
carnese  of  the  left  ventricle  which  is  so  common  in  valvular  affections  of 
the  heart  may  result  from  either  of  these  causes. 

Degenerations. — Cloudy  or  Albuminoid  Degeneration. — This  is  a 
very  common  condition.  The  heart  muscle  has  a  grayish,  cloudy, 
opaque  appearance  not  unlike  parboiled  meat  or  raw  fish.  Micro- 
scopically, the  fibers  are  seen  to  be  opaque  from  the  deposit  of  numerous 
minute  granules  which  obscure  the  nucleus  and  transverse  strise.  The 
condition  is  best  made  out  by  making  a  thin  section  of  the  fresh  muscle. 
The  nature  of  the  granules  is  not  definitely  settled,  but  they  appear  to 
be  albuminoid  in  their  character.  On  adding  a  drop  of  acetic  acid  to 
the  section,  the  granules  dissolve  and  the  normal  translucency  of  the 
fibers  is  restored. 

Cloudy  swelling  is  very  common  in  the  course  of  the  infectious  fevers, 
and  is  due  to  the  action  of  specific  toxins  with  or  without  prolonged 
high  temperature,  or  to  disorders  of  metabolism. 

Fatty  Degeneration. — This  common  condition  is  characterized  by  the 
presence  of  minute  globules  of  fat  in  the  muscle  fibers,  which  are  de- 


PATTY  DEGENERATION 


161 


posited  in  small  droplets,  generally  in  the  line  of  the  longitudinal  fibrillae 
of  the  cell.  In  the  severer  affections  the  whole  muscle  fiber  may  be  full 
of  fat.  The  change  often  begins  with  cloudy  swelling,  but  while  acetic 
acid  and  potassium  hydrate  have  the  power  of  restoring  a  cloudy  cell 
to  its  normal  appearance,  these  agents  have  no  effect  on  fat  globules. 
Osmic  acid,  however,  stains  the  fat  black,  and  Sudan  III,  a  carmine 
red.  At  first,  the  transverse  striation  of  the  cells  is  readily  made  out, 
but  tends  to  become  obscured  the  more  advanced  the  degeneration. 
The  condition  may  affect  the  whole  heart,  one  side  of  it,  or  one  side  of 
the  wall  of  a  cavity. 

Microscopically,  the  muscle  is  soft,  friable,  and  of  a  pale  color,  the 
degenerated    portion   showing  up   as   yellowish   streaks   or  specks,  by 

FIG.  23 


Fatty  degeneration  of  the  heart  muscle.  The  fibers  in  the  upper  and  lower  portions  of  the 
field  are  normal.  Those  in  the  centre  contain  fat  droplets,  showing  as  black  dots.  Zeiss  obj 
DD,  ocular  No.  1.  (From  the  Pathological  Laboratory  of  McGill  University.) 

preference  on  the  endocardium,  the  papillary  muscles,  and  in  the  wall 
of  the  left  ventricle.  In  well-marked  cases  the  muscle  has  a  mottled 
appearance — the  so-called  "thrush-breast"  heart. 

In  advanced  fatty  change,  when  there  is  anemia  as  well,  the  heart 
muscle  is  very  pale  and  of  a  yellowish  white  or  clay  color.  In  this  state 
it  is  soft  and  cuts  like  cheese. 

The  main  causes  of  fatty  degeneration  are  general,  that  is  to  say, 
those  acting  through  the  circulation,  and  local. 

The  infectious  fevers,  particularly  typhoid,  and  diphtheria;  the  anemias, 
as  pernicious  anemia,  and  leukemia;  poisons,  like  phosphorus,  arsenic, 
and  sulphuric  acid,  are  the  common  general  causes.  Whether  we  are 


152  THE  MYOCARDIUM 

to  regard  with  Virchow  the  fatty  degeneration  occurring  in  the  infections 
as  a  sequel  of  a  parenchymatous  myocarditis  is  perhaps  doubtful.  Many 
cases,  however,  are  beyond  a  doubt  pure  degenerations. 

The  local  causes  are  sclerosis  of  the  coronaries  and  valvular  lesions. 
Whenever  hypertrophy,  dilatation,  and  incompetence  co-exist,  fatty 
degeneration  is  almost  certain  to  be  present. 

Fatty  degeneration  also  accompanies  inflammation,  as  in  acute  peri- 
carditis and  acute  interstitial  myocarditis. 

Fatty  Infiltration. — Fatty  infiltration  is  not  to  be  confused  with  the  last 
condition.  In  this  the  normal  amount  of  fat  which  is  present  about  the 
base  of  the  heart  and  in  the  coronary  sulci  is  greatly  increased.  The 
fat  forces  its  way  in  between  the  muscle  bundles  and  may  even  appear 
on  the  endocardium.  While  the  heart  is  much  enlarged,  it  is  at  the  same 
time  greatly  weakened,  since  the  muscle  fibers  are  markedly  atrophic — 
a  true  pressure  atrophy  (atrophia  lipomatosa).  In  advanced  cases  the 
front  of  the  right  ventricle  and  even  the  left  may  be  permeated  with 
fat,  in  addition  to  that  which  is  present  in  the  sulci  and  about  the  main 
vessels.  Often  the  apex  of  the  right  ventricle  in  cross-section  shows 
scarcely  any  muscle.  Obesitas  cordis  is  always  a  manifestation  of  general 
corpulency. 

Microscopic  sections  show  merely  masses  of  fat  lying  between  the 
muscle  bundles,  which  are  usually  paler  and  more  yellowish  brown  in 
color  than  healthy  muscle.  The  fibers  are  atrophic,  often  granular,  and 
may  show  signs  of  fatty  degeneration. 

Hyaline  Degeneration. — Hyaline  degeneration  is  a  glassy  or  waxy 
condition  of  the  muscle  fibers,  in  which  they  gradually  lose  their  striation 
and  become  homogeneous,  presenting  a  hyaline,  shiny  appearance  not 
unlike  amyloid,  but  not  giving  the  same  chemical  reactions.  More  often 
the  hyaline  change  involves  areas  of  fibrosis. 

Vitreous  Degeneration. — The  vitreous  degeneration,  which  von  Zenker 
described  in  the  muscles,  especially  the  recti  of  the  abdomen,  and  occur- 
ring in  typhoid  and  other  infectious  diseases,  is  probably  a  localized 
necrotic  change  (vol.  i,  p.  900).  The  fibers  of  the  heart,  when  affected, 
become  brittle  and  break  up  into  lengths,  or  we  may  see  globules  of 
hyaline  material  embedded  in  the  fiber.  In  the  later  stages,  the  inter- 
stitial fibrous  substance  proliferates,  and  we  may  find  it  passing  in  be- 
tween the  various  muscle  fragments.  The  change  is  seen  mainly  in  the 
acute  infections  or  associated  with  parenchymatous  inflammation. 

Segmentation. — Segmentation  of  the  fibers  is  seen  in  hearts  that  are 
dilated,  relaxed,  soft,  and  brittle.  The  individual  fibers  are  separated 
and  broken  up  into  short  lengths,  the  "myocardite  segmentaire"  of  the 
French  writers  (Renaut,  Landouzy).  The  parts  usually  affected  are 
the  septum  and  the  papillary  muscles.  Many  opinions  have  been  ad- 
vanced as  to  the  nature  of  this  condition.  It  occurs  in  cases  of  sudden 
death  from  acute  dilatation  of  the  heart,  in  prolonged  muscular  strain, 
in  the  various  infections  and  intoxications,  in  Bright's  disease,  in 
hyaline  degeneration,  and,  as  Landry  has  shown  at  the  Royal  Victoria 
Hospital,  in  a  large  proportion  of  cases  of  aneurism  of  the  aorta.  Others 


DEGENERATION  OF  THE  HEART  GANGLIA  153 

think  it  is  produced  during  the  death  agony.     It  can  be  produced  experi- 
mentally by  the  action  of  digestive  ferments  upon  the  heart  muscle. 

Hektoen1  recognizes  two  forms,  true  fragmentation,  in  which  the  muscle 
fiber  is  actually  ruptured  at  some  part  of  its  course,  and  segmentation, 
in  which  the  primitive  segments  of  the  fibers  become  simply  dissociated. 
The  first  form  is  due  to  strain  upon  a  weak  and  insufficiently  acting 
muscle.  J.  B.  MacCallum2  concurs  in  this  division  and  is  disposed  to 
regard  segmentation  as  a  form  of  "reversionary"  degeneration.  Both 
Hektoen  and  MacCallum  regard  it  as  of  antemortem  occurrence,  but 
there  are  not  wanting  those  who  regard  the  condition  as  a  simple  artefact. 

FIG.  24 


Fragmentation  of  the  fibers  of  the  heart.      Leitz  obj.  No.  7,  without  ocular.      (From  the 
collection  of  Dr.  A.  G.  Nicholls.) 

Amyloid  Degeneration. — This  never  affects  the  muscle  fibers,  but  the 
interstitial  cement  substance  and  the  bloodvessels.  It  is  part  and  parcel 
of  a  general  amyloid  degeneration.  It  is  rare  to  find  the  process  so 
extreme  that  it  can  be  recognized  macroscopically. 

Calcification. — This  is  usually  a  terminal  event  in  the  course  of  myo- 
malacia  or  of  fibrosis.  The  deposit  of  lime  salts  which  is  seen  so  often 
in  the  thickened  mitral  ring  in  cases  of  mitral  stenosis  may  encroach 
considerably  upon  the  heart  muscle,  and  we  have  seen  one  case  in  which 
a  large  calcareous  mass  in  this  situation  all  but  penetrated  the  wall  of  the 
ventricle. 

Degeneration  of  the  Heart  Ganglia. — This  has  been  described.  Putjatin 
found  it  in  various  chronic  affections  of  the  heart  and  aorta,  and  in 

1  Amer.  Jour.  Med.  Sci.,  114:  1897:  555.          2  Jour.  Exper.  Med.,  4: 1899:409. 


154  THE  MYOCARDIUM 

syphilis.  The  changes  were  fatty  and  pigmentary  degeneration  of  the 
ganglia,  with  hyperemia  and  productive  inflammation  in  the  neighbor- 
hood. No  doubt  some  nutritional  disturbance  is  at  work,  but  the  sig- 
nificance of  the  changes  is  not  made  out,  further  than  that  serious  inter- 
ference with  the  power  and  function  of  the  heart  is  the  result. 

Rupture  of  the  Heart. — Spontaneous  rupture  is  a  rare  event,  and 
never  occurs  unless  the  heart  has  been  weakened  by  disease.  The 
most  frequent  causes  are  fatty  degeneration  of  the  muscle  and  occlusion 
of  the  coronary  arteries.  Less  commonly,  myomalacia,  abscess,  gumma, 
echinococcus  cyst,  and  new-growths  are  responsible.  Rarely  an  aortic 
aneurism  has  ruptured  into  the  auricle,  as  in  cases  reported  by  von 
Wunscheim1  and  A.  McPhedran.2  Death  is  usually  instantaneous, 
but  a  few  cases  have  been  recorded  where  the  patient  has  lived  several 
hours  or  even  days.  Deposits  of  fibrin  are  usually  found  in  the  neighbor- 
hood of  the  tear,  and  more  or  less  blood  is  effused  into  the  pericardial 
sac. 

PROGRESSIVE  METAMORPHOSES. 

Enlargement  of  the  Heart. — This  is  due  to  hypertrophy  or  to  dila- 
tation, or  both,  affecting  one  or  more  cavities.  In  general  hyper- 
trophy of  the  heart  the  organ  has  a  more  rounded  appearance  than 

FIG.  25 


A,  normal  heart  of  rabbit;  B,  hypertrophied  heart  of  rabbit  due  to  repeated  inoculations  with 
small  doses  of  adrenalin  extending  over  several  weeks.  The  adrenalin  causes  contraction  of  the 
arterioles,  heightened  blood  pressure,  and  increased  heart  work.  (From  specimens  of  Dr.  Klotz 
in  the  McGill  Pathological  Museum.  Natural  size.) 

normal,  and  its  transverse  breadth  is  greatly  increased.  The  enlarge- 
ment may  be  so  great  that  the  heart  approaches  that  of  the  bullock 
in  size — cor  bovinum. 

1  Prager  medizinsche  Wochenschrift,  18:  1893:  175. 

2  Canadian  Practitioner,  21:1896:578. 


HYPERTROPHY  155 

Hypertrophy. — Hypertrophy  is  in  most  cases  due  to  increased  resist- 
ance to  the  heart's  action,  provided  that  the  muscle  is  able  to  respond 
to  the  increased  demand.  Or  otherwise,  anything  that  increases  the 
work  of  the  heart,  short  of  causing  cardiac  exhaustion,  leads  to  hyper- 
trophy. We  may  classify  the  causes  as  follows: 

I.  Obstruction  to  egress  of  blood. 

(a)  Endocardia!,  from  stenosis  of  one  of  the  valves. 

(b)  Arterial,  from  diminution  of  the  arterial  lumen,  sclerosis, 

contraction  of  the  smaller  arteries,  etc. 

(c)  Pericardial,  from  complete  synechia.1 

II.  Increase  in  the  volume  of  blood  to  be  propelled. 

(a)  Actual    increase   in    the   amount   of    circulating   blood, 

plethora,  Munich  beer  heart,  etc. 
(6)  From  regurgitation,  as  in  mitral  and  aortic  incompetence. 

III.  Increase  in  rate  of  blood  flow. 

(a)  From  tachycardia,  as  in  exophthalmic  goitre. 

(c)  As  a  response  to  systemic  needs,  as  in  the  athlete.2 

The  chamber  to  be  first  affected  is  that  one  which  first  experiences  the 
unusual  strain.  Thus,  in  arterial  sclerosis,  Bright's  disease,  and  aortic 
endocarditis  the  left  ventricle  is  the  first  to  suffer.  In  pulmonary 
endocarditis  and  many  lung  affections  (fibroid  tuberculosis,  emphysema, 
and  pleuritic  adhesions)  where  there  is  obstruction  in  the  lesser  circula- 
tion, it  is  the  right. 

General  hypertrophy  will,  in  time,  supervene  upon  partial  hypertrophy. 
It  may  occur  from  overexertion,  as  in  excessive  muscular  work,  hysteria , 
neurasthenia,  prolonged  mental  strain,  exophthalmic  goitre,  in  peri- 
cardial  synechiae,  and,  very  commonly,  from  overeating  and  drinking. 
The  Munich  "beer-heart"  is  a  good  example  of  general  hypertrophy 
due  to  the  prolonged  overdistension  of  the  vessels  with  fluid. 

H.  A.  Lafleur3  gives  the  following  statistics,  showing  the  proportion  of 
the  different  causes  in  360  autopsies  at  the  Johns  Hopkins  Hospital. 
Cardiac  hypertrophy  was  found  in  105  cases.  These  were  divided  as 
follows  (in  percentages) : 

1  Here  the  muscle  contracts  against  an  increased  load  and  the  increased  work  leads 
to  hypertrophy.      This   in  the   earlier   stages   of   complete   pericardial   adhesion- 
Through  eventual  exhaustion  the  hypertrophy  is  apt  to  give  place  to  dilatation  and 
atrophy. 

2  The  amount  of  blood  passing  from  the  actively  contracting  skeletal  muscle  is 
much  greater  than  that  from  the  resting  muscle.     In  those  taking  active  exercise 
it  is  only  at  first  that  the  blood  pressure  is  increased;  later  it  may  fall  to,  or  even 
below,  the  normal.     It  follows  thus,  that  to  supply  the  contracting  muscle  the  rate 
of  flow  must  undergo  increase.     In  both  of  these  cases,  a  and  b,  the  accompanying 
increased  rate  of  heart  beat  favoring  this  more  rapid  circulation  must  be  ascribed 
to  reflex  nervous  stimuli. 

3  Montreal  Med.  Jour.,  23:  1895:  851. 


156 


THE  MYOCARDIUM 


Arterial  sclerosis 50.0 

Chronic  nephritis 13.4 

Valvular  lesions  .  12.4 


Pericardial  synechise 
Excessive  muscular  work 

Tumors 

Aneurisms      .... 
Plethora   . 


7.6 

3.8 

1.9 

0.95 

0.95 


FIG.  26 


Cross-section  of    the  heart,  to  show  hypertrophy  of  the  walls.      The  cause;  increased  periphera 
tension.      (Pathological  Museum,  McGill  University.) 

Dilatation. — Reference  has  been  made  in  the  preceding  chapter  to 
the  distinction  between  (physiological)  distension  and  (pathological) 
dilatation.  As  the  hypertrophied  heart  becomes  weaker  the  latter  be- 
comes manifested.  Most  of  the  causes  that  produce  hypertrophy  are 
competent  to  cause  dilatation  in  time.  It  is  a  frequent  result  of  sudden 
strain,  as  in  prolonged  races.  The  most  frequent  cause  of  simple  dila- 
tation is  degeneration  of  the  muscle,  usually  fatty,  such  as  occurs  in 
typhoid  fever,  pneumonia,  and  other  infective  fevers,  and  also  in  per- 
nicious anemia.  Acute  dilatation  may  also  be  induced  by  alcoholic 
excess,  and  experimentally  by  chloroform  and  other  agents.  Local 
dilatation  (aneurism)  has  already  been  referred  to. 

In  dilatation  the  heart  wall  is  generally  thin,  flabby,  softer  than  normal, 
and  pale  in  color  from  fatty  change. 

The  determination  of  the  presence  of  hypertrophy  is  best  made  by 
consideration  of  the  volume  and  weight  of  the  heart.  The  heart  may 
weigh  from  600  to  800  grams.  Stokes  records  one  of  1980  grams.  The 
average  thickness  of  the  left  ventricular  muscle  is  from  15  to  20  mm., 


DILATATION 

FIG.  27 


157 


' 


- 

^  -  .  .  -,  ,  . 

^v">      .^    '%/      '     •£>  '• 

*  *    ,--.Xx"  ^*  **•  *«?  l 


Simple  hypertrophy  of  the  heart.      Leitz  No.  7,  without  ocular.      Shows  large  blunt-ended 
nuclei,  occasionally  dividing.      (Dr.  A.  G.  Nicholls.) 


FIG.  28 


Relative  dilatation  of  the  left  ventricle  of  the  heart.      Patent  foramen  ovale.      (From  the 
Pathological  Museum  of  McGill  University.) 


158  THE  MYOCARDIUM 

but  reference  to  this  standard  only  gives  imperfect  information,  since 
other  factors  may  intervene  to  prevent  a  correct  conclusion  being  formed. 

The  hypertrophied  muscle  is  of  a  marked  brownish-red  color,  with 
often  the  sheen  of  raw  ham.  Its  consistency  is  increased  so  that  when 
cut  it  does  not  collapse,  but  remains  firmly  in  position. 

Microscopically,  the  muscle  fibers  are  increased  in  thickness  and 
probably  in  number  (hyperplasia).  The  nuclei  are  very  large,  deeply 
staining,  with  bluntly  rounded  ends.  Sometimes  they  appear  to  be  in- 
creased in  numbers.  Often  fatty  changes  in  the  bands  can  be  made  out. 

Tumors. — Tumors  of  the  heart  can  be  either  primary  or  secondary. 
By  far  the  majority  are  due  to  the  extension  of  malignant  growths  from 
neighboring  parts,  as  the  pericardium,  mediastinum,  lung,  oesophagus, 
and  stomach,  or  to  metastasis. 

FIG.  29 


Heart  muscle  invaded  by  round-celled  sarcoma.      Reichert  obj.  No.  7a,  without  ocular. 
(Collection  of  the  Royal  Victoria  Hospital.) 

Primary  neoplasms  are  excessively  rare.  This  is  probably  to  be 
attributed  to  the  fact  that  the  heart  above  all  organs  is  constantly  in  a 
state  of  great  efficiency,  well  nourished,  well  innervated,  and  functionally 
always  active,  so  that  it  is  less  likely  to  take  on  aberrant  growth.  The 
usual  growths  are  fibroma,  myxoma,  and  lipoma,  the  relative  frequency 
being  in  the  order  named.  These  are  generally  subendocardial  in  situa- 
tion, and  form  nodular  or  polypoid  outgrowths,  which  project  into  the 
heart  cavity. 

Fibromas  have  been  found  in  various  parts  of  the  heart.  Age  has 
nothing  to  do  with  their  occurrence.  One  case  is  recorded  in  a  child 
aged  three  months.  Many  of  the  cases  are  congenital,  and  to  this  class 


THE  ENDOCARDIUM  159 

belongs  the  interesting  rhabdomyoma,  of  which  twelve  examples  have 
been  recorded.1  These  are  hyperplastic  growths,  with  stellate  trans- 
versely striated  muscle  cells.  One  of  them  was  also  telangiectatic,  and 
is,  no  doubt,  similar  to  a  recorded  case  of  cavernous  angioma,  which 
probably  was  originally  a  myoma.  The  secondary  tumors  are  carcinoma, 
sarcoma,  lymphosarcoma,  and  osteoid  chondroma.  Metastatic  growths 
may  be  found  in  any  position,  subpericardial,  subendocardial,  or  intra- 
mural. Metastatic  carcinoma  is  recorded,  but  is  rarer  than  secondary 
sarcoma:  of  the  latter,  the  melanomas  present  the  most  abundant 
metastases  throughout  the  organ. 

The  results  of  such  growths  are  various.  When  they  project  into  a 
cavity,  thrombosis  and  its  attendant  embolism  may  occur;  fragmentation 
of  the  tumor,  causing  disseminated  metastases;  sudden  death  from 
pulmonary  embolism;  or,  lastly,  rupture  of  the  heart. 


THE  ENDOCARDIUM. 

The  endocardium,  or  lining  membrane  of  the  heart,  consists  of  a  thin 
layer  of  connective  tissue,  containing  elastic  fibrillas  arranged  after 
the  fashion  of  a  fenestrated  membrane.  The  inner  surface  is  covered 
with  a  single  layer  of  flattened  endothelial  plates  similar  to  those  lining 
bloodvessels  everywhere.  Further,  the  endothelial  lining  of  the  heart  is 
in  direct  continuity  with  that  of  the  bloodvessels. 

The  valves  are  formed  by  a  reduplication  of  this  membrane  in  which 
the  fibrous  tissue  has  become  somewhat  thickened.  In  the  case  of  the 
tricuspid  and  mitral  valves,  a  few  muscle  fibers  from  the  myocardium, 
together  with  a  few  small  bloodvessels,  pass  in  to  the  base  of  the  valve, 
but  the  aortic  and  pulmonary  valves  are  devoid  of  even  these. 

The  endocardium  is  richly  supplied  with  lymphatics,  and  the  nerve- 
supply  is  from  a  plexus  situated  in  the  connective  tissue  beneath  the 
endocardial  layer. 

The  anatomical  structure  of  the  membrane  has  an  important  bearing 
upon  the  character  of  the  pathological  processes  affecting  it.  Since 
that  portion  of  the  lining  usually  affected  by  disease  is  devoid  or  rela- 
tively devoid  of  bloodvessels,  we  get  in  inflammation  a  good  example 
of  the  process  as  it  affects  a  non-vascular  structure,  and  for  the  same 
reason  infective  processes  of  embolic  origin  are  of  the  rarest  occurrence. 

Further,  when  erosion  of  the  endothelium  takes  place,  we  find  a 
deposit  of  blood  platelets,  fibrin,  with  resulting  thrombosis. 

Lastly,  the  endocardial  cells  possess  a  marked  phagocytic  power. 

CONGENITAL  ANOMALIES. 

Apart  from  those  conditions  due  to  intra-uterine  inflammation, 
several  malformations  must  be  mentioned.  Fenestration  of  the  aortic 

1  Wolbach,  Jour,  of  Med.  Research,  16:1907:495. 


160  THE  ENDOCARDIUM 

and  pulmonary  similunar  valves  is  so  common  that  it  need  only  be 
referred  to.  An  abnormal  shape  of  the  valves,  owing  to  a  loss  of  sub- 
stance on  the  free  margin,  is  occasionally  met  with.  The  number 
of  the  cusps  of  the  aortic  and  pulmonary  valves  may  be  diminished,  01 
increased  to  four  or  even  five.  The  pulmonary  valve  is  the  more  apt 
to  be  affected.  A  perfect  additional  cusp  may  be  inserted,  or  there  may 
be  merely  a  septum  attaching  the  free  border  of  the  cusp  to  the  ring, 
thus  dividing  the  cusp  into  two  portions.  When  an  extra  cusp  is  inserted 
it  may  be  only  one-half  the  height  of  the  others.  Such  reduplication  of 
the  cusps  is  not  excessively  rare.  Three  instances  have  been  met  with  by 
us  in  1500  autopsies,  and  two  more  are  recorded  by  Powell  White.1 
Three  instances  of  reduplication  of  the  aortic  valves  was  also  met  with 
in  our  series.  Complete  doubling  of  the  left  atrioventricular  ostium 
with  doubled  mitral  orifice  is  much  rarer.  Dr.  M.  E.  Abbott  figures  an 
example  from  the  McGill  collection.2 

Mitral  stenosis  and  atresia  and  bicuspid  stenosis  and  atresia  are 
recorded. 

Hematoma  of  Valves. — These  are  small  bodies,  the  color  of  rasp- 
berry jelly  and  of  pin-head  size,  seen  most  frequently  along  the  closing 
edge  of  the  mitral  segments,  more  rarely  near  the  edge  of  the  aorta  cusps. 
They  are  seen  only  in  infants,  disappearing  in  childhood.  According 
to  Kaufmann,  they  represent  unused  remains  of  the  (vascular)  nodes 
or  eminences  from  which  the  valves  are  developed.  Wegelin,3  in  a 
quite  recent  publication,  points  out  that  in  the  course  of  development 
small  pockets  lined  with  endothelium  are  formed  on  the  ventricular 
aspect  of  the  auriculoventricular  valves  and  on  the  distal  side  of  the 
semilunar  valves.  These  cavities  eventually  become  covered  in  by  the 
proliferation  of  the  endothelium  to  form  closed  sacs.  Valvular  hema- 
tomas  are  always  found  to  be  covered  by  endothelium,  and  Wegelin 
holds  that  they  are  formed  by  the  extravasation  of  blood  into  the 
aforesaid  pockets.  They  are  not  vascular  ectases,  as  some  have 
thought. 


CIRCULATORY  DISTURBANCES. 

(Edema. — Marked  oedema  does  not  occur,  but  a  slight  grade  may 
be  evidenced  by  a  shiny  gelatinous  appearance  of  the  endocardium. 

Hemorrhages. — Subendocardial  hemorrhages  occur  from  blood 
dyscrasias,  such  as  scurvy,  morbus  maculosus  Werlhofii,  pernicious 
anemia,  leukemia,  and  in  the  infections,  like  scarlatina  and  smallpox. 
Venous  hyperemia  or  increased  arterial  pressure  ma,y  lead  to  rupture 
and  hemorrhage.  When,  in  consequence  of  inflammation,  newly  formed 

1  Lancet,  Lond.,  ii:  1898:  1194.  See  also  Osier,  Montreal  General  Hospital  Reports, 
1880. 

2  Osier  and  McCrae's  Modern  Medicine,  4:1908:394. 

3  Frankfurter  Ztschrft.  f.  Pathol.,  2:  1909:  411. 


INFLAMMATIONS  161 

capillaries  are  present  in  the  valves,  plugging  of  these  may  lead  to  hemor- 
rhagic  infarction  of  the  segments. 

Blood  Imbibition. — A  reddish  staining  due  to  blood  imbibition  is 
often  met  with.  It  is  most  probably  a  postmortem  change  and  is  specially 
common  in  septicemia  and  in  infection  with  the  B.  Welchii. 

Thrombi. — When  atheromatous  ulceration  of  the  endocardium  exists, 
or  stenosis  of  any  of  the  ostia,  thrombi  are  apt  to  form  in  the  cavities. 
These  may  form  a  dense  layer  over  the  atheromatous  plaques,  or  may 
form  polypoid  excrescences.  Occasionally  a  ball  thrombus  may  be  pro- 
duced. This  form  is  fairly  frequently  seen  in  the  left  auricle  in  cases  of 
mitral  stenosis.  A  ball  thrombus  may  soften  in  the  centre  and  form  a 
bladder-like  mass  containing  puriform  or  grumous  fluid.  Thrombi  also 
occasionally  calcify. 

Among  other  causes  of  thrombosis  may  be  mentioned  dilatation 
of  the  heart,  slowing  of  the  blood  stream,  and  changes  in  the  composition 
of  the  blood.  Thrombi  are  usually  found  in  the  right  side  of  the  heart, 
particularly  in  the  appendix  and  near  the  apex,  but  are  not  uncommonly 
present  in  the  left  auricle.  A  distinction  should  be  made  between  true 
thrombosis  and  the  clotting  of  the  blood  that  is  so  commonly  produced 
at  the  time  of  death  or  subsequent  to  it  (see  p.  58).  Postmortem 
clots  are  soft,  moist,  translucent,  and  elastic,  and  are  of  a  yellowish-red, 
or  mixed  color.  Extensive  oily  looking  yellow  clots  are  common  in  those 
diseases  where  leukocytosis  is  a  marked  feature,  notably  pneumonia. 
Real  thrombi,  however,  are  reddish  gray,  dry,  friable,  and  opaque.  They 
are  generally  firmly  adherent  to  the  endocardium,  which  often  shows 
some  pathological  change. 

The  thrombi  produced  intra  vitam  are  of  grave  significance,  as  they 
may  lead  to  obstruction  of  the  valvular  orifices  or  to  emboli  in  distant 
parts.  Ewart  and  Rolleston1  have  reported  a  case  where  a  thrombus 
arising  from  the  fossa  ovalis  passed  through  the  mitral  orifice  and  gave 
rise  to  signs  of  mitral  stenosis. 


INFLAMMATIONS. 

Endocarditis. — Inflammatory  changes  in  the  endocardium  may 
affect  any  portion  of  it,  the  valves,  the  tendinous  cords,  the  papillary 
muscles,  or  the  mural  lining.  The  valvular  form  is  by  far  the  commonest, 
and  is  often  associated  with  inflammation  of  the  neighboring  heart 
wall.  For  the  mural  endocardium  to  be  alone  affected  is  one  of  the 
greatest  rarities. 

Acute  Endocarditis. — Primary  endocarditis  is  said  to  occur,  and  does  no 
doubt  from  a  clinical  point  of  view,  but  careful  search  post  mortem  often 
reveals  an  external  cause  for  the  condition,  so  that  from  pathological 
experience  we  must  believe  that  every  case  is  secondary  to  some  other 
condition,  such  as  intoxication  or  infection.  Indeed,  it  may  even  be 

1  Clinical  Society's  Transactions,,  30:  1897:  190. 

M 


162  THE  ENDOCARDIUM 

doubted  whether  intoxication  has  any  influence  except  as  a  predisposing 
cause,  and  whether  all  acute  cases  are  not  due  to  bacterial  invasion. 

Repeated  studies  have  proved  that  the  cases  usually  classed  as  "simple" 
are  due  to  microorganisms.  Consequently,  the  old  division  of  endo- 
carditis into  "  simple"  and  "ulcerative"  no  longer  holds  good  except 
for  convenience  of  description,  for  these  terms  merely  represent  grades 
in  the  one  process. 

Etiologically,  the  condition  cannot  be  regarded  as  a  distinct  entity, 
for  a  great  variety  of  microorganisms  enter  into  its  causation.  Strictly 
speaking,  it  is  more  correct  to  speak  of  a  pneumococcus  or  streptococcus 
infection,  etc.,  with  endocarditis. 

Acute  endocarditis  may  occur  as  a  secondary  manifestation  in  the 
following  diseases :  chorea,  inflammatory  rheumatism,  septicemia,  pneu- 
monia, scarlatina,  tonsillitis,  erythema  nodosum,  peliosis  rheumatica, 
Bright's  disease,  diabetes,  pyelonephritis,  tuberculosis,  smallpox,  typhoid, 
gonorrhoea,  and  malignant  growths.  That  overstrain  is  a  possible  cause 
is  to  some  extent  supported  by  experimental  evidence.1 

The  bacteria  at  work  are  numerous  and  the  infection  may  be  mixed. 
The  chief  organisms  are  the  Diplococcus  pneumonise,  the  Streptococcus 
parvus,  the  Streptococcus  pyogenes,  and  the  Staphylococcus  pyogenes 
aureus;  but  the  B.  coli,  B.  diphtheriae,  B.  influenza?,  B.  pyocyaneus, 
gonococcus,  B.  tuberculosis,  Micrococcus  endocarditidis  rugatus,  Micro- 
coccus  endocarditidis  capsulatus,  B.  endocarditidis  griseus,  have  been 
met  with. 

Endocarditis  due  to  the  gonococcus  is  now  well  recognized,  although 
many  cases  occurring  in  gonorrhoea  are  the  result  of  secondary  infection 
with  pus  organisms.  Thayer  and  Lazear2  have  analyzed  sixteen  cases. 
The  lesions  usually  are  ulcerative  with  large  vegetations,  and  the  aortic 
valve  may  be  affected. 

A  peculiarity  of  endocarditis  that  deserves  mention  is  its  tendency 
to  relapse.  *  Atheromatous  valves  are  also  liable  to  become  inflamed. 
Statistics  show  that  acute  inflammation  supervenes  on  chronically 
sclerosed  valves  in  from  60  to  90  per  cent. 

With  regard  to  the  frequency  with  which  the  various  valves  are  in- 
volved, Washbourn3  refers  to  309  cases  of  infective  endocarditis;  the 
mitral  was  alone  affected  in  115,  the  aortic  in  69,  the  aortic  and  mitral 
together  in  73,  the  tricuspid  in  28,  the  pulmonary  in  19. 

Acute  Granulating  Endocarditis. — Acute  granulating  endocarditis,  some- 
times called  simple  or  rheumatic  endocarditis,  is  produced  by  the  inva- 
sion of  the  endothelial  and  subendothelial  cells  by  bacteria  reaching  these 
tissues  from  the  blood  stream.  The  germs  are,  no  doubt,  taken  up  by 
the  phagocytic  action  of  the  endothelial  cells  and  soon  spread  to  the 
deeper  parts,  where  they  produce  swelling  and  coagulation  necrosis  of 
the  affected  region.  This  process  begins  on  the  closing  surface  of  the 

1  Roy  and  Adami,  Brit.  Med.  Jour.,  ii :  1888 : 1325. 

2  Johns  Hopkins  Hospital  Bulletin,  7:  1896:  57. 

3  The  Pathology  of  Infectious  Endocarditis,  British  Medical  Journal,  ii :  1899 ;  1269, 


ACUTE  GRANULATING  ENDOCARDITIS 


163 


valve,  and  at  first  leads  to  a  grayish,  condensed  appearance.  The  endo- 
thelium  is  soon  shed,  and  as  a  result  a  thrombus,  formed  at  first  of  blood 
platelets,  but  ultimately  of  leukocytes  and  fibrin,  is  produced.  This 
results  in  the  formation  of  warty  excrescences  or  granulations  (endocar- 
ditis verrucosa),  either  as  small  nodules,  or  villous  or  polypoid  out- 
growths (vegetative  endocarditis)  that  may  reach  the  size  of  a  plum. 
These  are  particularly  large  in  pneumococcus  and  gonorrhocel  cases. 
Subsequently,  the  intimal  cells  proliferate  and  there  is  an  exudation 
of  leukocytes.  This  is  a  late  event  in  the  case  of  the  aortic  and  pul- 
monary valves,  as  they  do  riot  contain  bloodvessels,  but  may  be  a 


Acute  verrucose  endocarditis  affecting  the  mitral  valve.  The  efflorescence  of  granulation 
tissue  is  seen  as  a  dark  band  along  the  closing  surface  of  the  mitral  cusps.  (From  the  Patho- 
ogical  Museum  of  McGill  University.) 

marked  feature  in  the  mitral  and  tricuspid  forms  from  an  early  stage. 
New  bloodvessels  may  be  formed  and  grow  into  the  exudation,  so  that 
typical  granulation  tissue  is  produced. 

That  the  infection  may  take  place  through  infective  emboli  in  the 
bloodvessels  of  a  valve  is  said  by  some  to  occur,  but  Orth,  in  a  wide 
experience,  has  never  met  with  an  instance  of  it,  although  it  is  certainly 
possible  in  relapsing  cases,  when  new  vessels  have  been  formed  in  the 


164 


THE  ENDOCARDIUM 


cusps.     Welch  has  pointed  out  the  striking  similarity  between  the  endo- 
carditic  process  and  venous  thrombosis. 

In  very  severe  cases  the  necrotic  process  is  in  excess  of  the  reparative. 
When  this  is  the  case,  if  the  granulations  be  broken  off,  we  often  see 
little  areas  of  suppuration  in  the  affected  valve  (endocarditis  pustulosa). 
This  leads  to  actual  ulceration  with  necrosis,  rupture,  or  perforation  of 
the  valve,  and  the  formation  of  an  acute  valvular  aneurism. 


FIG.  31 


Acute  ulcerative  endocarditis,  affecting  the  aortic  valve  and  the  wall  of  the  left  ventricle. 
(From  the  Pathological  Museum  of  McGill  University.) 

The  characteristics  of  endocarditis  ulcerosa  acuta  are  that  a  part  of  the 
valve  is  destroyed  while  the  remainder  shows  signs  of  a  fresh  inflamma- 
tory process.  The  process  in  its  earlier  stages  reminds  one  of  pharyngeal 
diphtheria,  and  might  easily  be  overlooked.  The  first  stage  is  a  small, 
somewhat  opaque,  yellow  patch,  with  a  slightly  uneven  surface.  In 
other  parts  such  a  patch  may  show  a  distinct  loss  of  substance.  Upon 
these  shallow  ulcers  reddish-gray  thrombotic  masses  are  deposited, 
which  may  attain  considerable  size.  If  the  clot  be  carefully  removed, 
the  tissue  beneath  is  seen  to  be  swollen,  ulcerated,  and  of  a  yellowish 
color. 


CHRONIC  ENDOCARDITIS  165 

Microscopically,  such  a  section  would  reveal  in  the  fibrinous  deposit 
countless  bacteria.  The  tissue  about  the  microorganisms  shows  coagula- 
tion necrosis  with  loss  of  the  nuclei.  Later  on  in  the  process  there  is 
a  definite  leukocytic  infiltration  of  the  necrosing  parts.  In  relapsing 
cases  we  often  see  newly  formed  capillaries  distended  with  blood,  and 
frequently  hemorrhage  into  the  inflamed  area.  True  suppuration  in 
this  form  is  decidedly  rare. 

The  affection  may  spread  to  the  base  of  the  valve,  to  the  tendinous 
cords,  or  to  the  endocardium  of  the  ventricular  and  even  of  the  auricular 
wall. 

An  unbroken  continuity  of  the  inflammatory  process  is  not  necessary, 
as  the  inflamed  areas  may  be  sporadic  in  their  distribution.  This  is 
due  to  the  vegetations  on  the  valve  coming  in  contact  with  the  heart  wall 
during  the  movements  of  the  heart,  thus  bringing  about  a  direct  infec- 
tion. In  the  milder  forms,  healing  may  take  place  with  fibroblastic 
change  and  calcification.  Generally,  however,  the  condition  leads  sooner 
or  later  to  death. 

One  of  the  results  of  acute  endocarditis  may  be  that  small  portions  of 
the  thrombi  may  break  loose  and  cause  infarction  in  the  brain,  spleen, 
or  kidneys.  These  are  the  more  dangerous  if  they  contain  micro- 
organisms, for  in  this  way  miliary  metastatic  abscesses  are  produced. 
Besides  the  production  of  incompetence  or  stenosis  of  a  valve,  already 
referred  to,  the  inflammation  may  spread  to  the  myocardium  or  even  the 
pericardium,  and  sometimes  leads  to  abscess  of  the  heart  and  rupture. 

Chronic  Endocarditis. — There  are,  etiologically,  two  conditions  which, 
since  their  end  results  are  very  similar,  have  until  the  last  few  years  been 
regarded  as  one  process  under  the  common  heading  of  Chronic  Endo- 
carditis. These  are  chronic  endocarditis  proper  or  secondary  sclerosis, 
and  primary  or  work  sclerosis. 

Secondary  Sclerosis. — It  is  the  outer  non-vascular  portions  of  the 
valves,  along  the  proximal  margin  of  the  line  of  apposition  of  the  cusps, 
that,  with  rare  exceptions,  become  the  primary  seat  of  acute  inflamma- 
tion. And,  to  repeat,  the  succession  of  events  occurring  in  the  inflamed 
areas  is  identical  with  that  seen  in  the  cornea  and  other  non-vascular 
areas,  with,  in  addition,  a  marked  liability  to  the  formation  of  thrombotic 
vegetations  upon  the  ulcerated  surfaces.  Thus,  save  when  the  process 
assumes  a  rapid,  progressively  destructive  type,  there  occurs  a  vasculari- 
zation  of  the  cusps,  and  later  a  new-growth  of  fibrous  cicatricial  tissue 
in  the  region  of  the  previous  ulcer;  this,  in  part  through  proliferation  of 
the  connective  tissue  of  the  deep  layers  of  the  cusps,  in  part  through 
organization  of  the  thrombi.  The  result,  therefore,  of  a  single  localized 
focus  of  ulcerative  inflammation  is  a  local  fibrosis  with  some  contraction 
of  the  involved  area  of  the  cusp. 

Thus,  localized  cicatricial  areas  may  surely  be  ascribed  to  previous  in- 
flammation. The  cusp  so  affected,  becoming  deformed,  is  relatively 
weakened,  and  at  the  same  time  subject  to  increased  strain  in  the  per- 
formance of  normal  function.  Hence,  in  the  first  place,  as  a  locus  minoris 
resistentice,  it  is  more  liable  to  succumb  to  a  second  infection  if  patho- 


166  THE  ENDOCARDIUM 

genie  agents  again  appear  in  the  circulation,  and  hence,  more  particularly 
in  acute  rheumatism,  in  which  recurrent  infection  is  peculiarly  liable  to 
occur,  we  are  apt  to  find  indications,  clinical  and  anatomical,  that  the 
same  valve  (most  often  the  mitral)  has  been  the  seat  of  repeated  attacks 
of  inflammation,  and,  as  a  consequence,  exhibits  extensive  fibrosis  and 
deformity.  In  the  second  place  the  deformed  valve  cannot  function 
normally :  the  new  tissue  is  not  the  equivalent  of  the  old :  now  under 
what  for  a  normal  valve  are  normal  conditions  it  is  subjected  to  strain, 
.and  the  result  is  that  complicating  the  inflammatory  sclerosis,  there  is  a 
marked  liability  for  adaptive  or  work  sclerosis  to  show  itself  of  the  type 
to  be  described  immediately.  It  is  this  frequent  combination  in  the 
same  cusp  of  the  two  types  that  renders  it  difficult  in  case  after  case  to 
distinguish  the  sequence  of  events. 

FIG.  32 


Calcareous  infiltration  of  the  aortic  valves,  with  regurgitation.      Dilatation  of  the  left  ventricle: 
atrophy  of  the  columnse  carnese.      (From  the  Pathological  Museum  of  McGill  University.) 

Primary  Sclerosis. — In  discussing  the  nature  of  arteriosclerosis  in 
Chapter  VIII,  it  will  be  demonstrated  that  the  pronounced  fibroid 
thickening  of  the  intima  of  the  arteries  seen  in  this  condition  is  the 
direct  result  of  increased  strain  to  which  this  layer  may  become  sub- 
jected: that,  in  brief,  increased  work  within  physiological  limits  leads  to 
increased  growth  of  connective  tissue,  as  of  other  cells  of  the  organism. 
That  strain,  we  shall  show,  may  be  brought  about  by  either  actual  of 
relative  increase  in  the  blood  pressure.  Now,  accompanying  this  condi- 
tion of  arteriosclerosis  we  encounter  time  and  again  an  identical  condition 
of,  more  particularly,  the  aortic  valves,  although  in  succession  all  the 


TUBERCULOSIS  1G7 

valves  may  come  to  show  the  same  changes.  (These  valves,  it  must  be 
remembered,  are  merely  folds  of  the  vascular  intima.)  Namely,  we  find 
that  the  cusps  are  the  seat  of  a  diffuse  fibrosis.  The  new  tissue  is  laid 
down  in  orderly  layers,  the  most  superficial  being  the  most  recent;  it  is 
non-vascular;  there  is  the  greatest  development  in  those  parts  of  the 
cusps  that  are  subjected  to  the  greatest  strain — at  the  bases  and  along 
the  margins  of  apposition  of  the  aortic  cusps;  along  the  areas  of  appo- 
sition of  the  mitral  cusps ;  the  deeper  layers  like  those  of  the  sclerosed 
intima  are  apt  to  exhibit  hyaline  degeneration  necrobiosis  or  atheroma, 
calcification  and  ulceration.  The  process  is  identical  with  what  we  find 
in  arteriosclerosis,  and  just  as  in  that  condition  we  conclude  that  the 
intimal  changes  are  non-inflammatory,  so  we  cannot  but  conclude  that 
this  type  of  valve  thickening  is  equally  non-inflammatory. 

FIG.  33 


Heart  viewed  from  above,  showing  stenosis  of  aortic,  mitral,  and  pulmonary  ostia.      (From 
the  Pathological  Museum  of  McGill  University.) 

But  now,  just  as  in  the  inflammatory  type,  a  strain  sclerosis  is  apt  to 
supervene,  so  it  has  to  be  recognized  that  in  this  there  may  be  second- 
ary inflammatory  disturbances.  The  valves  are  abnormal  and  so  more 
liable  to  irritation.  Thus,  it  is  not  uncommon  to  find  the  aortic  cusps 
undergoing  fusion  along  their  opposed  angles. 

In  both  forms  of  sclerosis  we  find  certain  common  features.  The  new 
connective  tissue  manifests  the  characteristic  tendency  of  new  connective 
tissue  in  general  to  undergo  contraction,  hence  the  aortic  and  pulmonary 
cusps  undergo  shortening  along  their  free  edges,  with  resulting  incom- 
petence. It  is  usual  to  state  that  the  mitral  cusps  undergo  fusion.  This 
is  a  false  conception.  The  mitral  and  the  tricuspid  do  not  possess  dis- 
tinct cusps :  they  form  a  veil  or  tube  hanging  into  the  ventricular  cavity, 
which  veil  is  now  longer,  now  shorter,  but  continuous  around  its  whole 
circumference.  The  length  of  the  different  portions  varies  considerably 


168  THE  ENDOCARDIUM 

in  different  individuals.  When  there  is  fibrosis  and  contraction,  it  de- 
pends upon  the  relative  proportions  of  the  different  sections  whether 
there  develops  a  funnel-shaped  or  a  button-hole  stenosis,  the  latter 
occurring  when  the  short  areas  joining  the  longer  so-called  cusps  are  of 
small  dimensions. 

In  both  forms  also,  degenerative  changes,  calcification  and  athero- 
matous  ulceration,  may  show  themselves,  as  also  the  chordae  tendinese 
may  undergo  thickening  and  contraction,  the  latter  at  times  so  extreme 
that  the  cusps  appear  to  be  inserted  upon  the  apices  of  the  papillary 
muscles. 

Tuberculosis. — The  occurrence  of  simple  (so-called)  endocarditis 
in  connection  with  tuberculosis  elsewhere  is  not  uncommon  in  our 
experience.  True  tuberculous  lesions  of  the  valves  are,  however,  rare. 
Miliary  tubercles  have  been  seen  on  the  valves  in  cases  of  general  miliary 
affection,  but  such  are  more  common  on  the  mural  endocardium  about 
the  conus  arteriosus  pulmonalis.  The  specific  bacilli  have  been  detected 
in  the  lesions.  The  experimental  work  of  Michaelis  and  Blum  has 
demonstrated  the  possibility  of  this  form. 

Syphilis  is  also  rare. 

Traumatism. — Rupture  of  one  of  the  cusps  of  the  healthy  aortic  valve 
is  recorded,  but,  as  would  be  expected,  is  more  often  found  in  a  diseased 
valve.  It  is  due  to  excessive  muscular  strain.  The  tendinous  cords 
may  give  way  from  the  same  cause. 

ALTERATIONS  IN  THE  SIZE  OF  THE  OSTIA. 

Tricuspid  Valve. —Stenosis. — Stenosis  of  the  tricuspid  valve  may 
be  congenital  or  acquired.  The  congenital  form  is  due  either  to  a  defect 
of  development  or  to  fcetal  endocarditis.  It  is  rare  for  the  tricuspid 
to  be  alone  affected.  Usually  mitral  stenosis  exists  as  well;  less  fre- 
quently both  mitral  and  aortic  stenosis  are  present.  Rarely,  an  adherent 
thrombus  may  obstruct  the  passage. 

Insufficiency. — Insufficiency  is  usually  caused  by  relative  dilatation  of 
the  right  ventricle  owing  secondarily  to  mitral  lesions  and  to  affections 
that  increase  the  vascular  tension  within  the  lung,  such  as  emphysema 
and  fibroid  induration.  Primary  insufficiency  due  to  endocarditis  is 
not  common. 

Pulmonary  Valve. — Stenosis. — Stenosis  of  the  pulmonary  ostium  is 
almost  invariably  congenital,  and  is  generally  associated  with  grave 
defects  of  development,  such  as  patent  ductus  Botalli  or  imperfect  septum. 
Rarely,  in  endocarditis,  a  thrombus  may  more  or  less  completely  occlude 
the  opening. 

Insufficiency. — Insufficiency  is  excessively  rare.  It  may  occur  from 
rupture  of  an  acute  aneurism  of  the  valve,  or  from  other  manifestations 
of  endocarditis. 

Mitral  Valve. — Stenosis. — Stenosis  is  nearly  always  caused  by 
endocarditis,  and  is  usually  combined  with  insufficiency.  As  a  rule, 
the  obstruction  is  brought  about  by  the  fusion  of  the  valve  segments, 


RETROGRESSIVE  METAMORPHOSES  169 

together  with  calcification,  so  that  a  narrow  orifice  vis  produced.  An 
adherent  thrombus  upon  one  of  the  cusps  may  produce  a  similar  effect. 
Not  only  does  the  thickening  and  fusion  of  the  cusps  lead  to  obstruction, 
but,  owing  to  the  impossibility  of  closure,  leakage  is  a  common  result. 
The  relationship  between  the  stenosis  and  regurgitation  varies  con- 
siderably in  different  cases. 

Insufficiency. — Insufficiency  may  be  due  to  loss  of  substance  of  the 
cusps,  retraction,  or  dilatation  of  the  ventricle  (relative  insufficiency). 

Aortic  Valve. — Stenosis. — Pure  aortic  stenosis  is  rare.  It  is  due  in 
part  to  the  fusion  of  the  semilunar  valves  or  to  narrowing  of  the  aortic 
ring,  but  the  most  important  element  is  the  deposit  of  lime  salts  in  the 
cusps  and  ring.  This  prevents  the  proper  collapse  of  the  cusps  during 
systole.  Aortic  stenosis  is  commonly  associated  with  insufficiency. 

Aortic  Insufficiency. — Insufficiency  may  be  due  to  ulceration  and  loss 
of  substance  of  the  valve  or  to  the  contraction  that  results  from  chronic 
endocarditis.  Rarely,  it  is  due  to  rupture  of  a  cusp.  Relative  insuffi- 
ciency is  not  very  uncommon,  and  is  usually  secondary  to  dilatation  of 
the  left  ventricle,  or  to  dilatation  of  the  first  part  of  the  aorta  from 
atheroma  or  aneurism. 

The  Lesions  Associated  with  Valvular  Disease. — In  acute  endo- 
carditis the  heart  muscle  is  invariably  affected  to  some  extent,  owing  to 
the  action  of  the  same  toxic  cause.  We  find  not  only  cloudy  and  fatty 
degeneration  of  the  fibers,  but  also  in  some  cases  acute  interstitial 
myocarditis,  and  hyaline  thrombi  in  the  smaller  vessels.  Again,  acute 
pericarditis  may  be  set  up.  This  is  more  frequent  in  children,  and 
is  most  likely  to  occur  in  aortic  endocarditis,  for  the  reason  that  there 
is  but  a  small  distance  between  the  valve  and  the  pericardial  sac,  viz., 
the  thickness  of  the  aortic  wall.  It  is  fairly  common  at  autopsy  to 
find  in  cases  of  endocarditis  fine  adhesions  at  the  upper  cul-de-sac 
of  the  pericardium.  Small  portions  of  the  vegetations  may  break  off 
and  give  rise  to  embolism  in  remote  organs,  or,  if  infective,  to  multiple 
abscesses. 

In  the  case  of  chronic  endocarditis,  changes  in  the  heart  wall  are 
often  marked.  In  the  degenerative  and  sclerotic  form,  the  coronaries 
are  apt  to  be  affected  from  the  same  cause,  leading  to  myodegeneration. 
In  aortic  insufficiency,  owing  to  the  imperfect  filling  of  the  aorta,  the 
blood  pressure  within  the  coronaries  is  diminished,  and,  in  consequence 
the  heart  becomes  atrophic.  Banti  has  also  pointed  out  that  venous 
stasis  within  the  heart  causes  degeneration  of  the  muscle  bands  and 
interstitial  fibrosis,  a  condition  that  he  terms  venous  cirrhosis.  A  most 
important  result  of  valvular  affections  is  hypertrophy  and  dilatation 
of  the  heart  muscle  and  cavities.  The  mechanics  of  this,  however, 
has  been  discussed  fully  in  another  place  (see  p.  128). 

RETROGRESSIVE  METAMORPHOSES. 

Degenerations. — Fatty  Degeneration. — Fatty  degeneration  appears 
in  the  form  of  slightly  elevated  scattered  patches  of  a  yellowish-white 


170  THE  ENDOCARDIUM 

color  upon  the  valves  and  less  frequently  on  the  mural  endocardium. 
These  are  due  to  the  transformation  of  the  protoplasm  of  the  connective 
tissue  and  endothelial  cells  into  fat.  In  advanced  cases,  fat  droplets 
may  be  seen  in  the  spaces  between  the  connective-tissue  cells.  The 
condition  is  seen  usually  in  elderly  people,  but  is  not  very  uncommon  in 
younger  individuals,  in  cases  of  marasmus,  anemia,  valvular  disease, 
intoxications,  and  infections.  The  first  stage  of  atheroma  is  fatty  change. 

Mucoid  Degeneration. — Mucoid  degeneration  occurs  particularly  in 
advanced  life  and  almost  without  exception  upon  the  valves.  Circum- 
scribed nodules  of  gelatinous  appearance  at  the  closing  edge  of  the  valve 
are  of  this  nature.  They  may  contain  true  myoma  cells  or  may  be 
merely  masses  of  gelatinous  substance.  The  condition  is  often  associated 
with  fatty  degeneration. 

Amyloid  Disease. — Amyloid  disease'  not  infrequently  affects  the  sub- 
endocardial  connective  tissue  under  the  same  conditions  as  elsewhere. 
It  is  often  combined  with  hyaline  degeneration. 


PROGRESSIVE  METAMORPHOSES. 

Tumors  have  already  been  dealt  with  under  the  heading  "Myo- 
cardium." Kanthack  and  Pigg1  have  recorded  a  unique  casein  which 
a  carcinoma  of  the  testis,  or,  more  accurately,  a  teratogenous  blastoma 
(see  vol.  i,  p.  603),  formed  secondary  growths  lying  free  in  the  right  heart 
and  inferior  vena  cava. 

1  Trans.  Path.  Soc.,  London,  48: 1897: 1391. 


CHAPTER    VIII. 

THE   VESSELS.     VASCULAR   FUNCTION   AND   ITS   DISTURBANCES. 

\Yi:  are  apt  to  repeat  glibly  that  the  arteries  are  composed  of  three 
coats — intima,  media,  and  adventitia — and  with  this  to  regard  the  whole 
arterial  tree  as  uniform  throughout,  save  that  the  constituents  of  the 
different  coats  become  progressively  reduced  as  we  pass  from  the  aorta 
to  the  arterioles.  Undoubtedly  there  is  a  certain  amount  of  truth  in 
this  general  conception,  but  undoubtedly,  also,  we  have  thus  far  allowed 
the  view  to  prevail  too  fully,  to  a  neglect  of  the  study  of  the  histology  of 
individual  arteries.  We  still  need,  for  example,  more  exact  information 
regarding  the  extent  and  variations  in  the  deeper  musculo-elastic  layer 
of  the  intima,  to  which  Jores  has  called  attention,  and,  as  Meigs  points 
out,  until  we  study  the  arteries  of  different  regions  not  under  various 
degrees  of  contraction  as  we  encounter  them  in  the  usual  run  of  post- 
mortem tissue,  but  uniformly  expanded  (and  this  has  not  been  done), 
we.  obtain  wholly  false  ideas  as  to  the  relative  development  of  the  dif- 
ferent layers. 

Failing  such  exact  study,  we  have  to  content  ourselves  with  laying 
down  that  the  arteries  may  be  divided  into  the  two  broad  groups  of  those 
of  the  elastic  type,  and  of  the  muscular,  respectively.  The  presence  of 
abundant  layers  of  yellow  elastic  tissue  such  as  we  find  characteristically 
in  the  aorta  and  its  main  branches  connotes  two  things:  (1)  That 
the  vessel  is  capable  of  undergoing  passive  dilatation  up  to  a  certain 
point,  and  of  returning  passively  to  the  normal  when  the  distending 
force  is  removed;  and  (2)  that  only  with  difficulty  can  it  be  contracted 
beyond  a  certain  mean,  the  very  elasticity  of  the  tissue  acting  as  a  counter- 
acting force  against  obliteration  or  collapse  of  the  vessel.  Here  it  may 
be  noted  that  the  wrinkled,  wavy  appearance  of  the  internal  elastic 
lamina,  the  "  plicated"  membrane,  or,  as  the  French  term  it,  the  "bande- 
lette,"  of  medium-sized  arteries  is  a  postmortem  appearance,  due  to  rigor 
and  contraction  of  the  muscle  coat.  This  appearance,  of  course,  must 
be  reproduced  when  the  arterial  muscle  is  contracted,  but  be  absent  in 
the  uncontracted  vessel.  As  we  shall  have  to  note  later,  even  in  arteries 
of  the  elastic  type,  there  is  fairly  abundant  muscle  in  the  form  of  layers 
between  the  elastic  sheaths  which  must  play  a  part  in  modifying  the 
caliber.  It  is  generally  accepted  that  this  musculature,  from  the  begin- 
ning of  the  aorta  down  to  the  smallest  arterioles,  has  the  property  of  auto- 
matic contraction  similar  to  that  possessed  by  the  heart  muscle.  It  is 


172  THE  VESSELS 

at  the  other  extreme,  in  the  arterioles,  that  the  muscular  sheath  is  most 
prominent,  the  elastic  least.1 

Very  slight  changes  in  the  tonus  of  the  small  arteries  of  the  body  must 
induce  great  changes  in  the  stream  bed,  and  must  materially  alter  the 
volume  of  the  blood  passing  through  the  vessels  and  the  presence  of  the 
same. 

So  far  as  we  can  see,  capillary  contraction  must  be  left  out  of  account 
as  a  primary  factor  in  causing  rise  of  blood  pressure.  With  Meltzer 
and  Leonard  Hill  we  are  prepared  to  admit  that  the  capillaries  possess 
some  power  of  contraction;  we  doubt,  however,  whether  this  is  sufficiently 
great  to  be  of  any  effect  when  the  arterioles  are  uncontracted  and  supply 
blood  to  the  capillary  area  at  relatively  high  pressure;  only  when  the 
arterioles  are  contracted  and  the  blood  supply  low  does  it  appear  possible 
that  the  contraction  can  be  effective.  It  is  by  capillary  contraction  under 
those  conditions  that  we  would  explain  the  rather  striking  pallor  of  the 
ordinary  arteriosclerotic  individual.2  The  size  of  the  capillaries,  that  is, 
depends  in  the  main  upon  the  amount  of  arterial  blood  supply  on  the 
one  hand,  the  venous  pressure  on  the  other.  At  the  same  time,  the  capa- 
city of  the  capillary  system  is  so  much  greater  than  that  of  the  arterioles, 
that  were  the  latter  generally  dilated,  the  free  passage  of  blood  into  the 
wider  capillary  channels  would  almost  immediately  empty  the  arterial 
tree  and  bring  the  circulation  to  a  stop  by  the  individual  bleeding 
into  his  own  capillaries.  We  know  that  this  may  happen  when  dilata- 
tion occurs  merely  of  the  arteries  of  the  splanchnic  area.  The  muscular 
arterioles,  then,  as  guarding  the  gateway  into  the  capillaries,  are,  with  the 
heart,  the  main  agents  in  maintaining  the  circulation;  and,  as  a  rule, 
through  the  marvellously  developed  vasomotor  apparatus,  they  act  in 
absolute  harmony  with  the  heart  on  the  one  hand,  with  the  needs  of  indi- 
vidual organs  on  the  other.  Now  one,  now  another  organ  may  demand 
a  larger  blood  supply,  and  presumably  in  the  main,  through  reflex  stimu- 
lation, the  arteries  to  that  organ  undergo  dilatation,  but  with  this,  pre- 
sumably also  in  the  main  under  the  control  of  the  vasomotor  centres, 
other  arteries  contract,  and  thus  a  mean  arterial  blood  pressure  of  about 
120  mm.  Hg  is  preserved  with  great  regularity. 

1  It  may  well  be  that  the  size  of  a  plain  muscle  fiber  relative  to  the  caliber  of  a 
vessel  determines  to  some  extent  the  need  or  lack  of  need  of  an  elastic-tissue  frame- 
work; that  in  an  arteriole,  for  example,  the  range  of  contraction  of  a  muscle  fiber 
relative  to  the  load  that  fiber  has  to  bear  (namely,  to  the  blood  pressure)  may  be 
such  that  there  is  little  danger  of  overextension  of  the   muscle;   that,  in  short 
the  presence  of  elastic  tissue  in  the  aorta  and  larger  arteries  is  a  factor  of  safety, 
preventing  excessive  strain  on  the  muscular  elements  of  the  wall.     In  a  small  artery 
a  very  slight  expansion  or  contraction  of  the  circular  fibers  will  induce  a  relatively 
great  alteration  in  the  size  of  the  lumen,  altogether  out  of  proportion  to  the  change 
in  size  of  the  large  aorta  produced  by  the  same  extent  of  contraction  of  its  muscula- 
ture. 

2  With  prolonged  reduction  of  blood  supply  to  a  part  we  should  expect  to  obtain 
a  venous  congestion  owing  to  the  lack  of  vis  a  tergo;  this,  characteristically,  is  wanting 
in  the  arteriosclerotic  as  contrasted  with  the  sufferer  from  Raynaud's  disease. 


VASOMOTOR  MECHANISM  173 

Everyone  is  familiar  with  the  existence  of  vasoconstrictor  and  vaso- 
dilator nerves;  we  need  not  here  enter  into  a  description  of  their  action; 
what  is  important  is  to  determine  whether  these  alone  determine  the 
contraction  and  expansion  of  the  arteries.  The  indications  are  that  we 
have  the  identical  problem  before  us  that  we  had  in  connection  with 
the  heart.  There  is  evidence,  that  is,  that  besides  the  central  nervous 
control  exerted  from  centres  in  the  bulb,  there  exists  a  system  of  nerve 
cells  with  processes  tending  to  form  a  plexus  in  the  arterial  wall,  and 
further,  that  the  muscle  fibers  of  the  media  are  capable  of  direct  stimula- 
tion. Cut  through  the  nerve  supply  of  a  limb,  and  immediately  the  arte- 
ries lose  their  tone  and  dilate,  but  eventually,  although  still  unconnected 
with  the  central  nervous  system,  they  regain  their  tone.  There  is  one 
very  important  series  of  arteries  in  which  no  one  has  yet  been  able  to 
discover  any  proper  vasomotor  system — namely,  the  cerebral  arteries. 
At  most,  individual  nerve  cells  are  to  be  made  out  in  the  arterial  walls, 
provided  with  processes;  from  which  it  would  appear  that  these  cerebral 
vessels  possess  a  self-regulating  apparatus — that  the  condition  of  the 
brain  matter  and  the  nature  of  the  circulating  blood  determine  the  blood 
flow  through  them.  The  indications  are  that  the  brain  is  superior  to 
all  other  organs,  and  that  it  governs  its  own  blood  supply  untrammelled 
by  vasomotor  interference  from  other  parts. 

The  above  conditions  might  be  determined  through  the  local  nerve 
mechanism.  On  the  other  hand,  we  know  how  rapidly  nerve  cells  die 
when  cut  off  from  their  blood  supply  or  when  removed  from  the  living 
body.  Now,  as  Mac  William  has  shown,  the  larger  arteries  removed 
from  the  body  after  death  respond  to  direct  stimuli,  and  are  capable  of 
a  strong  contraction  many  hours  after  death.  More  precise  data  to  the 
same  effect  have  been  supplied  by  Brodie  and  Dixon,1  who  have  shown 
that  the  vasoconstrictor  nerves  to  the  limbs  are  no  longer  irritable 
three  hours  after  death,  while  six  hours  after  death  the  arteries  still  con- 
tract under  the  influence  of  adrenalin.  This  indicates  independent 
contractibility  of  the  arterial  muscle  fibers  in  response  to  direct  stimuli. 
We  will  not  here  discuss  the  finer  points  brought  out  by  those  observers, 
Ellicott2  and  H.  H.  Dale,  as  to  the  existence  of  modes  of  stimulation 
through  the  neuromuscular  junction  and  through  the  muscle  substance 
proper.  Adrenalin,  pituitary  extract,  and  ergot  have  all  been  shown  to 
act  directly  upon  the  arterial  muscle,  and  judging  from  the  similarity 
of  their  effects,  the  same  is  true  of  a  large  number  of  other  bodies,  barium 
chloride,  nicotine,  etc.  We  must  accept,  therefore,  all  three  modes  of 
stimulation  as  inducing  contraction  of  the  arterial  muscle,  giving  the 
place  of  honor,  in  normal  conditions,  to  control  from  the  vasomotor 
centres.  It  is  this  that  determines  the  dilatation  with  profound  lowering 
of  the  blood  pressure  seen  in  syncope  and  shock.  Whether  it  is  the 
essential  cause  of  general  relaxation  of  the  arteries,  with  lowering  of  the 
blood  pressure  seen  in  the  acute  fevers,  is  still  a  matter  of  some  debate. 

1  Jour,  of  Physiol.,  30:  1904:  494. 

2  Ibid.,  32:  1905:  401 


174  THE  VESSELS 

That  relaxation  may  show  itself  early  in  a  fever  is  evidenced  by  the  dicrotic 
pulse,  and  then  it  may  be  accompanied  by  no  weakening  of  the  heart 
action,  but,  if  anything,  by  the  very  reverse.  It  has  to  be  admitted  from 
the  experiments  of  Roger  and  others  that  different  bacterial  toxins  act  in 
different  degrees  upon  the  different  segments  of  the  coordinating  appa- 
ratus; some,  like  the  diphtheria  toxin,  appear  to  act  directly  upon  the 
heart,  others  directly  upon  the  arterial  wall.  But  the  general  trend  at 
the  present  time,  strongly  supported  by  the  experiments  of  Romberg 
and  his  pupils,  is  to  lay  the  greatest  stress  upon  the  direct  action  of  the 
bacterial  toxins  upon  the  vasomotor  centres  in  the  medulla.  The  oppo- 
site condition  of  vasocontraction  has,  of  late  years,  attracted  not  a  little 
notice,  and  that  from  a  clinical  point  of  view.  The  indications  are  that 
arterial  spasm — prolonged  extreme  contraction  of  the  arteries  of  indi- 
vidual areas — is  a  not  uncommon  condition.  To  this  condition  and  its 
results  we  have  referred  in  discussing  Raynaud's  disease.  Pal1  more 
particularly  has  studied  the  vascular  crises  affecting  the  splanchnic 
vessels;  Osier  and  others,  the  contracture  of  arteries  of  the  lower 
extremities  leading  to  intermittent  claudication  or  limping,  due  to  the 
sudden  cutting  off  of  blood  supply  and  muscular  anemia.  As  Sir  Lauder 
Brunton  indicates,  arterial  contraction  probably  plays  an  important 
part  in  a  very  common  form  of  migraine  known  at  times  as  "bilious 
headache."  In  this  form,  with  the  development  of  premonitory  symp- 
toms, the  blood  pressure  is  found  distinctly  raised  well  above  the  normal, 
and  it  continues  to  rise.  Such  rise  can  only  be  due  to  arterial  or  arteriolar 
contraction,  and,  as  a  matter  of  fact,  superficial  arteries,  like  the  tern 
porals,  can  be  seen  firm,  contracted,  and  whipcord-like.  When  the 
condition  has  become  almost  unendurable,  and  the  patient  finds  himself 
thoroughly  exhausted,  the  attack  passes  off,  the  headache  disappears, 
the  pulse  becomes  soft,  the  circulation  resumes  its  normal  condition. 
In  such  cases  there  would  seem  to  have  been  not  merely  a  local,  but  a 
general  vascular  crisis;  it  is  difficult  to  explain  the  raised  blood  pressure 
otherwise.  How  this  is  brought  about  we  cannot  positively  say;  those 
liable  to  the  condition  know  full  well  that  certain  errors  in  diet  surely 
invoke  this  Nemesis.  It  is  possible,  on  the  one  hand,  that  certain  products 
of  imperfect  metabolism  or  other  poisons  act  generally  on  the  arteries, 
but  the  direct  action  of  the  same  on  the  vasoconstrictor  centres  would 
equally  explain. 

Here,  leading  up  to  the  consideration  of  arteriosclerosis  and  its  causa- 
tion, a  phenomenon  must  be  noted  which  has  been  emphasized  by 
Leonard  Hill — namely,  the  paradox  that  arteries  tend  not  to  expand  but 
to  contract  under  heightened  internal  pressure,  and  this  so  immediately 
that  the  contraction  must  be  a  local  reaction,  not  reflex.  When  in  con- 
junction with  this  we  realize  that  the  smaller  the  circumference  of  the 
artery  the  greater  is  the  effect  of  the  contraction  of  the  muscle  cells  upon 
the  reduction  of  the  arterial  lumen  and  the  diminution  of  the  blood 
stream,  we  are  led  to  see  that  this  phenomenon  leads  to  the  establishment 

1  Pal/Gefiisskrisen,  Leipz.,  1905.    S.  Hirzel. 


ARTERIOSCLEROSIS  175 

of  a  vicious  cycle.  The  higher  the  blood  pressure,  the  greater  becomes 
the  contraction  of  the  arterioles;  the  less,  therefore,  the  blood  supply 
to  the  tissues  and  the  greater  the  call  upon  the  central  nervous  system 
for  more  blood.  Whether  from  reflex  stimulation  of  the  heart  to  in- 
creased activity  in  order  to  supply  the  tissues,  or  from  direct  automatic 
action  of  the  increased  aortic  pressure  in  raising  the  intraventricular 
pressure,  and  so  stimulating  the  ventricles  to  more  forcible  contraction, 
the  blood  pressure  becomes  yet  higher,  and,  as  a  result,  the  arteries  still 
further  contracted.  It  is  along  these  lines  that  we  would  explain  the 
progressive  rise  of  blood  pressure  and  contraction  of  the  smaller  arteries 
in  migraine.  In  this  order  of  cases  we  must  suppose  that  the  eventual 
result  is  a  veritable  spasm  of  the  arteries,  which  continues  until  the 
muscle  fibers  become  exhausted,  dilate,  and  cause  lowering  of  the  blood 
pressure  and  return  to  the  normal.  That  exhaustion  is  the  only  means 
whereby,  in  general,  the  blood  pressure,  once  raised,  becomes  reduced, 
we  do  not  for  a  moment  mean  to  suggest.  These  vascular  crises  are  the 
exception  and  not  the  rule;  there  must  be  other  reflexes;  must,  for  ex- 
ample, be  the  means  of  pouring  into  the  blood  substances  which  neutralize 
the  agents  causing  arterial  contraction  and  high  blood  pressure  in  the 
first  place.  But  in  this  class  of  cases  these  opposing  agencies  must  be 
either  inadequate  or  temporarily  inhibited.  The  studies  on  the  ductless 
glands  have  demonstrated  that  the  system  produces  both  internal  secre- 
tions, which  raise  the  blood  pressure  (e.  g.,  products  of  the  activity  of 
the  adrenal  and  pituitary  bodies),  and  others  which,  on  the  contrary, 
reduce  it  (e.  g.,  the  thyroid  extract).  At  most,  what  we  desire  to  empha- 
size here  is  that  arterial  contraction,  and  particularly  a  generalized 
arteriolar  contraction,  is  the  primary  cause  of  heightened  blood  pressure. 
It  would  require  a  very  much  greater  increase  in  force  and  frequency 
of  the  cardiac  contractions  to  raise  and  maintain  the  blood  pressure  that 
we  ordinarily  encounter  if,  with  rise  of  blood  pressure,  the  arteries 
underwent  a  corresponding  dilatation.  The  drugs  which  characteristic- 
ally cause  heightened  blood  pressure  of  any  duration  act  by  contracting 
the  arterioles. 

Clinically,  what  is  a  more  common  event  than  arterial  spasm  is  a 
persistent  rise  of  blood  pressure,  or  state  of  "hyperpiesis,"  as  Sir  Clifford 
Allbutt  has  termed  it.  In  this,  for  long  periods  the  blood  pressure, 
instead  of  being  in  the  neighborhood  of  120  mm.  Hg,  is  raised  to  180, 
200,  or  250  mm.  Hg.,  or  even  higher.  It  is  this  continued  rise  of  blood 
pressure,  due  as  we  have  said  to  increased  contraction  of  the  arterioles, 
that  is  the  commonest  precursor  of  arteriosclerosis. 


ARTERIOSCLEROSIS. 

Strange  to  say,  for  that  which,  in  civilized  lands  among  those  attain- 
ing adult  life,  is  the  commonest  of  all  morbid  states,  we  possess  no 
adequate  and  comprehensive  name.  It  is  a  condition  in  which  evi- 
dently the  arteries  are  primarily  involved,  and  what  is  the  most  obvious 


176  ARTERIOSCLEROSIS 

lesion  is  a  fibrotic  thickening  of  the  intima,  whence  the  term  arterio- 
sclerosis has  obtained  wide  acceptance.  It  is,  however,  doubtful  whether 
sclerosis  (hardening)  is  the  essential  change,  and  certainly  the  later 
stages  in  the  larger  vessels  are  of  the  nature  of  a  softening  and  degenera- 
tion; whence  Marchand  has  suggested  the  name  atherosis,  and  this  is 
being  widely  taken  up  by  German  workers.  But  here  again  the 
"d&qpy"  or  porridgy  state  of  intimal  degeneration,  only  affects  the 
larger  arteries,  and  is  a  secondary  and  not  a  primary  condition.  The 
name  given  thirty  years  ago  by  Gull  and  Sutton  of  "  arteriocapillary 
fibrosis"  is  quite  as  defensible,  and  that  because  a  fibrosis  of  the  arterioles 
characterizes  the  most  important  group  of  cases,  and  has  a  very  clear 
relationship  to  the  development  of  the  changes  seen  in  the  larger  vessels. 
We  shall  speak  of  general  arteriosclerosis,  waiting  for  some  thoroughly 
satisfactory  name  to  be  proposed  in  the  future. 

More  accurately  there  are  two  main  causes  of  arteriosclerosis,  either 
(1)  increased  strain  thrown  upon  the  arterial  wall  by  heightened  blood 
pressure,  or  (2)  a  weakened  state  of  the  wall,  either  from  congenital 
causes  or  from  disease  of  the  same.  If  the  pressure  be  normal  but 
the  walls  weakened,  the  results  are  of  the  same  order  as  when  the 
pressure  is  heightened  but  the  walls  of  normal  resisting  power.  It 
is  the  lack  of  recognition  of  this  central  fact  that  is  at  the  bottom  of 
the  confusion  that  has  reigned  all  these  years  regarding  the  etiology  of 
arteriosclerosis.  It  is  not  high  pressure  alone  that  causes  arteriosclerosis; 
that  condition  may  show  itself  without  rise  of  blood  pressure  above  the 
normal;  but  it  is  the  ratio  between  the  resisting  powers  of  the  vessel  wall 
and  the  pressure  to  which  they  are  subjected  from  within.  If  this  be  ac- 
cepted, then,  secondly,  we  are  forced  to  realize  that  the  arterial  tree  is 
by  no  means  necessarily  equally  resistant  in  all  its  parts.  In  some  indi- 
viduals, whether  from  hereditary  or  acquired  conditions,  the  aortic 
wall  is  relatively  weak  compared  with  the  walls  of  the  smaller  vessels; 
in  them  the  aorta  may  become  affected  when  there  is  little  or  no  change 
in  the  smaller  arteries  or  arterioles.  In  others  the  aorta  is  resistant  and 
the  smaller  arteries  weak  and  apt  to  show  arteriosclerotic  change.  In 
others  the  change  is  universal — although  in  these  the  series  of  alterations 
seen  in  the  smaller  vessels  are  from  a  histological  point  of  view  wholly 
different  from  those  seen  in  the  aorta. 

Before  going  farther  and  adducing  the  observations  upon  which 
these  statements  are  based,  it  becomes  essential  to  describe  briefly  the 
changes  seen  in  arteriosclerosis  of  arteries  of  different  size,  that  the  series 
of  changes  to  which  we  must  refer  may  be  clearly  understood. 

Aortic  Arteriosclerosis. — The  slightest  change  observable  in  the  aorta 
is  the  appearance  of  small  streaks  in  the  intima,  opaque,  white,  fatty 
looking,  and  tending  in  general  to  a  longitudinal  arrangement.  They 
are  most  common  in  those  dying  from  infection  and  acute  intoxication. 
Microscopic  examination  shows  that  they  are  not,  as  usually  considered, 
confined  to  the  endothelium,  but  represent  a  fatty  degeneration  of  the 
deepest  layer  of  the  intima,  the  musculo-elastic  layer.  Their  arrange- 
ment and  position  makes  us  doubt  whether  these  "fatty  streaks"  beaj 


AORTIC  ARTERIOSCLEROSIS  177 

any  relationship  to  the  series  of  changes  to  be  presently  noted   con- 
stituting arteriosclerosis  proper. 

Besides  these,  as  demonstrated  by  Klotz  and  confirmed  by  Saltykow, 
certain  bacteria  and  their  toxins  lead  to  a  definite  proliferation  of  the 
aortic  endothelium  and  intima — a  true  proliferative  intimitis.  Again, 
we  are  doubtful  whether  this  should  be  regarded  as  a  true  arterio- 
sclerosis, or,  more  accurately,  we  lack  evidence  that  this  intimal  thicken- 
ing proceeds  onward  to  afford  the  familiar  picture  of  the  atheromatous 
aorta.  The  typical  arteriosclerotic  aorta  shows  changes  which,  to  the 
naked  eye,  characteristically  affect  the  intima.  That  undergoes  a 
notable  thickening,  not  uniform  but  nodose,  although  in  advanced 
cases  the  thickened  plaques  may  be  so  close  that  they  fuse  into  large 
areas.  In  these  plaques  we  observe  a  succession  of  stages.  The  slightest 
cases  are  those  of  either  proliferation  of  the  superficial  layers  of  the  in- 
tima, forming  a  layer  of  dense  fibrous  tissue,  the  fibers  running  parallel 
to  the  surface,  or  of  a  somewhat  similar  proliferation  of  the  deeper, 
musculo-elastic  layer  of  the  intima,  not  so  purely  fibrous,  but  exhibiting 

FIG.  34 


Section  of  the  aorta  from  a  case  of  nodose  arteriosclerosis,  to  show  the  bulging  and  thinning 
of  the  media,  prepared  by  Dr.  Matthewson.  X  8  diameters.  The  section  shows  also  the  hyaline 
degeneration  of  the  deeper  layers  of  the  overgrown  intima,  and  the  persistence  of  a  fine  layer  of 
less  altered  intima  tissue  immediately  beneath  the  media.  The  media  in  this  case  showed 
evidences  of  calcareous  degeneration  in  patches  with  some  hyaline  change. 

also  a  proliferation  of  yellow  elastic  tissue.  In  either  case  we  deal  \v  ith 
simple  hyperplasia  of  the  intima,  with  no  sign  of  leukocytic  infiltration, 
of  new  vessel  formation,  or  of  inflammation  as  usually  accepted.  More 
often  we  encounter  degenerative  changes  in  these  plaques.  The  layers 
nearest  to  the  intimal  surface  may  show  little  or  no  change,  but  deeper 
down  (1)  the  layers  become  swollen  and  hyaline,  or  (2)  as  a  more  ad- 
vanced change,  exhibit  fatty  degeneration,  loss  of  nuclear  staining,  dis- 
integration of  the  tissue,  with  presence  of  tablets  of  cholesterin;  in  short, 
evidence  of  necrosis  and  autolysis;  this  is  the  typical  atheromatous 
material.  (3)  Suitably  stained,  as  by  von  Kossa's  method,  such  softened 
areas  show  also  the  presence  of  calcareous  matter,  and  this  may  accumu- 
late, becoming  more  and  more  abundant,  until  gritty  masses  and  extensive 
brittle  plates  of  calcification  become  developed.  In  either  of  these 
later  stages  the  superficial,  thin,  but  hitherto  intact  layer  of  the  intima 
may  give  way  and  be  torn  off,  an  atheromatous  ulcer  becoming  formed, 
shallow,  and  with  rough  necrotic  floor. 

These  in  brief  are  the  changes  undergone  by  the  intima.      Normally 
that  intima  possess  no  vessels;  its  nutrition — and  that  also,  we  may  add 
12 


178 


ARTERIOSCLEROSIS 


of  the  inner  portion  of  the  media — is  gained  in  part  from  the  aortic 
lumen  by  infiltration  of  the  blood  plasma.  Obviously,  with  the  progres- 
sive laying  down  of  layers  of  dense  fibrous  tissue,  the  nourishment  of  the 
older,  deeper  layers  becomes  cut  off  and  necrosis  results.  Save  in  syphi- 
litic cases,  it  is  only  exceptionally  that  we  encounter  a  secondary  granu- 
lation process  occurring  in  the  atheromatous  plaque,  with  entry  of  capil- 
lary loops  from  the  vasa  vasorum  of  the  media,  and  when  this  is  the  case 
we  meet  with  a  distinct  reparative  process,  absorption  of  the  atheroma- 
tous material,  and  laying  down  of  new  fibrous  tissue  to  replace  that 
which  has  undergone  necrosis.  Where  this  is  the  case,  the  plaques, 
instead  of  remaining  flattened,  become  puckered,  often  with  an  obscurely 
stellate  depression.  This  puckering  is,  we  may  add,  the  main  naked 
eye  indication  of  syphilitic  aortitis. 

But  while  thus,  macroscopically,  the  intima  is  the  site  of  the  most 
marked  change  in  aortic  arteriosclerosis,  microscopic  examination  with 
the  employment  of  suitable  stains  demonstrates  that  the  media  is  also 
involved — nay,  is  the  seat  of  the  primary  change.  This  change,  so  far  as 
we  have  at  present  determined,  may  be  of  one  of  two  orders. 

Syphilitic  Mesaortitis. — A  frequent  source  of  aortic  arteriosclerosis 
in  those  of  early  middle  age  is  syphilis.  The  researches  of  Heller  and 
his  pupils  and  of  Chiari  (which  since  have  been  abundantly  confirmed 
in  other  laboratories)  have  proved  that  the  primary  lesion  here  is  a 
small-celled  or  granulomatous  infiltration  of  the  media,  along  the  course 


FIG.  35 


y 


Section  from  aorta  of  syphilitic  mesaortitis  to  show  extreme  degeneration  of  media  and  ab- 
sorption of  elastic  tissue:  I,  thickened  intima;  M,  media,  the  darkest  parts  being  the  elastic 
tissue.  At  X  this  has  disappeared.  At  Y,  round-celled  infiltration. 

of  individual  vasa  vasorum.  Klotz,  Bruns,1  and  Wiesner2  have  pointed 
out  that  a  similar  change  may  be  encountered  in  congenital  syphilis. 
Accompanying  the  infiltration  there  is  a  well-marked  localized  atrophy 


1  Berl.  klin.  Woch.,  8:  1906:  217. 

2  Centralb.  f.  allg.  Path.,  16:  1905:  822. 


SENILE  DEGENERATION  OF  MEDIA  AND  ARTERIOSCLEROSIS     179 


and  disappearance  of  both  elements  of  the  medial  coat,  of  the  muscular 
and  elastic-tissue  layers,  the  absorption  of  the  latter  being  very  striking 
(Klotz).  This  is  the  primary  change.  The  process  does  not  extend 
beyond  the  media  into  the  intima,  but,  as  a  secondary  process,  that 
intima  undergoes  proliferative  thickeneng.  It  is  only  at  a  later  period, 
when  the  fibrosis  has  given  place  to  atheroma,  that  the  vessels  above 
noted  extend  into  the  necrotic  area.  We  see  here  a  syphilitic  mesaortitis 
followed  by  intimal  sclerosis  and  its  sequelae.  As  to  the  relationship 
of  the  syphilitic  lesion  to  aneurism  production,  we  shall  speak  later. 
This  form  of  arteriosclerosis  is  frequently,  but  not  necessarily,  accom- 
panied by  high  pressure  and  peripheral  arterial  sclerosis — frequently 
because  your  syphilitic  is  apt  to  indulge  his  various  appetities,  and  if, 
according  to  Cabot,  alcoholism,  contrary  to  the  general  opinion,  is  not 
a  cause  of  arteriosclerosis,  all  are  agreed  that  overeating  is. 

Senile  Degeneration  of  the  Media  and  Arteriosclerosis.— 
Another  well-marked  type  of  aortic  arteriosclerosis  is  the  senile.  This 
also  is  not  necessarily  accompanied 
by  high  pressure,  and  that  in  spite 
of  well-marked  signs  of  peripheral 
sclerosis.  With  advancing  age  the 
force  of  the  heart  beat  becomes  pro- 
gressively weaker,  and  the  blood 
pressure  tends  normally  to  diminish ; 
and  thus,  what  for  a  middle-aged 
adult  would  be  a  normal  blood 
pressure  is  relatively  a  high  pressure 
in  an  old  man.  The  main  feature 
is  the  widespread  alteration  in  the 
media  of  the  aorta  and  larger  ves- 
sels with  dilatation  and  tortuosity. 
In  these  cases  the  internal  thicken- 
ing of  the  aorta  is  apt  to  be  not 
nodular,  but  more  diffuse,  without 
puckering,  and  whereas  the  syphi- 
litic lesion  has  as  its  site  of  election 
the  first  part  of  the  aorta,  here  we  not 

infrequently  find  comparatively  little  intimal  change  in  the  arch,  which,  on 
the  contrary,  may  show  thinning  of  its  wall  and  some  diffuse  enlargement; 
where  the  condition  is  not  generalized  it  is  the  lower  part  of  the  abdominal 
aorta  that  is  most  involved.  Working  in  our  laboratory  at  the  Royal 
Victoria  Hospital,  Klotz  has  called  attention  to  the  fact  that  if  the  aortas 
of  those  thirty-five  and  upward  be  examined — aortas  not  necessarily 
showing  any  sign  whatever  of  intimal  sclerosis — and  if  these  be  treated 
to  demonstrate  the  existence  of  calcareous  deposits,  it  is  rare  to  encounter 
a  section  which  does  not  show  some  degeneration  of  the  media.  The 
earliest  change  is  in  the  middle  layers  of  this  coat,  and  then  in  connection 
with  the  muscle  cells.  These  show  first  some  fatty  change,  later  a  fine 
powdering  with  calcareous  granules;  later  the  muscle  cells,  as  such, 


Section  of  human  aorta  of  elderly  individual 
treated  by  von  Kossa's  method,  to  demonstrate 
calcification  of  media,  and  more  particularly  of 
the  muscular  bands.  (Klotz.) 


180  ARTERIOSCLEROSIS 

become  indistinguishable,  much  shrunken,  so  that  the  elastic  bands 
on  either  side  become  approximated,  separated  by  a  collection  of  the 
fine  calcareous  granules;  later  the  elastic  lamellae  exhibit  also  cal- 
careous degeneration.  It  is  at  times  remarkable  what  extreme  degen- 
eration of  the  media  may  be  found  in  a  comparatively  thin  and  not 
particularly  rigid  aorta  exhibiting  none  of  the  ordinary  signs  of  arterio- 
sclerosis. These  observations  are  in  complete  harmony  with  earlier 
observations  upon  the  progressive  loss  of  elasticity  of  the  aorta  with 
advancing  life.  Whereas  strips  of  the  aortas  of  young  individuals 
have  great  elasticity,  there  is  little  stretching  power  in  strips  taken  from 
elderly  individuals.  These  observations  of  Klotz  show  that  not  only 
the  elastica  but  also  the  muscle  cells  participate  in  the  progressive 
degeneration. 

Moenckeberg's  Sclerosis.— Moenckeberg  was  the  first  to  direct 
attention  to  the  widespread  degeneration  of  the  media.  He  was  of 
the  opinion  that  it  was  a  condition  quite  distinct  from  intimal  sclerosis. 
Now,  it  is  quite  true,  as  we  have  pointed  out,  that  it  may  occur  in  a 
diffuse  form  without  accompanying  thickening  of  the  intima ;  indeed,  with 
some  thinning  of  the  same.  Indeed,  it  can  immediately  be  diagnosticated 
in  the  lower  abdominal  aorta  and  the  common  iliacs  and  their  branches 
by  the  development  of  a  succession  of  what  are  truly  shallow  aneurismal 
pouches  lying  with  their  long  axes  transverse,  with  intervening  ridges. 
But  here  we  have,  only  on  a  somewhat  larger  scale,  the  phenomenon 
noted  in  connection  with  syphilis,  namely,  that  one  and  the  same  cause 
now  leads  to  intimal  thickening,  now  to  aneurism.  Why  this  is  we  shall 
explain  later.  We  would  only  note  here  that  all  arteriosclerosis  of  the 
senile  type  presents  this  underlying  medial  degeneration;  that  this  is 
very  common;  that  it  involves  also  the  middle-sized  arteries  in  which 
medial  calcification  may  be  extreme;  and  that  the  " pipe-stem  radial," 
for  example,  is  not  an  example  of  intimal,  but  of  medial  calcification. 

Nodose  Aortic  Sclerosis:  Hyperpiesis.— It  is  clear  that  the  un- 
ceasing recurrent  strain  of  the  pulse  wave  seventy  times  a  minute  or 
thereabouts,  through  the  whole  twenty-four  hours,  day  after  day, 
year  after  year,  eventually  wears  out  the  elastic  tissue  of  the  aorta. 
The  rate  at  which  it  does  so  varies,  and  undoubtedly  there  enters  an 
hereditary  factor,  so  that  in  some  families  this  senile  change  appears  at 
a  comparatively  early  period,  in  others  is  long  delayed;  but  in  all,  sooner 
or  later,  this  loss  of  elasticity  shows  itself.  Where,  in  addition,  there  are 
causes  leading  to  marked  continued  contraction  of  the  arterioles,  there 
the  prolonged  rise  of  blood  pressure  materially  hastens  the  giving  way 
of  the  media.  It  is,  we  admit,  difficult  to  draw  a  sharp  line  between  the 
senile  arteriosclerosis  and  the  arteriosclerosis  accompanying  this  state 
of  hyperpiesis.  The  two  merge  one  into  the  other;  but  when  clinically 
we  encounter  high  blood  pressure  in  those  without  syphilitic  taint,  there 
anatomically  we  find  not  so  much  a  diffuse  intimal  thickening  as  a  nodose 
arteriosclerosis.  And  here,  as  Thoma  was  the  first  to  lay  down  with 
precision,  we  find  evidence  of  local  and  restricted  giving  way  of  the 
media.  As  he  showed,  the  first  localities  to  give  way  are  those  of  natural 


N6D03E  AOkflC  SCLEROSIS:  HYPERPIESIS  l8l 

weakness.  Thus,  the  earliest  regions  to  show  the  arteriosclerotic  change 
are  at  and  around  the  mouths  of  the  intercostal  and  other  arteries,  where 
the  regular  order  of  the  muscle  and  elastic  bands  of  the  media  becomes 
interrupted  or  pushed  to  one  side  to  allow  the  exit  of  the  lateral  arteries.- 
Here,  we  find  the  intima  presenting  the  sanle  proliferation  and  fibroid 
change  noted  in  the  other  conditions,  passing  on  lo  necrotiCj  and  later' 
calcareous  change  in  the  lower  layers,  With  this,  other  scattered  of' 
sporadic,  plaque-like  foci  of  fibrosis  show  themselves  along  the  length 
of  the  aorta,  and  these  may  eventually  show  atheroniatous  change  and 
ulceration.  That  the  media  gives  way  in  these  cases  was  demonstrated 
in  a  striking  manner  by  Thoma.  Post  mortem,  when  the  aorta  is  opened, 

FIG.  37 


Atheromatous  plaques  on  the  lining  of  the  aorta.      (Graupner  and  Zimmermann.) 

these  nodes  of  intimal  thickening  project  well  above  the  general  surface; 
Thoma  showed  that  if  the  recently  removed  aorta  be  filled  with  warm 
tallow  at  blood  pressure,  and  it  be  cooled  and  the  aorta  cut  away 
from  the  solid  core  of  tallow,  this  is  found  perfectly  cylindrical  with  no 
depressions  corresponding  to  the  plaques;  or  otherwise,  in  life  these 
intimal  thickenings  evidently  fill  little  bays  in  the  media,  the  intimal 
proliferation  compensating  for  the  giving  way  of  the  middle  coat,  and 
the  aortic  lumen  being  thus  kept  of  even  diameter.  This  experiment 
of  Thoma's  does  not  always  succeed;  there  are  even  cases  in  which  the 
intimal  thickening  is  in  excess  of  the  medial  giving  way — cases  of  over- 
compensation  ;  and  there  are  those  who  deny  that  in  every  case  the  media 
shows  thinning  at  the  regions  corresponding  to  the  intimal  plaques. 


182 


ARTER IOSCLEROSIS 


FIG.  38 


But  if  in  certain  cases  this  thinning  is  not  very  obvious,  appropriate  stain- 
ing shows  often  that  the  media  at  these  areas  is  degenerated;  that  the 
thickening  is  only  apparent,  due  to  the  elastic  contraction  which  causes 
the  intimal  mass  to  be  projected  post  mortem  into  the  aortic  lumen. 

It  should  be  added  regarding  the  extent  of  necrosis  and  calcification 
that  this  may  involve  the  most  internal  layers  of  the  media.  As  already 
noted,  the  nutrition  of  these  inner  layers  is,  in  part,  at  least,  from  the 
lumen  of  the  aorta,  and  consequently  suffers  when  there  is  this  deposit 

of  impermeable  fibrous  tissue  in  the 
intima.  So  as  not  to  confuse  the 
reader  by  the  introduction  of  minute 
details,  we  have  purposely  neglected 
to  lay  stress  upon  the  minute  anat- 
omy of  the  arteriosclerotic  change. 
Many  recent  workers,  notably  Jores 
and  Marchand  and  Aschoff  and 
their  schools,  have  paid  attention 
to  the  histology  of  the  changes  here 
described;  they  have  more  particu- 
larly called  attention  to  the  increase 
in  yellow  elastic,  as  well  as  white 
connective  tissue  in  the  intima,  and 
there  has  been  not  a  little  diver- 
gence regarding  the  respective  parts 
played  by  muscular  and  elastic  tis- 
sue degeneration  in  the  media,  the 
existence  or  non-existence  of  spon- 
taneous rupture  of  the  elastic  tissue 
lamellse,  etc.  But  these  matters  do 
not  modify  our  conception  of  the 
broad  nature  of  the  main  process. 
To  epitomize  so  far  as  concerns 
aortic  sclerosis  we  have  determined: 

1.  In  the  vast  majority  of  cases,  if  not  in  all,  a  weakness  and  giving 
way  of  the  media  is  the  primary  anatomical  lesion. 

2.  There  is  the  possibility  that,  as  the  result  of  a  subacute  proliferative 
intimitis,  due  to  bacteria  and  their  toxins,  the  thickening  of  the  intima, 
by  cutting  off  the  nutrition  of  the  inner  layers  of  the  media,  may  weaken 
that  coat,  and  so  cause  a  local  dilatation  of  the  aortic  lumen,  followed  by 
a  secondary  and  further  thickening  of  the  intima;  but  it  is  also  possible 
that  the  infective  endaortitis  which  undoubtedly  exists  has  no  direct 
association  with  the  general  process  here  described,  and  that  when, 
after  typhoid  and  other  infections,  there  develops  a  premature  arterio- 
sclerosis, here,  again,  we  deal  with  a  primary  sporadic  degeneration  of 
the  media,  set  up  by  the  bacterial  toxins. 

3.  The  affection  of  the  media  may  be  either  a  primary  degeneration 
without  signs  of  preceding  inflammation,  or  may  be  of  inflammatory 
origin  (as  in  syphilis). 


I,  media  weakened  at  Mf  with  overgrowth 
of  intima  filling  in  the  depression;  II,  with 
postmortem  rigor  and  contraction  of  the 
muscle  of  the  media  and  removal  of  the  blood 
pressure  from  within,  the  stretched  media  at 
M"  contracts,  the  intimal  thickening  thus 
projecting  into  the  arterial  lumen. 


SCLEROSIS  OF  THE  SMALLER  ARTERIES  AND  ARTERIOLES     183 

4.  The  intimal  change  secondary  to  the  medial  degeneration  has  none 
of  the  features  of  an  extension  of  the  morbid  process  from  the  media, 
but  is  of  a  wholly  different  nature.  It  is  primarily  of  hyperplastic  type 
— a  simple  connective-tissue  hyperplasia  unaccompanied  by  the  phe- 
nomena which  we  associate  with  inflammation. 

Sclerosis  of  Arteries  of  the  Second  and  Third  Degree. — These 
same  changes — syphilitic,  senile,  and  ordinary  nodose — affect  also  the 
l>nmches  of  the  aorta  and  their  ramifications,  but  with  this  main  dif- 
ference, that  only  in  the  larger  branches  do  we  encounter  anything 
like  extensive  necrosis  and  atheroma  of  the  intimal  thickening.  These 
thickenings,  compared  with  the  size  of  the  artery,  may  be  extreme,  but 
in  absolute  size  they  do  not  compare  with  what  may  be  found  in  the 
aorta.  The  absence  of  necrosis  is  to  be  ascribed  to  the  fact  that  in  general 
their  size  is  not  such  as  to  inhibit  the  percolation  of  lymph  through  them; 

FIG.  39 


From  a  syphilitic  aorta,  showing  a  moderate  grade  of  medial  degeneration  and  giving  way, 
to  demonstrate  the  simple  connective-tissue  hyperplasia  of  the  intima,  in  regular  layers.  The 
outer  layers  at  a  exhibited  diffuse  fatty  degeneration,  at  c  they  were  more  hyaline.  (From 
Dr.  Klotz's  collection.) 

all  parts  are  able  to  gain  some  nourishment.  Saying  this,  it  must  not 
be  thought  that  these  arteries  do  not  present  calcification;  on  the  contrary, 
that  may  be  extreme.  Long  stretches  of  such  arteries  as  the  radials, 
the  circle  of  Willis  and  its  branches,  the  splenic,  etc.,  may  be  found  con- 
verted into  rigid  tubes.  But  this  deposit  is  in  the  media,  and  at  most 
involves  the  internal  elastic  lamina  and  the  deepest  portion  of  the  thick- 
ened intima.  It  may  be  added  that  it  is  the  smaller  arteries  that  demon- 
strate most  strikingly  the  thinning  and  giving  way  of  the  media  beneath 
the  overlying  great  thickening  of  the  intima. 

Sclerosis  of  the  Smaller  Arteries  and  Arterioles. — There  is  great 
variation  in  the  appearance  of  the  arterioles  in  different  cases,  and, 
indeed,  in  different  organs  from  the  same  case — differences  which,  never- 
theless, we  believe  represent  different  stages  in  the  same  process,  modi- 
fied, it  may  be,  by  variations  in  the  reactive  powers  of  the  different  tissues, 
intimal  and  medial,  to  like  noxse.  In  this  way  two  broad  groups  of  cases 
may  be  distinguished:  (1)  That  in  which  pronounced  thickening  and 


184  ARTERIOSCLEROSIS 

hypertrophy  of  the  muscularis  is  the  most  marked  feature,  and  (2)  that  in 
which  a  generalized  proliferation  of  the  intima  dominates  the  field.  We 
are  inclined,  on  general  principles,  to  believe  that  the  first  represents  the 
earlier  condition;  that  the  first  effect  of  substances  circulating  in  the  blood, 
stimulating  the  smaller  arteries  to  increased  contraction,  must,  of  neces- 
sity, be  to  bring  about  an  hypertrophy  of  the  muscle  cells ;  it  has  already 
been  noted  that  to  this  generalized  contraction  of  the  smaller  arteries  and 
arterioles  must  be  ascribed  the  continued  elevation  of  the  blood  pressure. 

But  just  as  in  the  heart  hypertrophy  beyond  a  certain  point  is  suc- 
ceeded by  incompetency  and  degeneration,  so  here  eventually  the  muscle 
fibers  degenerate  and  fail  to  maintain  the  narrowed  lumen;  and  where 
this  is  a  progressive  process,  and  the  artery  as  a  whole  tends  to  give 
way  under  the  internal  pressure,  there  is  developed  a  compensatory 
fibrosis  and  thickening  of  the  intima,  with  simultaneous  evidence  of 
atrophy  and  fibrosis  of  the  media,  so  that  now  we  encounter  vessels  with 
greatly  increased  intima,  a  media  which  approximates  now,  it  may  be, 
to  the  normal  width,  or  if  thickened  is  fibroid  and  hyaline,  presenting 
a  replacement  fibrosis.  The  indications  are  that  in  some  individuals 
and  tissues  the  muscular  elements  are  incapable  of  pronounced  hyper- 
trophy, and  give  way  at  an  early  stage  in  the  process,  so  that  in  them  the 
intimal  change  is  the  more  pronounced;  in  others  the  muscular  hyper- 
trophy is  exceptionally  well  marked,  the  intimal  change  slight.  This 
pronounced  muscular  hypertrophy,  we  should  add,  is  by  no  means 
confined  to  the  arterioles;  it  is  to  be  observed  in  arteries  of  much  larger 
size,  in  the  radials,  for  example;  and  then,  as  Savill  and  Russel  both 
point  out,  it  may  be  present  either  with  or  without  intimal  thickening. 

What  is  a  characteristic  feature  in  connection  with  the  arterioles  is 
the  very  frequent  surrounding  fibrosis,  or,  as  it  is  termed,  chronic  peri- 
arteritis.  We  know  little  or  nothing  regarding  its  causation — whether 
it  is  irritative,  due  to  seepage  of  irritative  substances  out  of  the  vessels, 
or  whether  it  is  of  compensatory  nature,  or  of  the  same  order  as  the 
intimal  fibrosis.  That  it  is  due  to  malnutrition  from  the  lessened  circu- 
lation, the  fibrous  tissue  replacing  nobler  tissue  elements,  is  scarce 
likely;  the  histology  does  not  suggest  this,  while,  further,  any  such  mal- 
nutrition should  show  itself  at  the  periphery  of  the  capillary  area  sup- 
plied by  a  given  arteriole,  rather  than  at  the  centre.  As  Huchard  has 
pointed  out,  we  occasionally  encounter  examples  of  this  dystrophic 
peripheral  fibrosis.  They  clearly  are  of  another  order. 

In  the  arterioles,  as  in  the  aorta,  we  encounter  a  very  definite  infective 
or  toxic  endarteritis  that  causes  confusion  from  its  similarity  to  certain 
phases  of  arteriosclerosis.  More  particularly  in  connection  with  sec- 
ondary subacute  syphilitic  disturbances,  in  the  neighborhood  of  tuber- 
culous foci,  and,  as  our  former  colleague,  Duval,1  has  shown  in  connection 
with  subacute  glanders,  the  same  is  to  be  met  with.  His  very  full  study 
shows  that  this  is  primarily  a  proliferation  of  the  endothelium  of  the 
arterioles;  the  cells  attain  great  size,  exhibit  mitoses,  and  soon  com- 

1  Journal  of  Exp.  Medicine,  9:1908:241. 


EXPERIMENTAL  ARTERIOSCLEROSIS  185 

pletely  fill  the  lumen.  At  times  they  form  giant  cells.  More  often  their 
overgrowth  results  in  the  production  of  several  layers  of  a  flattened  type 
of  cell.  According  to  him,  degeneration  of  the  media  is  secondary  to 
this  proliferation.  His  figure,  however,  shows  the  familiar  picture  of 
localized  giving  way  of  the  media  with  overlying  intimal  proliferation, 
and  as  he  expressly  notes  that  the  internal  elastic  lamina  at  the  region  of 
this  giving  way  loses  its  plicated,  wavy  appearance  and  becomes  even 
and  without  curves,  the  alternative  explanation  seems  to  us  possible  that 
where  this  occurs,  the  giving  way  of  the  media  is  primary,  the  intimal 
overgrowth  a  secondary  phenomenon.  We  admit,  freely,  that  is,  the 
endothelial  proliferation  due  to  bacterial  toxins;  we  doubt  whether  the 
medial  degeneration  is  truly  secondary  to  this,  believing  it  to  be  equally 
primary,  and  due  to  the  action  of  the  toxins. 

Experimental  Arteriosclerosis. — What,  then,  is  the  exact  meaning 
of  all  these  changes?  The  answer  is  supplied  by  the  abundant  experi- 
ments of  the  last  few  years  upon  artificially  produced  arteriosclerosis. 
There  had  been  many  attempts  to  reproduce  the  condition  by  setting 
up  internal  and  external  injury  to  the  arteries,  and  by  causing  localized 
infection.  None  of  these  were  surely  successful  until  Jores  reported  his 
results  with  adrenalin.  It  is  now  one  of  the  most  familiar  facts  of 
physiology  that  intravenous  injections  of  adrenalin  induce  a  most  pro- 
nounced rise  of  blood  pressure.  As  Langley  has  shown,  these  injections 
reproduce  exactly  the  effects  of  sympathetic  stimulation;  or  otherwise, 
adrenalin  directly  acts  upon  the  muscle  of  the  smaller  arteries  and  causes 
these  to  contract.  The  effect  is  temporary,  but  if  the  injections  be  re- 
peated in  the  rabbit,  eventually  there  is  developed  a  profound  alteration 
in  the  aorta.  There  have  been  doubts  as  to  whether  the  changes  pro- 
duced correspond  accurately  with  those  of  human  arteriosclerosis.  Cer- 
tainly they  do  not  correspond  with  those  of  the  ordinary  nodose  sclerosis. 
They  are,  however,  indistinguishable  from  the  changes  seen  in  Moencke- 
berg's  type  of  medial  degeneration.  There  is  the  same  atrophy  and  giving 
way  of  the  media,  with  the  production  of  pouchings  which  at  times  are 
so  extreme  as  to  become  definite,  small  saccular  aneurisms.  And,  as 
Klotz  has  shown,  what  happens  is  a  fatty,  followed  by  a  calcareous, 
degeneration  of  the  muscular  layers,  with  subsequently  a  similar  cal- 
careous degeneration  of  the  elastic-tissue  elements  of  the  coat.  Identical 
changes  have  been  produced  by  other  observers,  using  barium  chloride, 
nicotine,  and  other  drugs  which  cause  pronounced  rise  of  blood  pressure. 

There  has  been  great  debate  as  to  what  precisely  is  the  action  of  these 
drugs;  do  they  act  directly  as  poisons  of  the  muscular  coat,  or  of  the 
elastica;  do  they  contract  the  vasa  vasorum,  and  so  bring  about  mal- 
nutrition, or  is  the  degeneration  due  not  to  the  drugs  but  to  the  high 
pressure  they  induce  ?  This  last  has  been  shown  to  be  correct  by  Harvey1 
(of  Toronto),  working  in  Professor  Dixon's  laboratory  at  Cambridge, 
and  independently  by  Klotz2  in  our  laboratory.  Harvey  employed 

1  Jour,  of  Med.  Research,  17:1907:25;  Virchow's  Archiv,  196:  1909:  303. 

2  Centralbl.  f.  Allgem.  Pathol.,  19:1908-535. 


186 


ARTERIOSCLEROSIS 


temporary  digital  compression  of  the  abdominal  aorta  of  a  rabbit  for 
many  successive  days;  Klotz,  taking  healthy,  young  rabbits,  suspended 
them  head  downward  for  three  minutes  daily  for  one  hundred  and  twenty 
days  or  more.  In  both  cases  the  only  disturbance  induced  was  rise  of 
blood  pressure  in  the  thoracic  aorta  and  its  branches;  no  drug  was 
introduced ;  but  changes  were  gained  of  the  same  order  as  those  obtained 
with  adrenalin.  Klotz's  results  were  especially  valuable.  The  heart 
was  found  distinctly  hypertrophied ;  the  thoracic  aorta  showed  a  diffuse, 
almost  aneurismal  enlargement  compared  with  the  abdominal  aorta. 
There  was  little  sign  of  intimal  sclerosis,  but  sections  showed  well-marked 
medial  degeneration  of  the  Moenckeberg  type.  But  now  upon  examina- 
tion the  main  vessels  of  the  neck,  which,  if  anything,  had  through  gravity 
experienced  the  daily  rise  of  blood  pressure  to  an  even  greater  degree 
than  the  aorta,  exhibited  most  exquisitely  a  sporadic  intimal  sclerosis 
of  the  nodose  type.  The  condition  was  indistinguishable  from  that 


seen  in  man. 


FIG.  40 


Transverse  section  of  thoracic  aorta  of  rabbit  that  had  been  suspended  by  the  hind  legs  for 
three  minutes  daily  for  130  days:  7,  intirna;  M1 ',  unaffected  inner  layer  of  media;  Mff,  degener- 
ated middle  layer  of  media  with  calcareous  degeneration;  M'n ',  outer  layer  of  media.  The 
portion  of  the  artery  between  A  and  C  has  not  undergone  extreme  distension;  at  C  there  is 
beginning  degeneration  of  the  media;  at  A  and  B,  slight  patches  of  intimal  overgrowth.  (Dr.  Klotz.) 

Let  us  put  these  facts  together.  Raised  blood  pressure  may  induce 
(1)  localized  giving  way  of  the  media,  or  (2)  diffuse  giving  way  of  the 
same  with  no  accompanying  overgrowth,  in  the  first  place  causing  a 
saccular,  in  the  second  a  diffuse  fusiform  aneurism;  or  (3)  it  may  cause 
a  slighter  degeneration  and  giving  way  of  the  media,  which  now  is  accom- 
panied by  pronounced  proliferation  of  the  intima.  How  are  we  to 
reconcile  these  apparently  contradictory  results? 

The  reconciliation  is  simple  and  straightforward  once  we  accept  the 
existence  of  what  one  of  us  has  termed  "  strain  hypertrophy"  (vol.  i,  p.  412) 
and  of  "overstrain  atrophy."  It  is  a  matter  of  common  teaching  that, 
provided  the  nutrition  be  adequate,  muscle  fibers,  whether  striated 
or  plain,  subjected  to  strain  slightly  above  the  normal,  undergo  both 
hypertrophy  and  hyperplasia;  such  moderate  extra  work  is  a  stimulus  to 
increased  growth.  Subjected  to  greater  strain,  they,  on  the  contrary, 
become  exhausted  and  tend  to  atrophy.  Now,  this  same  law  holds  for 


EXPERIMENTAL  ARTERIOSCLEROSIS 


187 


Meet. 


the  tissues  in  general  (vol.  i,  pp.  ~>41  and  FIG.  41 

804).  If  the  media  gives  way  only  slightly 
and  gradually  at  the  region  where  it 
bulges,  the  overlying  endothelium  and 
intima,  being  pressed  outward,  become 
stretched,  subjected,  that  is,  to  increased 
strain;  and  the  strain  not  being  ex- 
cessive, the  cells  proceed  to  multiply 
until  the  concavity  is  filled  up  and  the 
strain  is  removed.  The  explanation  of 
the  difference  in  the  results  in  Dr.  Klotz's 
experiments  between  the  aorta  and  the 
carotids  is  that  the  artery  of  smaller  lumen 
and  relatively  more  powerful  media  can  1  fit 

stand  a  greater  dilating  force  than  the 
artery  of  large  lumen  and  relatively 
weaker  walls.  Regarded  thus,  the  scle- 
rotic thickening  of  the  intima  is  in  no 
sense  an  inflammatory  process,  any  more 
than  is  cardiac  hypertrophy.  At  the 
most,  it  is  compensatory  to  the  weaken- 
ing of  the  media.  When,  on  the  other 
hand,  the  giving  way  of  the  media  is 
more  extreme  and  more  rapid  in  its  pro- 
gress, there  the  strain  to  which  the  intima 
and  endothelium  are  subjected  becomes 
excessive,  and  proliferation  of  the  cells 
is  inhibited,  so  that  aneurism  formation 
takes  the  place  of  compensatory  intimal 
fibrosis  or  sclerosis.  These  views  were 
enunciated  by  one  of  us1  in  1896,  but 

then   gained  little   acceptance;    the   results       creased   strain   brought    to  bear  upon 

of  this  experimental  production  of  arte-     the  °ells  °f  the  int;™./»*.  when.the 

."  .          media  undergoes  a  localized  expansion, 

nosclerosis     have     demonstrated     their 


Schematic  representation  of  the  in- 


through  relative  weakness. 


FIG.  42 


To  show  nodose  sclerosis  of  intima  of  carotid  of  same  rabbit:  A,  outer  layers  of  new  fibrous 
tissue  showing  little  degeneration;  B,  atheromatous  degeneration  of  deeper  layers,  apparently  of 
musculo-elastic  layer;  C,  unaffected  intima. 


Adami,  Middleton  Goldsmith  Lectures,  New  York  Med.  Record,  1896 : 469  and  505 


188  ARTERIOSCLEROSIS 

accuracy.  The  final  and  complete  proof  has  been  afforded  by  Carrel's 
remarkable  observation,1  that  if  a  length  of  vein  be  transplanted  into 
the  course  of  an  artery,  that  vein  in  the  course  of  a  few  months  is  found 
to  present  an  extraordinary  fibroid  hypertrophy.  It  is  unnecessary,  with 
Thoma,  to  invoke  the  difficultly  comprehensible  local  changes  in  rate  of 
blood  flow  and  nutrition  to  account  for  the  intimal  hyperplasia. 

Throughout  the  whole  of  this  arteriosclerotic  process  we  see,  therefore, 
relatively  simple  forces  at  work.  In  one  important  series  of  cases,  direct 
stimuli  acting  upon  the  media  of  the  smaller  arteries  cause  increased  tonic 
contraction,  or,  as  Russel  terms  it,  hypertonus  of  the  same,  and  this,  raising 
the  blood  pressure,  affects  secondarily  the  media  of  the  aorta.  The 
musculature  of  the  media,  becoming  overworked,  undergoes  atrophy  and 
degeneration,  and,  gradually  giving  way,  induces  a  local  compensatory 
fibrosis  of  the  intima.  Similarly,  the  musculature  of  the  smaller  arteries 
giving  way  induces  in  them  also  fibrosis  of  the  intima.  In  the  other 
series  of  cases,  without,  of  necessity,  any  contraction  of  the  peripheral 
arteries  and  rise  of  blood  pressure,  weakness  of  the  media  of  the  aorta 
and  larger  vessels,  whether  inherited  or  acquired,  makes  the  media 
give  way  under  the  normal  blood  pressure,  and  again  the  dilatation  may 
be  followed  by  compensating  fibrosis  of  the  intima,  with  the  successive 
stages  leading  up  to  atheroma.  In  either  series  of  cases,  if  the  giving 
way  be  widely  diffused  or  sudden  and  extreme,  in  place  of  this  com- 
pensatory sclerosis  aneurism  formation  results. 

In  these  cases  of  hyperpiesis  and  hypertonus  of  the  more  peripheral 
arteries  with  the  increase  in  blood  pressure  there  is  also  increased  strain 
thrown  first  upon  the  cusps  of  the  aortic  valve,  and  next  upon  those  of 
the  mitral.  The  arteriosclerotic  thickening  and  fibrosis  of  the  cardiac 
valves  in  these  cases  is  of  exactly  the  same  nature  as  the  intimal 
thickening  of  the  arteries;  the  cusps,  indeed,  are  but  infoldings  of  the 
intima,  or  its  homologue,  the  endocardium.  These  also  afford  examples 
of  strain  hypertrophy  and  fibrosis,  and,  as  already  noted,  are  apt  to 
present  identical  degenerative  processes,  atheroma,  calcification,  and 
atheromatous  ulceration. 

Sclerosis  (Functional)  and  Regeneration  of  the  Uterine  and 
Ovarian  Arteries. — A  remarkable  condition  to  which  attention  was  first 
directed  by  Westphalen2  in  1886,  that  has  come  in  for  renewed  atten- 
tion during  the  last  few  years  by  Pankow,3  Sohma,4  Szasz-Schwarz,5 
Goodall,  and  others,  deserves  mention  here.  The  increased  blood 
supply  to  the  uterus  during  pregnancy  is  accompanied  by  great  dilata- 
tion of  the  uterine  arteries,  so  great  and  so  long  continued,  that  after 
childbirth  they  would  seem  unable  to  contract  to  their  previous  dimen- 
sions. And  now  there  may  be  the  development  of  a  complete  new 
artery  (as  regards  adventitia,  media,  and  intima),  within  the  old,  which 
exhibits  fibrosis,  hyaline  and  other  degenerative  changes.  Failing  this, 

1  Jour,  of  Exp.  Medicine,  10:1908:630.  2  Virch.  Arch.,  106:1886:420. 

3  Arch,  f .  Gynak.,  80 : 1907 :  pt.  2.  4  Ibid.,  84 : 1908 :  pt.  2. 

5  Re"vue  de  Gynec.,  7 : 1903 : 593. 


ANEURISM  189 

there  is  extensive  intimal  overgrowth.  Our  colleague,  Dr.  Goodall,  has 
followed  the  succession  of  changes,  and  concludes  that  there  is  an  inter- 
mediate stage  of  active  proliferation  and  wandering  inward  of  cells  from 
the  various  coats  resulting  in  these  new  cells  assuming  orderly  relation- 
ships with  the  production  of  a  new  arterial  wall. 

The  factors  determining  this  remarkable  process  have  not  been  fully 
worked  out.  It  may  be  suggested  that  with  the  contraction  of  the  uterine 
muscle,  and  it  may  be  the  contraction  also  of  the  terminal  arterioles,  there 
is  pronounced  obstruction  to  the  outflow  of  the  arterial  blood  and  the 
production  of  increased  strain:  that  the  condition  is  identical  with  the 
intimal  thickening  which  Thoma  noted  as  affecting  the  proximal  part  of 
an  artery  after  ligature.  But  this  is  not  sufficient  to  explain  the  devel- 
opment of  a  well-formed  new  artery  within  the  old.  Aschoff1  places  this 
in  a  special  class  as  functional  sclerosis. 

The  process  affecting  the  ovarian  arteries  after  menstruation  and 
ovulation  is  of  the  same  order. 


ANEURISM. 

We  shall,  in  the  next  chapter,  pass  in  review  the  various  forms  of 
aneurism,  or  localized  expansion  of  the  arterial  wall.  We  would,  in 
this  connection,  merely  emphasize  again  that  the  factors  which  are 
productive  of  arteriosclerosis  are  the  same  as  those  producing  aneurism 
— only  that  in  the  case  of  aneurism  we  have  a  severer  disturbance  of  the 
equilibrium  between  the  pressure  within  the  vessels  and  the  strength  of 
its  walls.  The  studies  of  the  last  thirty  years  have  amply  confirmed 
Scarpa's  observation  (1804)  that  the  strength  of  arteries  depends  upon 
the  middle  coat,  and  that  either  localized  degeneration  or  localized 
inflammation  of  the  media,  particularly  syphilitic  mesaortitis,2  is  the 
main  cause  of  the  condition  (Koster,  Eppinger,  Thoma,  Heller,  Chiari, 
Benda).  The  distension  induced  is  so  marked  that  characteristically 
in  the  aneurism  there  is  no  compensatory  hyperplasia  of  the  intima. 
On  the  contrary,  the  strain  thrown  upon  this  and  the  other  coats  is  so 
great  that  the  tendency  is  to  atrophy,  and  with  this  a  gradual  absorption 
of  the  thinned  coats  may  become  followed  by  a  complete  disappearance 
of  the  same  and  the  production  of  a  "false"  aneurism,  the  walls  of 
which  are  formed  of  the  condensed  tissue  of  surrounding  parts  and  organs. 
There  is  but  one  possible  factor  for  the  production  of  aneurisms  which, 
so  far,  has  not  been  recognizable  as  inducing  arteriosclerosis,  namely, 
trauma;  sudden  mechanical  injury  to  the  arterial  coats  is  most  apt  to 
be  followed  by  sudden  giving  way  of  the  vessel,  and  such  sudden  dis- 
tension of  the  intima  certainly  does  not  favor  hypertrophic  changes. 

1  Beihefte  zur  med.  Klinik.,  4:1908:pt.  1. 

2  The  majority  of  statistics  give  from  60  to  85  per  cent,  of  aneurisms  as  of  syphilitic 
origin.     Of  recent  workers,  Hausmann  is  the  only  one  who  depreciates  this  cause, 
ascribing  ouly  18.75  per  cent,  of  cases  thereto. 


CHAPTER    IX. 

PATHOLOGICAL  ANATOMY  AND   HISTOLOGY  OF  THE  ARTERIES, 
VEINS,  AND  LYMPHATIC  VESSELS. 

As  will  be  readily  understood,  the  various  portions  of  the  vascular 
system  stand  in  such  close  functional  and  anatomical  relationship  to  each 
other  that  the  pathological  processes  involving  them,  while  possessing 
some  few  characteristics  and  peculiarities,  on  the  whole  present  much 
similarity. 

ARTERIES. 

The  walls  of  the  arteries  are  composed  of  three  layers,  the  tunica 
intima,  the  tunica  media,  and  the  tunica  adventitia.  The  first  is  avascu- 
lar,  deriving  its  nourishment  from  the  circulating  blood  within  the 
vessel,  while  the  adventitia  derives  its  blood  supply  from  small  arterial 
twigs,  the  vasa  vasorum.  In  the  case  of  the  media  the  condition  varies 
in  different  places,  the  media  of  the  aorta  having  vascular  twigs  which 
reach  to  the  intima.  The  intima  is  an  endothelial  lining  directly  con- 
tinuous with  the  endocardium  and  the  wall  of  the  finest  capillaries. 

The  thickness  of  the  arterial  walls  and  the  caliber  of  the  vessels 
varies  at  different  periods  of  life  and  with  different  individuals.  Accord- 
ing to  Orth,  the  thickness  of  the  aortic  wall  from  the  age  of  twenty-five 
to  seventy-five  is,  on  the  average,  1.5  to  2  mm.  The  circumference  of 
the  aorta  just  above  the  valves  is  6.1  to  8.3  cm.;  of  the  thoracic  aorta,  4.4 
to  5.95cm.;  of  the  abdominal  aorta,  3.2  to  4.33  cm.  The  circumference 
of  the  pulmonary  artery  just  above  the  valves  is  from  6.4  to  7.5  cm. 
In  early  life  the  pulmonary  artery  is  somewhat  larger  than  the  aorta;  in 
middle  life  they  are  the  same  size;  and  in  old  age  the  aorta  is  the  larger. 
This  last  condition  is  due  to  the  fact  that  degenerative  processes  in  the 
aorta  are  so  common  after  middle  life. 


CONGENITAL  AND  DEVELOPMENTAL  ANOMALIES. 

Defects  of  development  have  a  close  relationship  to  those  of  the 
heart,  and  have  already  been  touched  upon.  Abnormalities  in  the  course 
or  number  of  the  arteries  have  no  pathological  interest. 

The  aorta  may  participate  in  the  condition  of  transposition  of  the 
viscera,  or  may  be  duplicated,  either  in  whole  or  in  part. 

More  important  is  general  hypoplasia  of  the  arterial  system,  which 
may  exist  alone  or  in  combination  with  a  similar  defect  in  the  heart. 
The  condition  is  found  in  both  sexes,  but  is  most  common  in  chlorotic 
girls  about  the  age  of  puberty.  We  have  found  it  with  striking  frequency 


INFLAMMATIONS  191 

in  young  people  who  have  died  of  tuberculosis.  The  aorta  is  narrowed 
and  the  circumference  may  be  only  2  cm.  At  the  same  time  the  wall  is 
thinned  and  the  elasticity  is  decreased.  Other  physical  defects  may  be 
associated,  particularly  hypoplasia  of  the  genital  system.  Arterial  hypo- 
plasia  has  also  been  met  with  in  cases  of  hemophilia. 


CIRCULATORY  DISTURBANCES. 

Blood  Imbibition. — Owing  to  the  avascular  character  of  the  intima, 
circulatory  disturbances  in  this  portion  do  not  occur.  At  most,  we  may 
find  a  diffuse  rosiness  due  to  blood  imbibition,  which  is  most  likely 
a  postmortem  change.  This  is  found  in  septicemia,  passive  congestion, 
and  infection  with  the  B.  Welchii. 

In  the  adventitia  and  media,  and  even  in  the  intima,  where  newly 
formed  vessels  have  invaded  this  coat,  small  hemorrhages  are  found  in 
passive  congestion  and  in  inflammation.  Of  special  interest  to  the 
medicolegal  expert  are  hemorrhages  into  the  wall  of  the  carotid  in  those 
who  have  been  hanged  or  throttled. 


INFLAMMATIONS. 

Arteritis. — Arteritis  may  be  hematogenic,  or  may  arise  from  trauma, 
from  direct  extension  of  inflammatory  processes,  or  as  a  complication 
of  degenerative  changes.  Traumatic  causes  are,  rupture,  wounds,  or 
ligature  of  a  vessel.  Apart  from  injury,  the  most  important  factors  are 
infections  and  intoxications,  due  to  pus  organisms,  tubercle  bacilli,  and 
the  syphilitic  virus.  A  very  common  occurrence  is  the  inflammation 
of  a  vessel  from  the  presence  of  a  thrombus,  either  infective  or  simple. 

Thrombo-arteritis. — Thrombo-arteritis  is  the  form  associated  with  the 
presence  of  an  autochthonous  thrombus  or  an  embolus.  Examples  of 
the  former  are  found  in  traumatism  to  the  vessel  wall  and  in  the  infections. 
As  a  rule,  inflammation  of  the  artery  is  primary  and  the  thrombus  is 
secondary,  but  undoubtedly  the  reverse  can  occur.  The  character  of 
the  inflammation,  whether  suppurative  or  proliferative,  depends  upon 
the  nature  of  the  obstruction. 

Suppuration  is  the  result  when  the  thrombus  or  embolus  contains 
pus  organisms.  The  affected  spot  is  of  a  yellowish-white  color,  swollen, 
and  more  friable  than  usual.  The  intima  is  first  swollen,  and  later  there 
is  rapid  infiltration  of  all  the  coats  from  within  outward,  with  inflam- 
matory aneurism  or  a  local  abscess. 

Thrombo-arteritis  proliferans  occurs  when  the  thrombus  or  embolus  is 
not  infective.  According  to  the  degree  of  proliferation,  localized  patches 
of  thickening  or  thread-like  projections  are  formed  upon  the  vessel 
wall.  If  the  process  be  extensive  enough  to  obliterate  the  artery,  we 
can  speak  of  an  endarteritis  obliterans. 

The  proliferative  change  consists  in  the  substitution  of  the  thrombus 
by  connective  tissue.  The  arterial  wall  is  infiltrated  with  leukocytes, 


192  THE  ARTERIES,   VEINS,   AND  LYMPHATIC  VESSELS 

and  many  fibroblasts  can  be  seen,  which  invade  the  intima  and  penetrate 
the  substance  of  the  thrombus,  eventually  bringing  about  complete 
organization.  Newly-formed  capillaries  can  be  made  out  within  the 
fibrous  mass.  If  the  intima  be  preserved,  it  may  also  show  changes 
of  a  proliferative  character.  The  thrombus  may  thus  be  converted  into 
a  solid  mass  of  tissue  or  may  be  tunnelled  through  (canalization),  the 
various  channels  being  ultimately  lined  with  endothelium  and  the  blood 
flow  thus  restored  (see  p.  74).  In  some  cases  a  calcareous  deposit  takes 
place,  and  an  arteriolith  is  the  result. 

Arteritis  also  arises  by  the  extension  to  the  vessel  of  a  neighboring 
inflammatory  process,  such  as  an  abscess,  ulceration,  infected  wounds, 
tuberculous  cavities,  and  the  like.  In  these  cases  the  inflammation 
begins  first  in  the  adventitia,  and  subsequently  invades  the  other  coats. 
It  may  lead  to  thrombosis  of  the  vessel  or  to  rupture. 

Proliferative  arteritis,  leading  to  gradual  thickening  of  the  vessel  and 
even  to  obliteration,  is  also  found  in  the  arteries  of  a  tissue  which  is 
chronically  inflamed.  It  is  well  seen  in  cases  of  chronic  interstitial 
nephritis,  tuberculosis,  and  syphilis,  and  is  very  common  in  almost 
every  form  of  tumor,  as  in  soft  sarcomata  and  carcinomata,  but  par- 
ticularly often  in  those  forms  which  contain  much  connective  tissue, 
such  as  elephantiasis,  fibroma,  and  scirrhous  carcinoma. 

Periarteritis  Nodosa. — This  is  a  curious  condition,  first  fully  described 
by  Kussmaul  and  Maier,1  in  which  small  nodules  are  formed  in  the 
walls  of  the  smaller  arteries  of  the  muscles,  serous  membranes,  spleen, 
abdominal  glands,  uterus,  and  mucous  glands.  According  to  Freund, 
who  has  published  a  careful  research,  the  changes  are  sometimes  most 
marked  in  the  adventitia,  sometimes  in  the  intima,  but  usually  the 
process  affects  chiefly  the  media. 

The  adventitia  shows  cellular  infiltration,  chiefly  of  the  mononuclear 
variety,  together  with  spindle  cells.  The  intima  is  often  thickened  and 
hyaline.  The  hyaline  change  also  extends  into  the  media.  In  the 
earlier  stages  round  cells  from  the  adventitia  penetrate  the  media. 
From  this  description  of  the  lesion  it  will  be  seen  that  the  term  "peri- 
arteritis  is  not  strictly  applicable.  The  character  and  the  multiplicity 
of  the  affection  render  it  probable  that  infective  agents  or  circulating 
toxins  are  the  cause  of  the  condition  (H.  Morley  Fletcher2).  Thrombosis 
and  acute  aneurismal  dilatation  often  accompany  the  process. 

Tuberculous  Arteritis. — Tuberculous  arteritis  may  arise  from  infection 
through  the  blood  or  from  the  extension  of  a  tuberculous  process  outside 
the  vessel.  The  latter  event  is  the  more  common.  In  the  arterial  wall 
typical  tubercles  can  be  seen,  or  a  more  diffuse,  inflammatory  infiltration. 
The  tubercles  sometimes  caseate,  and  rupture  of  the  vessel  takes  place; 
the  vessel  may  become  thrombosed,  or  the  infective  substance  may  be 
discharged  into  the  lumen.  Under  favorable  conditions,  fibrous  hyper- 
plasia  takes  place.  Most  frequently  the  adventitia  is  affected;  but  the 

1  Deutsch.  Arch.  f.  klin.  Med.,  1:  1866:484. 

2  Ueber  die  sogenannte  Periarteritis  nodosa,  Ziegler's  Beitrage,  11:1891:323. 


RETROGRESSIVE  METAMORPHOSES  193 

intima  may  be  considerably  thickened  as  well.  In  the  case  of  the  smaller 
vessels  the  lumen  may  be  entirely  occluded.  Tuberculosis  of  the  large 
arteries  is  rare.  Blumer1  has  recorded  two  cases. 

Syphilitic  Arteritis. — Syphilitic  arteritis  occurs  as  a  distinct  entity 
or  as  an  extension  of  a  local  syphilitic  infection.  The  arteries  of  the 
brain  and  heart,  and  the  aorta  are  the  vessels  chiefly  affected.  In  the 
first  form  a  thickening  of  the  intima  and  adventitia  occurs  either  as  cir- 
cumscribed gray  or  grayish- white,  semitranslucent  masses  (gummata),  or 
the  section  of  a  vessel  may  be  transformed  into  a  firm,  grayish-white 
cord.  Or,  again,  the  vessel  wall  is  infiltrated  with  gummatous  masses  or 
is  enclosed  in  dense,  fibrous  tissue.  In  the  aorta  the  disease  affects  pri- 
marily the  adventitia  and  media  with  secondary  fibrosis  of  the  intima 
(see  p.  178).  Reuter,2  Wright,  and  others  have  detected  the  treponema 
pallidum  in  cases  of  specific  aortitis. 

RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  arterial  system  occurs  in  general  maras- 
mus and  severe  anemias,  or  a  particular  organ  may  be  affected.  Stenosis 
of  the  aortic  ring  and  extreme  atrophy  of  the  heart  may  also  result  in 
this  condition.  In  amputated  limbs  the  vessels  of  the  stump  become 
smaller.  Increased  blood  pressure  leads  to  atrophy  of  the  tunica  media 

Degeneration. — Fatty  Degeneration. — This  is  a  very  common  con 
dition  found  at  autopsy,  and  while  it  usually  affects  the  intima,  is  also 
met  with  occasionally  in  the  media  and  adventitia.  It  is  due  to  anemia, 
to  circulatory  toxins,  or  again  to  increased  blood  pressure.  The  last  form 
is  often  seen  in  the  pulmonary  artery,  for  instance,  in  pulmonary  tuber- 
culosis, and  in  prolonged  congestion  of  the  lesser  circulation.  The  toxic 
form  is  well  illustrated  in  the  case  of  typhoid  fever  and  pulmonary 
tuberculosis.  The  fatty  patches  appear  in  small  streaks  or  flecks  of 
a  whitish  or  yellowish-white  color,  which  may  or  may  not  be  slightly 
elevated  above  the  general  surface.  Microscopically,  the  cells  of  the 
deeper  layers  of  the  intima  are  filled  with  fat  droplets.  The  condition 
can  result  in  a  small  local  loss  of  substance,  which,  in  the  capillaries  of 
the  brain  and  lungs,  may  even  lead  to  rupture  of  the  vessel. 

In  the  larger  vessels  a  locus  resistentice  minoris  may  be  thus  produced, 
which  may  very  possibly  prepare  the  way  for  sclerotic  changes. 

Necrosis  and  Calcification. — These  are  also  frequent  sequels.  The 
media  is  the  chief  seat  of  calcareous  deposit,  and  the  intima  may  at  the 
same  time  exhibit  productive  change.  The  thickened  vessels  of  the 
aged  are  no  doubt  of  this  type.  Such  arteries  are  a  frequent  cause  of 
thrombosis  and  anemic  necrosis,  as  well  as  glandular  atrophies. 

Hyaline  Degeneration. — Hyaline  degeneration  affects  chiefly  the  intima 
of  the  larger  vessels  or  the  wall  of  aneurismal  sacs,  but  is  frequently 
found  in  the  finest  capillaries.  It  is  a  common  accompaniment  of  arterio- 
sclerotic  change.  It  is  often  present  in  the  glomerular  tufts  of  the  kidney 
in  chronic  nephritis,  in  the  choroid  plexus  of  the  brain,  and  in  the 

1  Amer.  Jour.  Med.  Sci.,  117:1899:19.          2  Zeitschr.  f.  Hygiene,  54:1906:49. 
13 


194          THE  ARTERIES,   VEINS,   AND  LYMPHATIC   VESSELS 

capillaries  of  atrophic  lymph-glands.  The  change  consists  in  the 
formation  of  a  homogeneous  hyaline  substance,  resembling  amyloid, 
but  not  giving  the  same  chemical  reactions,  in  the  cell  protoplasm.  It 
is  due  to  mechanical,  chemical,  or  dyscrasic  causes.  In  certain  tumors, 
hence  called  "cylindromata,"  the  vessels  are  found  converted  into 
thick  tubes  of  this  material. 

Amyloid  Infiltration. — Amyloid  infiltration  attacks  by  preference  the 
smaller  arterioles  of  the  various  organs,  though  in  very  severe  cases  of 
the  affection  the  large  trunks  do  not  altogether  escape.  The  deposit 
is  seen  first  in  the  media,  following  the  course  of  the  circular  muscle 
fibers.  The  condition  may  spread  to  the  adventitia  and,  in  large  vessels, 
to  the  intima. 

Arteriosclerosis. — Arteriosclerosis  is  an  affection  of  the  arteries  most 
frequently  found  in  the  aorta,  but  often  also  in  the  arteries  of  the 
brain,  heart,  extremities,  the  kidneys,  and  the  spleen.  It  is  somewhat 
rare  in  the  mesenteric  and  pulmonary  arteries.  The  lesions  vary  much 
in  their  distribution;  at  one  time  the  major  trunks,  at  another  the  medium- 
sized  vessels,  or,  again,  the  finer  arterioles  show  the  most  advanced 
changes.  Even  the  capillaries  may  be  involved  in  a  widespread 
process,  the  arteriocapillary  fibrosis  of  Gull  and  Sutton  (angio- 
sclerosis  of  Thoma).  If  the  lesions  are  extensive  and  widespread,  we 
recognize  an  arteriosclerosis  diffusa;  if  scattered  and  localized,  an  arterio- 
sclerosis nodosa.  According  to  Rokitansky,  the  following  is  the  order  of 
frequency  with  which  the  vessels  are  affected:  Ascending  aorta,  the 
arch,  the  thoracic  aorta,  the  abdominal  aorta,  iliacs,  crurals,  coronaries  of 
the  heart,  cerebrals,  vertebrals,  uterine  arteries,  spermatics,  hypogastrics. 

At  an  early  stage  of  the  process,  gray,  semitranslucent  patches,  some- 
times of  a  gelatinous  appearance,  are  found  in  the  intima  (plaques 
gelatiniformes).  These  have,  in  part,  the  structure  of  mucoid  tissue, 
the  cells  of  which  are  either  well  preserved  or  more  often  fattily  degener- 
ated or  necrosed.  Later,  the  patches  are  harder,  of  a  cartilaginous 
appearance  and  gray-white  color,  forming  round,  oval,  or  irregular 
areas  more  or  less  raised  above  the  general  surface.  These  are  composed 
of  newly-formed  connective  tissue  which  already  begins  to  show  retro- 
gressive changes.  The  tissue  has  lost  its  stratification,  the  cells  are 
swollen  and  stain  poorly,  the  whole  eventually  forming  a  structureless, 
hyaline  mass.  Associated  with  this  may  be  fatty  degeneration  of  the 
cells  and  the  production  of  a  granular,  shreddy  detritus  (atheroma) .  A  true 
necrosis  is  thus  the  result,  and  in  the  advanced  stages  we  frequently 
find  a  deposit  of  lime  salts  in  the  areas  of  degeneration  (calcified  plaques). 
Very  frequently  the  necrobiotic  tissue  breaks  down  into  a  shallow 
ulcer  (atheromatous  ulcer),  the  base  of  which  is  formed  of  cholesterin 
and  granular  debris.  On  such  ulcers  thrombi  may  form,  although 
more  often  they  are  characteristically  not  produced.  If  the  destructive* 
process  goes  on  in  the  deeper  layers,  leaving  the  necrotic  area  still  covered 
by  the  thickened  intima  and  endothelium,  nn  abscess-like  cavity  is  the 
result.  The  degenerative  changes  just  described  are  by  no  means  re- 
stricted to  the  intima,  but  may  extend  quite  deeply  into  the  media.  The 


ARTERIOSCLEROSIS  195 

muscle  fibers  are  atrophic  and  show  hyaline,  fatty,  and  calcareous  change. 
The  elastic  fibrillre  are  usually  degenerated,  and  often  torn.  New 
formation  of  elastic  tissue  takes  place,  especially  in  the  intima.  In 
addition  to  the  appearances  mentioned  in  the  class  of  case  just  described, 
there  are  others  of  an  inflammatory  nature  which  have  led  many  patholo- 
gists  to  regard  the  whole  process  as  inflammatory.  Both  in  the  media 
and  the  adventitia  small  collections  of  leukocytes  are  found,  situated 
around  the  vasa  vasorum,  which  also  seem  to  proliferate,  for  we  find 
newly-formed  capillaries  developing  in  the  media  and  even  pushing  their 
way  into  the  intima,  indications  of  a  reparative  process.  Where  this  is 
notably  the  case  we  deal  with  syphilitic  mesaortitis  (see  p.  178).  With  the 
vessels  a  certain  number  of  fibroblasts  are  carried  in  which  go  to  form 
scar  tissue,  thus  assisting  repair.  When  the  vessels  come  in  contact  with 
calcareous  deposits  these  may  be  absorbed,  and  in  the  aorta,  at  least, 
a  formation  of  bone  may  take  place.  All  stages  of  the  affection  are 
found  in  the  vessels  at  the  same  time. 

The  recent  studies  upon  experimental  arteriosclerosis  have  demon- 
strated that  at  least  three  forms  of  sclerotic  disease  of  the  arteries  must 
be  recognized:  (1)  Monckeberg's  type  of  medial  degeneration  followed 
by  medial  calcification;  this  is  the  form  present  in  the  radials  of 
clinical  arteriosclerosis,  and  may  be  reproduced  by  adrenalin  injections. 
(2)  Productive  endarteritis ;  this  may  be  reproduced  experimentally, 
even  in  the  aorta,  by  injections  of  pyococcus  toxins  (Klotz1  and 
Saltykow2) ;  and  (3)  inflammatory  periarteritis  extending  into  the  media 
by  injury  to  the  outer  walls.  The  syphilitic  virus,  it  may  be  noted, 
more  particularly  invades  the  arteries  through  the  vasa.  The  relation- 
ship of  the  common  nodose  type  of  arteriosclerosis  of  the  aorta  to 
these,  whether  in  the  main  compensatory  to  medial  giving  way,  or 
productive,  is  still  a  matter  of  dispute. 

As  would  naturally  be  expected,  such  grave  disturbances  bring  in 
their  train  further  secondary  manifestations.  The  larger  vessels  become 
elongated  and  dilated  in  whole  or  in  part.  In  the  smaller  vessels,  such  as 
those  of  the  brain  and  heart,  owing  to  the  thickening  of  the  intima,  the 
lumen  is  greatly  obstructed  or  even  obliterated.  Such  vessels  on  cross- 
section  show  a  characteristic  signet-ring  appearance. 

According  to  Thoma,  the  thickening  of  the  intima  is  compensatory 
to  the  atrophy  and  weakening  of  the  media,  but,  unlike  Thoma,  we  do 
not  regard  this  as  of  an  inflammatory  nature  (see  p.  180). 

Among  the  consequences  of  arteriosclerosis  may  be  mentioned,  throm- 
bosis, embolism,  rupture  of  the  vessel  wall,  aneurism,  necrosis  from 
ischemia,  contracted  kidney,  and  enlargement  of  the  heart. 

Aneurisms. — As  to  what  constitutes  an  aneurism  authorities  differ. 
The  subject  is  still  further  confused  by  a  multiplicity  of  terms.  It  is 
perhaps  simplest  to  define  "aneurism,"  with  Orth,  as  any  circumscribed 
dilatation  of  the  lumen  of  an  artery. 

If  the  aneurismal  sac  be  constituted  of  all  or  any  of  the  coats  of  the 
arterial  wall,  it  is  called  a  "true"  aneurism.  If,  on  the  other  hand, 

1  British  Med.  Jour.,  2:  1906:  1767. 

»  Saltykow,  Cent,  f.  Path,  Anat.,  19:  1808;  321,    Ziegler's  Beitrage,  42;  1908:  147, 


196  THE  ARTERIES,  VEINS,  AND  LYMPHATIC  VESSELS 

a  portion  of  the  sac  be  composed  of  the  surrounding  tissues  or  a  newly- 
formed  fibrous  investment,  we  speak  of  a  "false"  aneurism.  In  ad- 
vanced cases,  however,  it  may  not  be  possible  to  draw  this  distinction. 
In  the  immense  majority  of  cases  aneurisms  are  due  to  the  action  of 
a  normal  or  increased  pressure  of  the  blood  upon  an  arterial  wall  weak- 
ened from  disease;  syphilis  is  the  most  potent  cause;  tobacco,  alco- 
holism (?),  gout,  and  lead  poisoning  are  also  effective  in  some  instances. 
Some  cases  are  due  to  muscular  strain,  direct  injury,  inflammatory 
processes  in  the  vessel,  or  rarely  to  defective  development  of  the  arteries. 
The  following  classification  is  offered  as  a  convenient  and  comprehensive 
one: 

I.  Aneurism  from  dilatation, 
(a)  Arteriectasis. 
(6)  Cirsoid  or  racemose  aneurism. 

(c)  Serpentine  aneurism. 

(d)  Cylindrical  aneurism. 

(e)  Fusiform  aneurism. 
(/)  Sacculated  aneurism. 

II.  Aneurisms  from  rupture, 
(a)  Dissecting  aneurism. 
(6)  Sacculated  aneurism. 

(c)  Anastomotic  aneurism    I  Varicose  aneurism. 

( Aneunsmal  vanx. 
III.  Aneurism  from  external  erosion. 
IV.  Aneurism  from  embolism. 
'  (a)  Tearing  of  the  intima. 
(6)  Mycotic. 
V.  Traumatic  aneurism. 
VI.  Aneurism  from  traction.1 

1  Osier,  in  his  Modern  Medicine  (vol.  4,  pp.  450,  451,  1908),  gives  the  following 
as  a  useful  classification  for  practical  purposes: 

1.  True  aneurism  (A.  verum,  A.  spontaneum),  in  which  one  or  more  of  the  coats 

of  the  artery  form  the  walls  of  the  tumor, 
(a)  Dilatation  aneurism. 

1.  Limited  to  a  certain  section  of  a  vessel — fusiform  aneurism,  cylin- 

droid  aneurism. 

2.  Extending  over  a  whole  artery  and  its  branches — cirsoid  aneurism. 
(6)  Circumscribed  saccular  aneurism — the  more  common  form  in  the  aorta, 

in  which  there  is  distension  of  two  or  more  of  the  coats,  or  distension  of 
the  adventitia  after  destruction  of  the  intima  and  media, 
(c)  Dissecting  aneurism,  with  splitting  of  the  coats  to  a  greater  or  less  extent 
and  occasionally  with  the  formation  of  a  new  tube  lined  with  intimal 
endothelium. 

2.  False  aneurism,  following  wound  or  rupture  of  an  artery,  causing  a  diffuse 

or  circumscribed  hematoma. 

3.  Arteriovenous  aneurism — communication  between  artery  and  vein,  either  direct 

— aneurismal  varix — or  with  the  intervention  of  a  sac — varicose  aneurism. 

4.  Special  forms,  such  as  the  traction  aneurism,  the  erosion  and  parasitic  forms/ 

which  have  a  pathological  rather  than  a  clinical  interest, 


ANEURISMS  197 

The  forms  in  the  first  group  are  differentiated  according  to  the  shape 
which  the  dilatation  takes. 

Arteriectasis  is  a  tubular,  spindle-formed,  or  nodular  dilatation  of 
an  artery  affecting  a  more  or  less  considerable  extent  of  the  vascular 
tree.  Such  is  commonest  in  the  aorta  or  some  portion  of  it,  as  the 
thoracic  aorta  and  the  arch. 

FIG.  43 


Sacculated  aneurism  of  the  ascending  and  transverse  arch  of  the  aorta.     (From  the  Patho- 
logical Laboratory  of  Royal  Victor  Hospital.) 


When,  in  addition  to  the  dilatation  of  the  vessels,  there  is  great  tortu- 
osity with  free  anastomosis,  we  speak  of  cirsoid  aneurism.  These  are 
found  in  the  large  vessels  of  the  pelvis,  and  on  the  scalp.  Some  of  them 
should  possibly  be  classified  with  the  angiomas.  A  sub-variety  is  the 
serpentine,  characterized  as  its  name  implies. 

Cylindrical  and  fusiform  aneurisms  are  found  commonly  in  the  thoracic 
aorta  and  in  the  great  vessels  springing  from  the  arch. 


198          THE  ARTERIES,  VEINS,  AND  LYMPHATIC  VESSELS 


FIG.  44 


A  very  important  form  is  the  sacculated,  in  which,  springing  from  the 
side  of  the  affected  vessel,  is  a  saccular  diverticulum,  often  of  large  size, 
and  communicating  with  the  lumen  of  the  vessel  by  a  comparatively 
narrow  opening.  Such  are  the  aneurisms  which  especially  give  rise  to 

pressure  symptoms,  erosion,  rupture,  and 
the  like. 

Sometimes  the  various  forms  may  be 
combined.  An  aneurismal  sac  usu- 
ally shows  some  variation  in  thickness  in 
its  various  parts,  inasmuch  as  the  wall 
is  generally  extensively  diseased;  some 
parts  showing  advanced  atheromatous 
degeneration,  while  in  others  the  wall 
of  the  sac  may  be  quite  thin,  one  or 
more  coats  being  absent.  Within  the 
sac  one  often  sees  local  deposits  of  fibrin 
or  possibly  organized  clot  adherent  to 
the  atheromatous  plaques.  Any  con- 
siderable amount  of  clot  or  reparative 
change  is  distinctly  rare;  in  one  speci- 
men, however,  in  the  museum  of  McGill 
University,  where  complete  cure  took 
place,  the  sac  was  quite  obliterated  by 
the  organization  of  clot. 

As  will  be  readily  understood,  in  a 
diseased  and  weakened  arterial  wall  rup- 
ture very  readily  takes  place. 

If  the  rupture  be  through  the  intima 
into  the  media,  the  blood  finds  its  way 
along  the  vessel  between  the  layers  of 
media,  and  a  dissecting  aneurism  is  the 
result.  These  are  found  frequently  in 
the  aorta  and  the  vessels  of  the  brain. 
Usually  swiftly  fatal,  they  have  been 
known  to  undergo  repair.1  The  exciting 
cause  is  usually  some  strain  or  injury. 
If  all  the  coats  be  ruptured,  there  is 
naturally  hemorrhage  about  the  vessel, 
with  the  formation  of  a  hematoma,  or 
hemorrhage  into  some  cavity.  In  some 
cases,  where  the  intima  and  media  are 

torn  through,  a  local  or  sacculated  dilatation  is  formed,  owing  to  the 
distension  of  the  weak  adventitia.  Such  may  be  quite  large,  or,  again, 
small  and  multiple  in  distribution.  They  sometimes  heal  up,  leaving 
few  traces. 


Dissecting  aneurism  of  the  aorta. 
(From  the  Pathological  Museum  of 
McGill  University.) 


1  Adami,  On  Arrested  or  Kepaired  Dissecting  Aneurisms,  Montreal  Med.  Jour., 
24: 1895-96;  945,  and  25:1896:23. 


ANEURISMS 


199 


In  the  outer  wall  of  the  sac  secondary  inflammatory  change  is  common, 
leading  to  a  development  of  fibrous  tissue. 

When  rupture  takes  place  into  a  vein,  in  the  cases  where  the  artery 
and  vein  have  become  closely  united,  we  get  an  aneurismal  varix  pro- 
duced. In  other  instances  a  false  aneurism  is  the  result,  which  later  breaks 
into  a  vein,  so  that  there  is  an  indirect  communication  between  vein  and 
artery.  This  is  a  varicose  aneurism. 

Aneurisms  from  erosion  are  found  chiefly  in  suppurating  wounds  and 
in  tuberculous  cavities.  They  are  due  to  the  extension  of  the  necrotic 
process  to  the  wall  of  the  vessel,  thus  rendering  it  weak  and  unsup- 
ported. In  tuberculous  cavities  one  frequently  sees  the  vessels  stretch- 


FIG.  45 


Aneurism  of  the  aorta.  Here  the  sternum  and  costal  cartilages  were  extensively  eroded,  a 
large  false  aneurism  pulsating  under  the  skin  and  eventually  undergoing  external  rupture.  (From 
the  Medical  Clinic  of  the  Royal  Victoria  Hospital,  under  the  late  Dr.  J.  Stewart.) 

ing  across  with  small  aneurismal  dilatations  upon  them  (see  Fig.  75). 
The  hemorrhage  so  common  in  cases  of  ulcerative  tuberculosis  of  the 
lungs  is  frequently  due  to  the  rupture  of  one  of  these  minute  aneu- 
risms. In  most  cases,  however,  the  vessel  is  thrombosed  and  the 
lumen  obliterated  before  the  tubercle  begins  to  soften. 

Embolic  Aneurisms  are  of  two  forms.  In  the  first,  sharp  calcareous 
particles  break  loose  from  an  ulcerated  valve  of  the  heart  or  an  athero- 


200  THE  ARTERIES,  VEINS,  AND  LYMPHATIC  VESSELS 

matous  patch,  and  are  carried  along  in  the  blood  stream  to  some  small 
vessel,  where  they  tear  the  intima,  producing  hemorrhage  or  an  aneurism 
from  rupture.  In  the  second  form,  the  mycotic,  infective  emboli  set 
up  degeneration  and  inflammation  of  the  arterial  wall  and  thus  bring 
about  weakening  and  rupture.  Osier  has  drawn  attention  to  multiple 
aneurisms  of  this  type  occurring  in  ulcerative  endocarditis.  J.  McCrae 
has  also  recorded  a  case.1  In  the  horse  similar  aneurisms  are  pro- 
duced by  parasites,  such  as  the  Strongylus  armatus. 

Traumatic  aneurisms  are  formed  by  the  rupture  of  one  or  all  of  the 
arterial  coats  due  to  external  violence,  particularly  penetrating  wounds. 
Many  of  the  false  aneurisms,  as  well  as  the  anastomotic,  come  under  this 
head. 

The  commonest  sites  for  aneurisms  are  the  aorta,  chiefly  the  arch, 
the  abdominal  aorta,  the  popliteal,  femoral,  subclavian,  carotid,  innomi- 
nate, and  iliac  arteries,  in  the  order  named.  Aneurisms  are  not  uncom- 
monly multiple  in  distribution.  Multiple  miliary  aneurisms  are  often 
found  in  the  arteries  of  the  brain,  particularly  those  supplying  the  lenticulo- 
striate  region,  and  are  a  fertile  cause  of  cerebral  hemorrhage.  Aneurisms 
of  the  aorta,  according  to  their  size  and  position,  produce  a  variety  of 
effects.  If  large,  they  dislocate  various  organs  and  produce  collapse  of 
the  lung,  necrosis,  or  form  adhesions.  Aneurisms  of  the  middle  part  of 
the  arch  press  upon  the  left  recurrent  laryngeal  nerve  and  the  oesophagus. 
The  ribs  or  the  vertebral  column  may  be  eroded  and  the  vertebral  canal 
may  be  opened.  Rupture  can  take  place  into  a  right  auricle  of  the 
heart,  as  in  a  case  recorded  by  McPhedran,2  the  bronchi,  the  oesophagus, 
the  trachea,  the  pleural  cavity,  or  externally. 


PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — The  cause  of  general  arterial  hypertrophy  is  in- 
creased function — due  to  excessive  intravascular  pressure,  overwork, 
or  nervous  influences. 

True  hypertrophy  is  found  in  certain  organs  or  portions  of  an  organ 
which  are  the  sites  of  compensatory  hypertrophy.  The  enlargement 
affects  both  the  thickness  and  the  length  of  the  vessel,  so  that  it  frequently 
becomes  tortuous.  All  coats  may  be  affected,  but  particularly  the  media, 
the  latter  condition  being  marked  in  that  form  of  arterial  hypertrophy 
found  in  chronic  Bright's  disease. 

Cirsoid  aneurism,  or  the  angioma  arteriale,  is  by  some  classed  under 
this  head. 

Tumors. — Primary  tumors  of  the  arteries  are  very  rare.  Brodowski 
has  recorded  a  case  of  primary  sarcoma  of  the  thoracic  aorta.  A  very 
interesting  form  of  tumor  is  the  perithelioma,  which  may  be  either  benign 
or  malignant.  This  is  found  in  the  suprarenals,  the  prostate,  the  thy- 

1  J.  McCrae,  Jour.  Path,  and  Bact.,  10: 1905:373. 

2  Canadian  Practitioner,  21:  1896:  578. 


VARICOSITY  OF  THE  VEINS  201 

roid,  and  in  the  brain.  It  is  a  vascular  tumor,  really  a  spindle-celled 
sarcoma,  which  originates  in  the  perithelium  of  the  vessels. 

Myomas  are  also  said  to  occur. 

Secondary  tumors  arising  by  direct  extension  or  from  embolism  are 
not  uncommon. 

THE  CAPILLARIES. 

Thrombosis  of  the  capillaries  is  rare,  but  embolism  is  not  uncommon. 

Capillary  aneurisms  are  met  with  frequently,  due  to  active  or  passive 
congestion.  These  are  seen  particularly  well  in  the  lung,  where  in  such 
a  condition  the  capillaries  of  the  alveolar  wall  assume  a  tortuous  or 
varicose  appearance. 

In  exudative  inflammations  the  capillaries  play  an  important  part. 
The  component  cells  show  swelling  of  the  nucleus  and  cell  body,  with 
some  granulation  of  the  protoplasm. 

In  proliferative  inflammation  the  appearances  are  similar,  but  are 
of  a  more  progressive  character.  Owing  to  local  collections  of  granular 
protoplasmic  substance,  small  buds  are  produced  which  ultimatel} 
develop  into  new  capillaries.  The  same  process  is  seen  in  many  tumors. 

Like  the  arteries,  the  capillaries  are  frequently  affected  by  fatty 
degeneration,  calcareous  deposit,  hyaline  or  colloid  degeneration.  Perhaps 
the  most  important  of  these  is  amyloid  disease.  In  the  case  of  the  lymph- 
glands,  the  liver,  spleen,  and  the  kidneys,  amyloid  disease  is  amyloid 
disease  of  the  capillaries. 

Capillary  angiomas,  telangiectases,  are  generally  congenital,  but  may 
appear  or  increase  in  size  after  birth.  They  form  usually  flat  tumor- 
like  masses  in  the  subcutaneous  tissues  of  soft  consistency,  and  of 
bright  red  color.  They  may  be  combined  with  true  tumor  formation, 
as  sarcoma,  carcinoma,  or  lipoma. 


THE  VEINS. 

The  pathological  changes  in  the  veins  are  very  similar  to  those  of  the 
arteries,  except  that  inflammation  is  distinctly  more  common,  and  there 
is  greater  tendency  to  tumor  formation. 

Varicosity  (Phlebectasia). — Varicosity  is  a  dilatation  of  a  vessel 
due  to  a  mechanical  hindrance  to  the  free  return  flow  of  the  blood,  to 
local  or  general  stasis,  to  compression  of  the  veins,  thrombosis,  and 
heart  weakness.  Disease  of  the  venous  wall  is  a  predisposing  cause. 
The  vessel  may  be  dilated  cylindrically,  or  become  sacculated,  or  com- 
plicated loops  may  be  formed.  Adjacent  loops  may  fuse  so  that  anasto- 
mosing venous  sinuses  are  produced.  The  walls  in  time  become 
thickened  or  even  calcified,  and  the  vessel  is  usually  both  tortuous  and 
elongated.  Varices  are  found  most  frequently  in  the  lower  extremities, 
pelvic  veins,  the  broad  ligaments,  spermatic  veins,  prostate,  bladder, 
scrotum,  labia,  and  the  rectum.  In  cases  of  cirrhosis  of  the  liver,  the 


202 


THE  ARTERIES,   VEINS,   AND  LYMPHATIC  VESSELS 


veins  of  the  oesophagus,  the  portal  vein,  the  veins  of  the  abdominal 
wall  and  hepatic  ligaments  are  often  greatly  dilated. 

Varices  are  a  frequent  cause  of  hemorrhage,  oedema,  inflammation, 
ulceration,  thrombosis,  calcification,  and  pachydermia. 


FIG.  46 


Varicose  veins  of  the  leg.      (From  the  Surgical  Clinic  of  the  Montreal  General  Hospital.) 


Phlebitis. — Simple  and  Suppurative  Phlebitis. — This  frequently  occurs, 
and  the  histological  changes  are  similar  to  those  in  the  case  of  the 
arteries. 

Proliferative  Thrombophlebitis. — Proliferative  thrombophlebitis  is  fre- 
quent in  the  veins  of  the  lower  extremities,  the  pelvis,  and  the  sinuses  of 
the  brain.  As  in  the  case  of  the  arteries,  it  can  lead  to  partial  or  com- 
plete obliteration  of  the  lumen.  The  thrombus  may  calcify,  forming 
a  phlebolith. 


LYMPHANGITIS  203 

Tuberculous    Phlebitis.— Tuberculous    phlebitis    and    periphlebitis    is 

analogous  to  tuberculous  arteritis  and  peri-arteritis. 

Syphilitic  Phlebitis. — Syphilitic  phlebitis  is  most  frequent  in  the  district 
of  the  portal  vein  and  in  the  umbilical  vein  of  the  newborn. 

Phlebosclerosis. — A  condition  of  fibroid  thickening  of  the  intima  and 
media  of  veins  closely  resembling  that  seen  in  arteriosclerosis  is  much 
more  common  than  is  usually  imagined.  Such  phlebosclerosis  affects 
more  particularly  veins  that  are  poorly  supported,  and  this  even  in 
young  adults:  it  is  unassociated  with  any  signs  of  progressive  inflamma- 
tion, and  as  our  colleague  Dr.  C.  F.  Martin1  concludes,  must  be  placed 
in  the  group  of  "strain  fibroses." 


THE  LYMPHATIC  VESSELS. 

Affections  of  the  smaller  lymphatics  are  almost  invariably  associated 
with  disease  of  the  tissue  in  which  they  lie.  Pathological  conditions  of 
the  larger  trunks,  in  the  main,  concern  us  here.  When  we  remember, 
however,  that  the  pleura,  the  peritoneum,  and  other  serous  sacs  are  large 
spaces  in  close  relationship  with  the  lymph-channels,  the  subject  attains 
important  proportions. 

The  lymphatics  derive  their  importance  from  the  fact  that  they 
afford  a  ready  means  for  the  invasion  of  the  organism  or  particular 
regions  by  inflammatory  processes,  and  for  the  dissemination  of  malig- 
nant growths.  Further,  by  their  obstruction  they  give  rise  to  several 
remarkable  conditions. 

Lymphangitis. — A  frequent  disorder  of  the  lymphatics  is  lymph- 
angitis and  perilymphangitis.  It  is  almost  invariably  due  to  the  presence 
of  an  infective  inflammation  in  some  part  drained  by  the  affected 
vessels.  Infective  organisms  pass  rapidly  up  the  channels  from  the 
wound,  and  may  ultimately  reach  the  lymph-glands,  where  they  often  set 
up  hyperplastic  and  even  suppurative  changes.  Thus,  infection  from  a 
wound  of  the  foot  may  reach  the  inguinal  glands.  The  affected  lym- 
phatics are  seen  as  reddish  lines  extending  up  the  limb,  and  are  somewhat 
painful.  In  the  mildest  form,  the  endothelial  cells  are  swollen  and 
show  nuclear  division.  In  more  advanced  cases,  they  are  desquamated 
and  the  lumen  of  the  vessel  is  filled  with  debris,  lymphoid  cells,  and 
fibrin.  In  the  suppurative  form  the  channels  may  be  dilated,  owing  to 
collection  of  the  pus,  and  may  thus  resemble  a  rosary.  The  inflamma- 
tion spreads  to  the  surrounding  tissues,  which  are  cedematous,  hyperemic, 
and  infiltrated  with  leukocytes.  Lymphangitis  may  heal  completely 
with  regeneration  of  the  destroyed  cells,  abscesses  may  form,  or  indura- 
tion from  fibrous  hyperplasia  may  result. 

Of  much  importance  are  the  specific  inflammations,  especially  the 
tuberculous. 

1  Transactions  Assoc.  of  Amer.  Phys.,  20 :  1905 :  525.  See  also  Fischer,  Ziegler's 
Beitr.,27:1900:494. 


204          THE  ARTERIES,  VEINS,  AND  LYMPHATIC  VESSELS 

Tuberculosis. — Tuberculosis  attacks  the  various  organs  with  great 
avidity  through  the  lymphatics.  Tuberculosis  of  the  thoracic  duct  is 
a  frequent  cause  of  miliary  tuberculosis  in  children,  and  tuberculous 
lymphangitis  is  a  common  method  of  infection  in  the  lungs.  In  tuber- 
culous ulceration  of  the  intestines,  one  can  frequently  see  subserous 
tubercles  situated  along  the  course  of  the  lymphatic  vessels. 

Syphilis. — In  syphilis,  in  addition  to  a  proliferative  lymphangitis, 
gummatous  infiltration  of  the  wall  is  seen.  The  lymphatics  are  also 
affected  in  leprosy  and  glanders. 


FIG.  47 


Cavernous  lymphangioma  of  the  axilla  of  congenital  origin.      (Warren.) 

Dilatation  (Lymphangiectasis). — Dilatation  is  the  result  of  some 
obstruction  to  the  free  outflow  of  lymph.  This  may  be  due  to  pressure 
of  external  tumors,  aneurisms,  enlarged  glands,  metastatic  growths  in 
the  wall,  chronic  inflammation,  impaction  of  filaria,  thrombosis  of  the 
left  innominate  vein  or  of  the  duct  itself,  backward  pressure  in  the 
subclavian  vein  from  tricuspid  insufficiency.  The  condition  is  often  seen 
very  prettily  on  the  serosa  of  the  intestine  in  the  neighborhood  of  tubercu- 
lous and  typhoid  ulcers,  where  the  chyle  vessels  are  found  as  delicate 
transparent  tubes  filled  with  clear  fluid.  As  a  consequence  of  overdis- 
tension,  or  in  the  course  of  operations,  the  vessel  wall  may  be  ruptured 
and  a  condition  of  lymphorrhagia  may  be  the  result.  The  interesting 
condition  of  chylous  ascites  is  due  to  the  rupture  of  the  lymph  channels 


TUMORS 


205 


from  traumatism,  erosion,  or  to  rupture  of  the  thoracic  duct  itself  (see 
p.  111).  Similarly  chylous  hydrothorax  may  be  produced.  The  allied 
conditions  of  chyliform  and  pseudochylous  ascites  have  already  been 
described  (p.  111). 

Parasites. — Among  parasites  found  in  the  lymph-vessels  may  be 
noted  the  echinococcus  and  the  Filaria  sanguinis.  The  latter  is  asso- 
ciated with  certain  cases  of  elephantiasis  found  in  the  tropics  and  the 
condition  known  as  chyluria. 

The  retrograde  changes  are  of  little  importance.  Fatty  degeneration 
of  the  endothelium  is  common;  and  calcification  of  the  thoracic  duct  as 
a  result  of  productive  inflammation  has  been  observed. 

Hypertrophy  of  the  walls  of  the  lymph  channels  has  been  noted  in  cases 
of  obstruction. 

FIG.  48 


Macroglossia.      (Dr.  Shepherd's  case,  Montreal  General  Hospital.) 


More  important  are  the  tumors.  Carcinomata  have  a  special  tendency 
to  spread  by  means  of  the  lymphatics,  and  often  considerable  portions 
of  a  vessel  may  be  blocked  by  cancer  cells.  This  occurs  not  merely  in  the 
neighborhood  of  the  original  growth,  but  affects  even  distant  parts,  as, 
for  example,  when  we  get  metastases  in  the  lymph-vessels  of  the  lungs 
in  carcinoma  of  the  stomach.  Besides  carcinomas,  chondromas  seem 
to  have  a  tendency  to  grow  into  the  lymphatics.  The  most  important 
and  interesting  primary  tumor  is  the  endothelioma,  a  growth  which  has 
some  resemblances  both  to  carcinoma  and  sarcoma,  and  hence  has  been 
called  by  some,  ill-advisedly,  sarcocarcinoma. 

This  tumor  is  found  particularly  in  connection  with  the  larger  serous 


206 


THE  ARTERIES,   VEINS,   AND  LYMPHATIC  VESSELS 


sacs,  but   also   originates   in   the  vessels.      It   develops,  consequently, 
in  the  pleura,  the  peritoneum,  the  dura  mater,  and  the  various  organs. 


FIG.  49 


Elephantiasis  of  the  leg. 


Enormous  enlargement  of  the  limb,  with  ichthyosis. 
Museum,  McGill  University.) 


(Pathological 


The  growth  forms  either  multiple  small  nodules  on  the  serous  mem- 
brane, only  slightly  elevated  above  the  general  level  and  often  so  minute 
as  to  be  easily  overlooked,  or  extensive  sheet-Uke  masses,  Microscopic- 


/•:/.  I'PHANTIASIS  207 

ally,  it  consists  in  masses  of  cells,  not  unlike  epithelial  cells,  having  an 
alveolar  arrangement,  which  are  really  only  lymphatic  channels  with 
thickened  and  proliferated  endothelium.  The  growth  is  malignant. 

A  second  primary  tumor  is  the  lymphangioma,  which  presents  itself 
either  as  a  diffuse  enlargement  of  the  part  with  preservation  of  its  out- 
ward contour,  or  as  a  definite  tumor  mass.  It  occurs  chiefly  in  the 
connective  and  submucous  tissues  in  the  throat,  neck,  fongue,  and  lips 
(macroglossia,  macrocheilia),  the  extremities,  mesentery,  and  kidneys. 
Most  of  these  are  more  accurately  conditions  of  congenital  lymphangiec- 
tasis  (vol.  i,  p.  754),  although  the  true  lymphangioma  does  occur. 

Allied  to  this  is  the  condition  known  as  elephantiasis  lymphangiectatica, 
which  is  seen  most  frequently  in  the  extremities,  the  scrotum,  and  the 
vulva. 


CHAPTER    X. 

THE  BLOOD-FORMING  ORGANS. 
THE  LYMPHATIC  GLANDS.1 

LYMPHADENOID  tissue  is  widely  distributed  throughout  the  body, 
being  found  in  almost  all  the  organs  in  the  form  of  follicles  or  scattered 
lymphoid  cells.  More  important  than  this,  it  is  aggregated  in  certain 
regions  into  definite  glands,  which  form  an  integral  part  of  the  lymph- 
vascular  system. 

The  structure  of  a  lymph-node  is  fairly  simple.  It  is  composed  of 
a  fibrous  capsule  sending  in  trabeculse,  which  break  up  into  innumerable 
fine  ramifications,  so  as  to  form  a  reticulated  stroma.  On  the  walls 
of  the  spaces  thus  produced  are  situated  large  mononuclear  cells  with 
clear  protoplasm — the  endothelioid  plates.  The  remaining  portion  of 
the  cavities  is  filled  up  with  small,  round  cells,  which  contain  a  single, 
relatively  large,  and  deeply  staining  nucleus,  in  all  respects  resembling 
the  lymphocyte  of  the  blood.  In  the  outer  zone  the  lymphoid  elements 
are  grouped  into  follicles  to  form  the  cortex  of  the  node.  In  the  central 
portion,  or  medulla,  wavy  strands  of  connective  tissue,  lined  with* 
endothelioid  cells,  are  found,  constituting  the  sinuses,  which  are  directly 
continuous  with  the  lymphatic  vessels.  The  cells  of  the  medulla  are  larger 
and  stain  more  feebly  than  those  of  the  cortex,  and  the  nuclei  frequently 
show  evidences  of  mitosis,  proving  that  this  portion  is  the  germinal 
centre  of  the  node.  A  striking  point  in  connection  with  the  lymphatic 
glands  is  that  they  possess  the  embryonal  characteristic  of  active  growth, 
so  that  the  cells,  under  the  influence  of  a  very  slight  stimulus,  rapidly 
undergo  nuclear  division  and  proliferation.  This  is  the  feature  that 
dominates  the  picture  in  all  the  important  pathological  processes  affecting 
these  structures. 

The  function  of  the  lymphatic  glands  is  to  act  as  a  sort  of  filter  for 
the  lymph,  which  enters  the  sinuses  in  the  medulla  and  gradually  per- 
colates into  the  cortex,  where  it  is  taken  up  by  the  efferent  lymphatics. 
In  this  way,  should  the  lymph  contain  any  foreign  substance  or  toxic 
material,  these  tend  to  be  stopped  within  the  gland,  and  thus,  not  only 
on  account  of  the  anatomical  peculiarities  of  the  structure,  but  also  of 
the  cellular  hyperplasia  that  results  from  the  irritation,  this  barrier  action, 
as  will  readily  be  understood,  is  a  most  important  one.  The  lymphatic 

1  While  strongly  objecting  to  this  designation,  on  the  ground  that,  speaking  of 
these  as  glands,  the  student  becomes  confused  in  his  conception  of  glandular  activity, 
we  realize  that  the  term  is  so  firmly  established  that  it  would  be  pedantic  to 
change  it. 


CONGENITAL  ANOMALIES  209 

u la  nds  are  set  like  sentinels  to  guard  all  the  orifices  and  channels  of  the 
body,  and  frequently  prevent  systemic  infection.  This  function  of  the 
glands  has  been  clearly  demonstrated  by  the  researches  of  Bizzozero, 
Huffier,  and  Ribbert,  who  have  shown  that  the  glands  in  the  pharynx, 
neck,  root  of  the  lungs,  and  mesentery  in  healthy  animals  contain 
bacteria.  A  further  important  function  is  said  to  be  the  formation 
of  leukocytes,  which  is  especially  active  under  most  conditions  of 
infection. 

Lymph-nodes  are  of  two  kinds — ordinary  lymph-nodes  and  hemo- 
lymph-nodes.  The  latter  were  first  discovered  by  Gibbes,1  and  more 
fully  described  by  Robertson2  and  Swale  Vincent.3  They  have  been 
found  in  the  sheep,  ox,  pig,  horse,  and  man.  The  most  recent  and 
comprehensive  study  of  these  structures  is  that  of  Warthin,4  who  divides 
them  into  two  varieties,  splenolymph-nodes  and  marrow  lymph-nodes,  with 
numerous  transitional  forms.  He  has  shown  that  there  is  a  close  relation- 
ship between  the  lymph-nodes,  the  spleen,  and  bone-marrow,  and  that 
the  hemolymph-nodes  can  take  on  more  or  less  completely  the  structure 
and  function  of  the  spleen  or  marrow  when  either  of  these  is  incapacitated 
through  disease.  Under  normal  conditions  the  hemolymph-nodes  are 
concerned  chiefly  in  hemolysis  and  leukocyte  production.  The  spleno- 
lymph-nodes are  found  chiefly  in  the  neighborhood  of  the  solar  plexus, 
adrenal  and  renal  vessels,  omentum,  mesentery,  epiploica,  thymus  and 
thyroid  glands.  The  marrow  lymph-nodes  are  found  only  in  the  retro- 
peritoneal  tissues,  near  the  great  vessels,  particularly  the  vena  cava, 
aorta,  and  common  iliacs.  Unlike  the  ordinary  lymph-nodes,  the  hemo- 
lymph-nodes, except  the  transitional  forms,  contain  blood-sinuses  but 
no  lymph-sinuses.  Hemolymph-nodes  are  dark  red  or  bluish  in  color, 
possess  a  hilus  into  which  a  large  vessel  enters,  and  are  usually  surrounded 
by  a  plexus  of  veins.  On  section  they  resemble  spleen  pulp.  They 
have  a  particular  importance  in  connection  with  the  various  forms  of 
anemia. 

As  will  readily  be  inferred,  the  most  important  affections  of  the  lymph- 
nodes  are  the  inflammatory,  and  this  from  a  clinical  as  well  as  a  patho- 
logical point  of  view.  They  are,  however,  rarely  affected  as  a  system, 
except  in  the  possible  case  of  leukemia  and  lymphosarcoma.  As  a  rule, 
only  the  glands  belonging  to  a  certain  anatomical  district  are  involved. 


CONGENITAL  ANOMALIES. 

These  are  not  important,  with  the  exception  of  that  excessive  produc- 
tion  of    lymphoid   tissue    throughout   the    body  characteristic  of  the 

1  Quart.  Jour.  Micr.  Sciences,  24: 1884: 186;  and  Amer.  Jour,  of  the  Med.  Sci., 
24:1893:316. 

2  Lancet,  London,  1890:11:1152. 

3  Jour  Anat.  and  Physiol.,  31 : 1897: 176. 

4  A  Contribution  to  the  Normal  Histology  and  Pathology  of  the  Hemolymph 
Glands,  Jour,  of  Medical  Research,  6: 1901 : 3. 

14 


210  THE  LYMPHATIC  GLANDS 

so-called  "  status  lymphaticus."  It  may,  perhaps,  be  noted  here  that 
in  infancy  and  childhood  the  lymph-glandular  system  is  very  prominent, 
and  as  puberty  is  reached  it  becomes  relatively  less  important. 


CIRCULATORY  DISTURBANCES. 

Anemia. — The  circulatory  disturbances  are  also  of  no  special  interest. 
The  lymph -glands  normally  contain  but  little  blood,  and  in  general 
anemia  even  this  may  disappear. 

Hyperemia. — Hyperemia  is  almost  inseparably  associated  with 
inflammation.  The  glands  are  reddened,  enlarged,  and  succulent. 

Hemorrhages. — Hemorrhages  in  such  cases  readily  occur,  which 
may  also  be  due  to  minute  emboli  in  the  cortical  vessels  leading  to 
rupture. 

(Edema. — (Edema  may  be  inflammatory  or  part  of  a  general  anasarca. 

Varices. — A  curious  occurrence  is  the  formation  of  varices  or  cysts 
in  the  centre  of  the  nodes  owing  to  obstruction  of  the  efferent  lymphatics, 
which  is  generally  brought  about  by  inflammation.  In  severe  cases  the 
dilated  and  tortuous  sinuses  may  coalesce  and  the  node-substance  be 
distended  into  a  large  cystic  space  (adenolymphocele)  /  varying  in  size 
from  that  of  a  nut  to  that  of  one's  head.  The  inguinal  glands  are  those 
usually  involved,  and  young  people  are  particularly  liable  to  be  affected. 
The  disease  is  endemic  in  some  tropical  countries. 


INFLAMMATIONS. 

Lymphadenitis. — Inflammation  of  the  lymph- glands — lymphaden- 
itis— is  one  of  the  commonest  of  conditions.  It  is  usually  brought 
about  by  infective  agents  or  toxins  reaching  them  through  the  afferent 
lymphatics.  Not  infrequently,  also,  the  process  arises  by  extension  of 
inflammation  from  the  adjacent  structures;  more  rarely  the  affection  is 
hematogenic.  Lymphadenitis  is  invariably  secondary  to  infection  else- 
where. The  glands  nearest  the  point  of  entrance  of  the  offending 
bacteria  are  chiefly  affected,  but  those  at  some  distance  are  quite  com- 
monly involved,  owing  to  the  action  of  diffusible  toxins.  In  many 
diseases,  such  as  diphtheria  and  variola,  lymphadenitis  is  a  marked 
feature,  and  in  one — plague — it  may  give  the  character  to  the  clinical 
type  (bubonic  plague).  In  certain  other  diseases,  such  as  leukemia 
and  Hodgkin's  disease,  the  involvement  of  the  glands  is  striking.  The 
relationship  of  chronic  inflammatory  change  to  the  latter  condition  has 
been  discussed  in  our  first  volume  (p.  683). 

Acute  Lymphadenitis. — Acute  lymphadenitis  is  simple  or  suppurative. 
Both  forms  have  much  in  common,  for  the  suppurative  variety  generally 

1  Auger,  Des  Tumeurs  e"rectiles  lymphatiques  (adenolymphoceles),  These  de 
Paris,  1867. 


'CUTE  LYMPHADENITIS  211 


supervenes  upon  the  other.  Suppurative  lymphadenitis  is  due  to  infec- 
tion with  pyogenic  microorganisms,  and  may  result  from  septic  wounds, 
puerperal  metritis  and  endometritis,  gonorrhoea,  chancroid,  diphtheria, 
and  scarlatina.  When  inflamed,  the  glands  are  enlarged,  hyperemic, 
and  soft  (bubo).  On  section,  they  vary  in  color  from  gray  or  grayish- 
white  to  pink,  and  are  succulent,  so  that  a  milky  juice  can  be  scraped 
from  the  surface.  In  the  early  stages  the  congestion  is  confined  to 
the  cortex,  but  sooner  or  later  it  becomes  impossible  to  differentiate 
between  the  cortex  and  medulla.  In  some  cases,  as  of  diphtheria  and 
typhoid,  one  can  make  out  dull,  opaque,  necrotic  areas,  and,  in  the  most 
severe  forms,  softening,  with  the  formation  of  thick,  greenish-yellow  pus. 
In  many  instances  the  fibrous  capsule  of  the  glands  and  the  neighboring 
tissues  are  cedematous,  infiltrated,  and  congested  (perilymphadenitis). 

FIG.  50 


*• .  w 

'    at.      9 


£•''. 


Section  of  mesenteric  gland  in  typhoid  fever,  showing  the  enlarged  endothelial  and  giant  cells. 
Zei.ss  ohj.  y1^,  oil  immersion,  without  ocular.  (From  the  Pathological  Laboratory  of  McGill 
University.) 

Microscopically,  the  enlargement  of  the  glands  is  found,  in  the  main, 
to  consist  of  a  hyperplasia  of  the  cellular  elements,  as  evidenced  by 
nuclear  division  and  increase  in  the  number  of  cells.  Not  only  are  the 
lymph  elements  affected  in  this  way,  but  the  endothelial  plates  prolifer- 
ate and  are  found  in  great  numbers,  often  with  several  nuclei,  more 
particularly  in  the  lymph-channels.  This  "catarrh"  of  the  plates,  as 
has  been  shown  by  Mallory,1  is  a  prominent  feature  in  the  mesenteric 
glands  and,  to  some  extent,  in  others,  in  typhoid  fever,  where  in  many 

1  Jour.  Exper.  Med.,  3:1898:611. 


212  THE  LYMPHATIC  GLANDS 

cases  they  fuse  into  giant  cells  having  phagocytic  properties.  The 
trabeculse  and  the  capsule  may  be  oedematous  and  infiltrated  with  cells. 
Not  infrequently  the  lymph- sinuses  are  dilated  with  an  exudate  con- 
taining but  few  cells.  In  the  severer  forms  of  inflammation  the  exudate 
may  be  fibrinous  or  hemorrhagic,  or  both.  Fibrinous  deposit  is  seen 
chiefly  in  the  sinuses,  and  is  especially  prominent  in  lobar  pneu- 
monia and  diphtheria.  Occasionally  spots  of  necrosis  may  be  seen 
where  the  lymphoid  cells  and  those  of  the  stroma  lose  their  staining 
power,  become  granular,  and  finally  disintegrate.  This  change  is 
brought  about  by  the  direct  action  of  the  toxin,  but  also,  no  doubt,  by 
the  obstruction  to  the  circulation  caused  by  the  cellular  proliferation 
and  accumulation  of  debris.  Should  suppuration  occur,  there  is  an 
abundant  aggregation  of  polymorphonuclear  leukocytes  in  the  lymph- 
spaces,  together  with  necrosis  and,  ultimately,  liquefaction.  When 
this  event  does  not  occur,  it  is  possible  for  the  inflammation  to  resolve. 
The  redundant  cells  and  fibrin  undergo  fatty  degeneration,  and  finally 
disintegrate  and  dissolve,  the  debris  being  carried  off  in  the  lymph-stream 
or  taken  up  by  the  phagocytes.  In  cases  where  hyperemia  has  been 
marked,  or  where  hemorrhage  has  taken  place,  it  is  common  for  pigment 
to  be  deposited.  In  the  severer  forms  it  is  usual  for  the  gland  to  remain 
somewhat  enlarged  and  firm,  owing  to  fibrous  hyperplasia.  Where 
necrosis  or  suppuration  has  occurred,  a  definite  scar  may  be  the  result, 
or,  if  the  necrosed  substance  be  not  absorbed,  the  gland  may  undergo 
calcification.  In  some  cases,  where  the  gland  has  burst  externally,  a 
sinus  discharging  lymph  has  persisted  for  some  time. 

Chronic  Lymphadenitis. — Chronic  lymphadenitis  occurs  as  a  sequel 
to  the  acute  form,  but  may  arise  independently.  In  the  latter  case  it  is 
due  to  the  action  of  mild  or  repeated  irritation.  Apart  from  syphilis 
or  tuberculosis,  the  most  common  cause  is  the  presence  of  dust  or  other 
foreign  material.  The  process  is  seen  most  commonly  in  the  peri- 
bronchial  glands,  where  large  amounts  of  coal,  stone,  steel,  or  other  dust 
may  be  deposited,  and  in  the  axillary  glands  after  tattooing. 

The  affected  nodes  are  enlarged,  firm,  and  variously  pigmented, 
according  to  the  nature  of  the  offending  material.  In  the  earlier  stages 
the  enlargement  is  due  to  simple  cellular  hyperplasia,  but  soon  the 
capsule  and  stroma  become  noticeably  thickened,  encroaching  grad- 
ually upon  the  lymphoid  cells,  until,  eventually,  there  is  atrophy  of  the 
lymphoid  elements,  and  the  node  is  converted  into  a  fibrous  nodule  or 
encapsulated  mass  of  pigment.  In  other  cases  the  necrosis  is  so  rapid 
that  the  node  softens  and  its  contents  may  be  discharged  into  the 
nearest  hollow  viscus. 

Tuberculosis. — Tuberculosis  of  the  lymph-glands,  in  most  cases,  is 
brought  about  by  bacilli  that  reach  them  through  the  afferent  lymph- 
vessels;  more  rarely  from  the  blood-stream.  The  glands  chiefly  affected 
are  the  cervical,  peribronchial,  and  mesenteric. 

In  exceptional  cases  the  disease  is  widespread,  involving  the  axillary, 
inguinal,  and  retroperitoneal  regions  as  well.  Often  the  organs  from 
which  the  infected  lymph  is  derived  show  evidences  of  tuberculosis, 


TUBERCULOSIS  213 

hut  this  is  hy  no  means  necessary.  In  the  case  of  the  lungs,  for  instance, 
while  a  flection  of  the  peribronchial  glands  is  attributable  in  some  cases 
to  a  primary  pulmonary  lesion,  yet,  as  Ilibhert  and  Baumgarten  have 
shown,  the  reverse  process  is  frequently  at  work,  and,  in  tuberculosis  of 
the  cervical  glands,  where  infection  takes  place  through  the  tonsils, 
gums,  and  nasopharynx,  it  is  rare  for  the  latter  structures  to  show 
the  lesions  of  the  disease.  Similarly,  in  tuberculosis  of  the  mesenteric 
glands,  the  mucous  membrane  of  the  intestines  may  entirely  escape. 

The  affected  glands  are  noticeably,  and  in  some  cases  enormously, 
enlarged,  presenting  all  the  signs  of  a  more  or  less  intense  inflammation. 
In  the  earlier  stages  section  reveals  a  homogeneous,  translucent  infil- 
tration of  grayish  color,  affecting  either  the  greater  portion  of  the  gland 
or  merely  scattered  areas.  Later,  this  becomes  opaque,  granular  look- 
ing, and  may  finally  caseate,  so  that  the  mass  becomes  yellowish- white 
in  color,  granular,  and  friable.  In  some  cases,  possibly  from  secondary 
infection,  the  glands  suppurate  and  may  discharge  their  contents  into  the 
nearest  cavity.  Rupture  into  a  vein  or  into  the  thoracic  duct  is  the  most 
potent  cause  of  systemic  miliary  infection  with  tuberculosis.  Rupture 
may  also  take  place  into  the  pericardium,  mediastinum,  oesophagus, 
and  intestinal  tract.  In  long-standing  cases  the  glands  become  shrunken, 
more  or  less  fibrosed,  and  destroyed,  and  may  contain  calcareous  spicules. 
Microscopically,  there  is  an  increase  in  the  number  of  round  cells, 
partly  from  the  proliferation  of  the  lymphoid  elements  and  partly  from 
the  diapedesis  from  the  vessels.  With  this,  there  is  more  or  less  hyper- 
plasia  of  the  endothelioid  cells.  In  the  more  advanced  stages  the  centre 
is  occupied  by  isolated  or  confluent  areas  of  caseation,  the  tissue  in  the 
neighborhood  showing  evidences  of  cellular  disintegration  and  nuclear 
destruction.  In  chronic  cases  giant  cells  make  their  appearance  about 
the  periphery  of  the  tubercle,  and  in  still  more  chronic  forms  there  is  an 
attempt  at  walling  off  the  dead  material  with  fibrous  tissue.  Calcifi- 
cation may  also  be  noted.  True  bone  has  occasionally  been  observed.1 

A  peculiar  form,  which  deserves  special  mention  on  account  of  its 
resemblance  to  Hodgkin's  disease,2  is  chronic  hyperplastic  tuberculosis. 
This  disease  has  been  described  in  connection  with  the  intestinal  tract, 
the  serous  membranes,  and  the  lymphatic  glands.  The  glands  are  con- 
siderably enlarged,  and  are  hard,  showing  no  striking  evidence  of 
acute  inflammatory  action.  On  section,  they  are  firm,  grayish,  and 
translucent  in  appearance,  or  present  small  grayish  dots.  Caseation  is 
absent,  or  at  least  reduced  to  a  minimum. 

Microscopically,  the  chief  feature  is  a  proliferation  of  the  endothelial 
plates,  with  little  or  no  leukocytosis.  The  endothelial  cells  at  first  form 
small  masses,  which  ultimately  coalesce  and  encroach  upon  the  lymphoid 
structure  of  the  gland,  so  that  the  hyperplastic  tissue  is  made  up  of  large 
mononuclear  cells,  either  round,  stellate,  or  spindle-shaped,  with  pale 

'Lubarsch,  Virch.  Arch.,  177-1904:371. 

2  See  Fagge,  Path.  Trans.,  Lond.,  25:1874:235:  and  Bonnet,  Jour,  de  Phys.  and 
Path,  gen.,  10:827:1899. 


214  THE  LYMPHATIC  GLANDS 

nuclei  and  relatively  abundant  protoplasm.  Very  often  these  cell 
collections  undergo  hyaline  change  and  are  converted  into  translucent, 
structureless  masses.  Caseation  is  seen  only  in  very  long-standing  cases. 
This  affection  is  apparently  the  benign  form  of  tuberculosis.  Duval1 
has  reproduced  it  in  the  rabbit  by  injections  of  attenuated  tubercle 
bacilli. 

Syphilis. — The  initial  lesion  of  syphilis  is  followed  by  a  slow,  painless 
enlargement  of  the  nearest  lymph-glands  (indolent  bubo).  The  inguinal, 
axillary,  and  epitrochlear,  cervical  and  prevertebral  glands  have  been 
found  affected.  The  glands  are  moderately  enlarged  and  firm.  Micro- 
scopically, the  condition  is  found  to  be  due  to  thickening  of  the  capsule 
and  septa,  with  hyperplasia  both  of  the  lymphoid  and  endothelial  cells. 
The  endothelial  plates  lining  the  lymph-spaces  are  the  ones  chiefly 
involved.  The  Spirocheta  pallida  may  often  be  detected  by  appropriate 
staining.2  The  condition  may  persist  for  months  or  even  years.  When 
healing  takes  place,  the  cells  undergo  fatty  degeneration,  the  swelling 
subsides,  and  the  glands  become  more  or  less  indurated.  Gumma 
formation  occurs  in  .tertiary  syphilis,  and  affects  usually  the  inguinal, 
submaxillary,  and  cervical  glands.  As  a  rule,  only  one  or  two  are 
affected. 

Plague. — In  one  form  of  plague,  "pestis  bubonica,"  the  involve- 
ment of  the  lymph-glands  is  striking.  In  about  one-half  of  the  cases 
the  inguinal  glands  are  those  first  attacked,  and  in  about  25  per  cent, 
the  axillary.  While  the  chain  of  glands  next  the  site  of  the  inoculation 
is  chiefly  involved,  those  elsewhere,  notably  of  the  pharynx,  the  root  of 
the  lungs,  and  the  mesentery,  are  usually  quickly  attacked.  The  nodes 
first  infected  are  greatly  enlarged,  reddened,  and  hemorrhagic,  and  the 
tissues  in  the  neighborhood  show  hemorrhagic  oedema.  In  the  early 
stages,  or  if  the  infection  be  not  very  virulent,  they  are  quite  firm,  but  in 
other  cases  they  are  softened  and  even  liquefied.  On  section,  the  nodes 
are  of  a  grayish-red  color,  with  areas  of  hemorrhage,  or  the  entire  sub- 
stance may  be  densely  infiltrated  with  blood.  In  the  severest  form  they 
undergo  colliquative  necrosis  and  contain  a  semifluid  gummy  material, 
or  a  substance  not  unlike  lard.  The  distinction  between  cortex  and 
medulla  is  usually  lost.  In  the  more  remote  nodes  hemorrhage  does  not 
occur  except  in  cases  of  relapse. 

Microscopically,  the  bloodvessels  and  the  lymphatics  are  engorged 
with  cells  and  contain  abundant  pest  bacilli.  There  is  proliferation 
both  of  the  lymphoid  and  the  endothelial  cells,  and  the  latter  contain 
large  numbers  of  bacilli  within  their  protoplasm.  An  unusual  number 
of  "  Mast-zellen"  is  also  noticeable.  Almost  the  whole  gland  may  be 
converted  into  finely  granular  material,  consisting  of  a  few  cells  with 
abundant  cellular  debris  and  hosts  of  bacteria.  A  few  plasma  cells 
may  also  be  seen,  but  polymorphonuclear  cells  are  quite  scarce.  It  is 
incorrect,  however,  to  call  the  softening  "suppuration/'  as  is  so  often 

1  Jour,  of  Exp.  Med.,  11:1909:403. 

2  Tchlenhoff,  Roussky  Vratch,  June  18,  1905,  confirming  Hoffmann. 


RETROGRESSIVE  METAMORPHOSES  215 

done.  Bacterial  thrombi  may  be  found  in  the  bloodvessels  and  lymph- 
atics, which  are  greatly  distended  and  show  proliferation  and  degenera- 
tion of  their  endothelium.  Fairly  numerous  and  well-preserved  red  cells 
can  usually  be  seen  within  the  necrotic  areas.  The  tissues  surrounding 
the  infected  glands  show  inflammatory  cedema,  the  bloodvessels  are 
dilated,  and  there  is  an  exudate  of  lymphoid  cells  and  erythrocytes  into  the 
lymph-spaces.  The  bloodvessels  contain  numerous  bacilli.  The  lymph- 
glands  at  some  distance  from  the  main  lesions  are  somewhat  enlarged  and 
of  a  darker  color  than  normal,  but  show  merely  congestion  and  hyper- 
plasia. 

Leprosy. — In  leprosy,  changes  in  the  axillary,  inguinal,  and  mesenteric 
glands  are  sometimes  met  with.  The  affected  structures  are  enlarged, 
soft,  and  of  yellow  color. 

Microscopically,  all  the  signs  of  hyperplastic  inflammation  may  be 
seen.  Fatty  changes  in  the  lymphoid  cells  and  reticulum  have  also  been 
observed,  as  well  as  phagocytes  containing  pigment  and  blood  cells. 
The  specific  bacilli  may  be  demonstrated  within  the  nodes. 

Parasites. — Filarise,  Trichinae,  Cysticerci,  and  Pentastoma  have  been 
met  with. 


RETROGRESSIVE  METAMORPHOSIS. 

Simple  Atrophy. — Simple  atrophy  occurs  as  an  involution  process 
in  old  age.  The  atrophy  affects  chiefly  the  lymphoid  elements,  although 
the  stroma  is  relatively  or  even  absolutely  increased.  The  glands  are 
small,  increased  in  consistency,  and  contain  but  little  juice.  An  inter- 
esting variety  is  the  so-called  "lipomatous"  atrophy.  Here  the  lymphoid 
cells  of  the  medulla  gradually  disappear  and  the  fibrous  stroma  under- 
goes fatty  metamorphosis.  The  process  gradually  extends  to  the  cortical 
portion  and  the  original  follicular  structure  may  in  time  be  utterly 
destroyed.  The  affection  is  said  to  occur  chiefly  in  the  glands  of  the 
mesentery,  especially  in  cases  of  chronic  alcoholism. 

Amyloid  Disease. — The  lymph-glands,  particularly  those  of  the 
abdomen,  may  be  affected  in  case  of  widespread  amyloid  change. 
Occasionally,  especially  where  there  is  chronic  bone  disease,  they 
may  be  early  or  alone  involved.  In  advanced  cases  the  nodes  are 
enlarged,  hard,  and  of  a  grayish-white  semitranslucent  appearance. 
The  disease  begins  in  the  walls  of  the  arteries  and  capillaries  of  the 
cortex  and  in  the  fibrous  septa,  where  it  leads  to  the  production  of  small 
flattened  nodules  that  gradually  encroach  upon  the  lymphoid  cells,  and 
in  time  bring  about  complete  atrophy.  In  rarer  cases  the  walls  of  the 
lymph-sinuses  in  the  medulla  are  chiefly  or  alone  affected. 

Hyaline  Degeneration. — This  is  found  particularly  as  a  secondary 
manifestation  in  tuberculous  nodes.  Generally,  the  reticulum  and  the 
lymphoid  cells  are  swollen,  hemorrhagic,  and  transparent,  and  are  fused 
into  irregular  masses.  Such  hyaline  degeneration  is  apt  to  be  the 
precursor  of  caseation,  though  it  is  not  invariably  associated  with  tuber- 


216  THE  LYMPHATIC  GLANDS 

culosis.  Less  frequently  the  degeneration  affects  the  walls  of  the  smaller 
vessels. 

Calcification. — Calcification  is  usually  met  with  in  connection  with 
chronic  tuberculosis  of  the  glands,  but  may  be  found  in  previously  healthy 
glands,  as  in  osteomalacia. 

Necrosis. — Necrosis  is  fairly  common  in  cases  of  inflammation,  occur- 
ring, for  instance,  in  diphtheria,  typhoid,  tuberculosis,  bubonic  plague, 
and  septicemia.  It  is  usually  due  to  blocking  of  vessels,  owing  to  hyper- 
plasia  of  their  endothelial  lining.  Extensive  glandular  necroses  have  been 
described  in  connection  with  severe  burns  of  the  skin.  J.  McCrae  has 
produced  a  careful  study  of  this  form.1 


PROGRESSIVE  METAMORPHOSES. 

In  the  present  transitional  state  of  our  ideas  it  is  impossible  to  give 
a  thoroughly  scientific  description  of  the  progressive  changes  affecting 
the  lymph-glands. 

Hyperplasia. — Hyperplasia  is  one  of  the  commonest  of  these  changes, 
and  arises  from  a  number  of  different  causes.  It  is  frequently  impossible 
to  draw  a  hard  and  fast  line  between  what  are  merely  compensatory 
or  inflammatory  hyperplasias  and  true  tumor  formation.  The  whole 
subject  of  overgrowth  of  the  lymphatic  glands  at  the  present  time  is 
in  such  almost  hopeless  confusion  that  at  this  point  we  have  thought 
it  better  to  deal  chiefly,  and  that  in  a  very  sketchy  way,  with  the  ana- 
tomical conditions  of  the  glands,  leaving  the  fuller  consideration  of  the 
subject  to  another  place  (see  vol.  i,  p.  680). 

Many  observers,  notably  Winogradow,  Pio  Foa,  and  Mosler,  have 
noted  compensatory  hyperplasia  of  the  lymph-glands  after  experimental 
extirpation  of  the  spleen  in  the  lower  animals.  A  similar  condition 
has  been  observed  occasionally  in  man.  The  compensation,  however, 
appears  to  be  temporary. 

Lymphadenia. — Under  the  term  lymphadenia,  or  "  progressive 
hyperplasia  of  the  lymph-glands,"  we  may  include  a  number  of  allied 
conditions  in  which  there  is  an  increase  in  the  lymphoid  and  other 
elements  of  the  glands,  whereby  they  become  notably  enlarged. 

Under  this  caption  we  may  conveniently  deal  with  the  conditions  known 
as  chronic  hyperplasia,  Hodgkin's  disease,  leukemia,  typical  or  benign 
lymphoma,  atypical  or  malignant  lymphoma  (lympho sarcoma). 

The  distinctions  between  these  various  affections  have  not  in  the  past 
been  by  any  means  clearly  defined,  nor  even  yet  is  their  pathogeny  fully 
understood.  The  confusion  that  has  existed  in  this  regard  is  to  be  seen, 
to  take  a  single  example,  in  the  numerous  synonyms  that  have  been  pro- 
posed for  Hodgkin's  disease,  namely,  simple  adenia  (Trousseau), 
lymphosarcoma  (Virchow),  malignant  lymphoma  (Billroth),  and  pseudo- 

1  The  Nature  of  Internal  Lesions  in  Death  from  Superficial  Burns,  American 
Medicine,  2:1901:735. 


LYMPH  ADEN  I A 


217 


leukemia  (Cohnheim).     More  recent  studies  have  done  something  to 
bring  order  out  of  chaos. 

To  begin  with,  it  is  now  beyond  question  that  we  can  get  a  relatively 
enormous  enlargement  of  the  regional  lymph-nodes  as  a  result  of  inflam- 
mation. The  majority  of  such  cases  are  probably  due  to  tuberculosis, 
which  gives  rise  to  two  types  of  lesions,  the  ordinary  granuloma  with 
caseation,  and  a  more  chronic  hyperplastic  form  without  caseation. 
In  the  latter  the  fibrous  tissue  is  increased,  large  endothelial  cells  are 
numerous,  and  the  lymphoid  elements  are  diminished.  Caseation  is 

FIG.  51 


Hodgkin's  disease  in  a  young  girl.      (From  Dr.  F.  G.  Finley's  clinic,  Montreal  General  Hospital.) 

not  present  here,  and  the  specific  bacilli  can  be  detected  with  difficulty. 
These  are  the  cases  which,  as  Hilton  Fagge  was  the  first  to  point  out,1 
simulate  Hodgkin's  disease  very  closely,  and  no  doubt  the  two  affec- 
tions have  been  frequently  confused.  In  Hodgkin's  disease  the  cervical 
nodes  are  the  first  to  be  noticeably  involved,  becoming  gradually 
greatly  enlarged  and  consolidating  into  dense  masses.  Subsequently, 
the  axillary,  inguinal,  retroperitoneal,  peribronchial,  mediastinal,  and 

1  Path.  Trans.,  London,  25:  1874:  235. 


218  THE  LYMPHATIC  GLANDS 

mesenteric  glands  present  a  similar  change,  and  finally  the  spleen  and 
liver  become  enlarged. 

Histologically,  the  process  apparently  begins  with  the  proliferation 
of  the  endothelial  plates  of  the  lymph-sinuses  and  the  large  cells  in 
the  germinal  centres.  Later,  the  lymph-sinuses  and  reticular  spaces 
are  filled  with  proliferating  lymphoid  and  endothelial  cells.  Numerous 
giant  cells  and  plasma  cells  are  also  to  be  seen.  Goldmann  and  Kanter1 
pointed  out  the  great  abundance  of  the  eosinophile  cells  in  the  nodes 
in  this  disease,  a  fact  that  has  been  emphasized  by  some,  though,  in  our 
judgment,  erroneously,  as  a  diagnostic  point  between  Hodgkin's  disease 
and  tuberculous  adenitis  (Dietrich,  Fischer,  Reed2).  As  the  disease 
advances,  the  fibrous  septa  of  the  nodes  increase  in  size,  dividing  them  into 
coarse  lobules,  and  gradually  bring  about  a  degeneration  of  the  lymphoid 
and  other  cellular  elements.  This  connective  tissue  may  show  hyaline 
metamorphosis,  and  large  areas  resembling  ischemic  necrosis  are  fre- 
quently seen.  The  histological  appearances  in  Hodgkin's  disease 
suggest  undoubtedly  an  inflammatory  and  probably  an  infectious  origin. 

In  leukemia  (of  the  lymphatic  type)  the  lymph-nodes  all  over  the 
body  are  enlarged.  Lymphoid  tissue  everywhere  is  increased,  and 
the  spleen  reaches  truly  colossal  proportions.  Histologically,  all  the 
elements  of  the  nodes  are  increased,  and,  unlike  Hodgkin's  disease, 
there  is  no  special  increase  of  fibrous  tissue.  A  striking  and  character- 
istic feature  is  the  alteration  in  the  blood  (excessive  lymphocytosis). 

Benign  lymphoma  is  extremely  rare,  but  its  occurrence  is  recognized 
by  Le  Count3  and  Kundrat. 

Lymphosarcoma,  histologically  speaking,  is  a  small  round-celled 
sarcoma,  originating  in  the  proliferation  of  the  lymphoid  elements  of 
the  lymph-nodes.  The  nodes  enlarge,  fuse  together,  and  infiltrate 
locally,  so  that  enormous  solid  masses  of  new-growth  are  produced.  It 
is  found  commonly  in  the  thorax,  involving  the  mediastinum  and  the 
structures  in  that  region;  and  has  also  been  noted  as  beginning  in  the 
submucosa  of  the  intestine.  It  does  not  form  distant  metastases.  The 
relationship  of  this  condition  to  diffuse  lymphomatosis  has  already  been 
discussed  (vol.  i,  p.  684). 

Tumors. — The  only  primary  growths  are  lymphoma,  sarcoma,  fibro- 
sarcoma,  enchondroma,  and  carcinoma. 

According  to  histological  structure,  the  sarcomas  may  be  divided  into 
round-celled,  spindle-celled,  and  alveolar.  The  growth  may  originate  in 
the  perithelium  of  the  vessels  (angiosarcoma),  the  endothelial  lining  of 
the  lymph-sinuses  (endothelioma),  from  the  lymphoid  cells  (lymphosar- 
coma),  and  from  the  connective-tissue  stroma  (sarcoma).  The  true  round- 
celled  sarcoma  is  hard  to  distinguish  from  the  lymphosarcoma  previously 

1  Centralbl.  f.  allg.  Path.,  5:  1894:  299. 

2  See  Dorothy  M.  Reed,  On  the  Pathological  Changes  in  Hodgkin's    Disease, 
with  Special  Reference  to  its  Relationship  to  Tuberculosis,  Johns  Hopkins  Hospital 
Reports,  10- 1902  :133. 

3  Jour,  of  Exper.  Med.,  4:  1899:  559. 


LYMPHADENIA 


FIG.  52 


219 


Lymph-node  in  Hodgkin's  disease.  The  section  shows  a  portion  of  the  cortex  of  a  node  with 
the  capsule.  The  horse-chestnut-like  cells  are  eosinophiles  which  were  numerous  in  this  region. 
Zeiss  obj.  y1^,  oil  immersion,  ocular  No.  I.  (From  the  Pathological  Laboratory  of  the  Montreal 
General  Hospital.) 


FIG.  53 


Lymph-node  in  Hodgkin's  disease,  showing  the  extensive  fibrosis  occurring  in  the  later  stage. 
Reichert  obj.  7a,  without  ocular.      (From  the  collection  of  the  Montreal  General  Hospital.) 


220 


THE  LYMPHATIC  GLANDS 

FIG.  54 


',**  **»r% 
,  •  *  a  -fc  * 


„>* 


Lymphosarcoma.      Leitz  obj.  No.  7,  without  ocular.      (From  the  collection  of  Dr.  A.  G.  Nicholls.) 


FIG.  55 


Secondary  columnar-celled  carcinoma  of  lymph-node.     Zeiss  obj.  DD,  without  ocular.      (From 
the  collection  of  the  Royal  Victoria  Hospital.) 


THE  SPLEEN  221 

referred  to.  It,  however,  originates  in  a  single  gland  and  does  not  tend 
to  involve  the  neighboring  glands.  It  rather  invades  the  capsule  and 
other  tissues,  and  produces  distant  foci  of  metastatic  deposit. 

Primary  carcinoma  has  been  described.1  (This  should  probably  be 
designated  alveolar  endothelioma). 

The  secondary  growths  are  carcinoma,  sarcoma,  chondroma,  and 
inyxonm.  In  secondary  sarcoma  the  glands  nearest  the  primary  growth 
become  first  involved.  They  are  enlarged  and  infiltrated  with  a  new- 
formation  more  or  less  perfectly  resembling  the  primary  growth.  The  cells 
of  the  secondary  growth  are  formed  into  clusters  or  bands  surrounded 
by  connective  tissue  derived  from  the  proliferation  of  the  stroma. 


THE  SPLEEN. 

The  exact  function  of  the  spleen  is  still  in  doubt,  but,  from  the  ana- 
tomical structure  of  the  organ  and  from  experiment,  we  can  deduce 
certain  conclusions  that  are  important,  so  far  as  they  go.  In  general 
terms,  the  spleen  may  be  described  as  an  organ  composed  of  numerous 
bloodvessels  which  discharge  their  contents  into  a  peculiarly  arranged 
lymphadenoid  tissue.  The  organ,  which  weighs  on  an  average  170 
grams,  is  bounded  by  a  capsule  of  fibrous  and  elastic  tissue  which  sends 
prolongations  or  trabeculae  into  the  substance  of  the  gland.  In  the  central 
portion  these  trabeculse  break  up  into  finer  ramifications,  so  as  to  form 
a  spongy  matrix.  From  the  hilus  passes  in  the  artery,  whose  branches 
follow  the  connective-tissue  septa.  The  Malpighian  corpuscles  are  seen 
on  section  as  whitish  dots  that  contrast  with  the  red  of  the  spleen  pulp. 
They  consist  in  small  collections  of  lymphoid  cells,  either  encircling  or 
situated  to  one  side  of  the  afferent  arterioles,  together  with  reticulated 
tissue  derived  from  the  adventitia  of  the  vessels.  They  generally,  though 
not  invariably,  contain  capillaries  that  empty  into  the  spleen  pulp.  The 
circulation  is  very  free.  The  blood  enters  at  the  hilus  by  the  splenic 
artery,  whence  it  diffuses  through  to  the  cortex.  There  is  no  doubt  now 
that  the  arterioles  ending  in  small  dilatations,  the  ampullae  of  Thoma, 
discharge  directly  into  the  pulp,  while  at  the  same  time  they  communicate 
closely  with  the  veins. 

The  pulp  is  composed  of  a  reticulated  fibrous-tissue  stroma  in  which 
lie  numerous  lymphoid  cells.  The  walls  of  the  spaces  are  lined  by 
large  mononucleated  cells,  or  endothelial  elements,  similar  to  those  lining 
the  bloodvessels  of  the  lymph-sinuses.  Besides  this  we  may  see  red  blood 
cells  in  various  stages  of  degeneration,  blood  pigment,  and  phagocytes 
containing  cellular  debris  and  pigment.  From  this  it  may  be  inferred 
that  there  is  a  close  relationship  between  the  spleen  and  the  vascular 
system.  The  lymphoid  character  of  the  pulp  cells  suggests  that  one  of 
its  functions  is  to  produce  lymphocytes,  and  this  is  possibly  correct. 
It  used  to  be  taught  that  the  large  mononuclear  and  transitional  cells 
of  the  blood  were  formed  in  the  spleen,  hence  they  were  called  "spleno- 

1  V.  Willmann,  Inaug.  Diss.  Munch.,  1904, 


222  THE  SPLEEN 

cytes."  But  we  know  now  that  the  spleen  is  not  at  least  the  only  source 
of  origin,  for  the  splenocytes  may  still  be  found  in  the  blood  after  ex- 
perimental extirpation  of  the  organ.  The  main  function  of  the  spleen, 
therefore,  seems  to  be  the  destruction  of  the  red  blood-cells  which  are 
found  in  all  stages  of  disintegration  in  the  pulp.  For  it  has  been  found 
that  when  red  blood-corpuscles  in  excess  are  introduced  into  the  circu- 
lation, there  is  a  great  increase  in  the  amount  of  the  erythrocytes  and 
pigment  in  the  spleen.  It  is  now  practically  settled  that  the  spleen  is 
not  normally  concerned  in  the  formation  of  red  cells,  except  during 
foetal  life  and  in  the  first  year  after  birth.  In  cases  of  destruction  of 
bone-marrow,  it  may  resume  this  function.1  The  hyperplasia  of  the 
spleen,  which  is  so  common  a  feature  in  many  infective  fevers,  suggests 
also  that  this  organ  may  play  a  part  in  the  neutralization  of  toxins. 
As  demonstrated  by  Roy,  the  organ  exhibits  a  periodic  contraction  and 
expansion  (through  the  agency  of  the  plain  muscle  fibers  present  in  its 
capsule).  By  this  means  the  contents  of  the  sinuses  undergo  renewal. 
Owing  to  this  and  the  close  connection  with  the  vascular  system,  which  has 
suggested  the  name  "abdominal  heart"  for  the  spleen,  we  can  understand 
readily  how  it  is  that  the  spleen  is  especially  liable  to  be  involved  in 
changes  in  the  blood,  either  from  foreign  substances  reaching  it,  from  the 
action  of  soluble  toxins,  or  from  alterations  in  blood  pressure.  Again, 
as  the  splenic  vein  forms  part  of  the  portal  system,  the  pathological 
conditions  in  the  liver  react  upon  the  spleen,  and  vice  versa. 


CONGENITAL  ANOMALIES. 

The  rarest  anomaly  is  complete  absence  of  the  spleen.  This  is  most 
often  found  in  association  with  other  grave  defects  of  development. 
The  splenic  artery  is  usually  wanting  in  such  cases.  About  thirteen 
cases  are  on  record,2  but  only  one,  that  of  Birch-Hirschfeld,3  is  beyond 
cavil. 

More  often  the  place  of  the  spleen  is  taken  by  scattered  nodules  of 
splenic  tissue  in  various  parts  (splenunculi).  H.  Albrecht4  has  recorded 
a  remarkable  case  in  which  nearly  400  of  these  splenunculi  were 
found  scattered  throughout  the  abdominal  cavity.  Probably  the  most 
common  anomaly  is  the  occurrence  of  accessory  spleens.  These  occur 
in  our  experience  in  11  per  cent,  of  all  autopsies.  They  vary  in  number 
from  one  to  twenty,  and  may  be  scarcely  recognizable  or  as  large  as  a 
walnut.  Care  should  be  taken  not  to  mistake  hemolymph-nodes  for 
accessory  spleens.  The  latter  are  found  usually  on  the  under  side  of 
the  gastrosplenic  omentum,  the  mesentery,  the  wall  of  the  intestine,  and 

1  Meyer  u.  Heineke,  Verhandl.  d.  dent.  Path.  Gesellschaft,  9  :  1905  :  224;  Morris, 
Johns  Hopkins  Hospital  Bull,  18:  1907:  200. 

2  See  Hodenpyl,  Med.  Record,  54:1898:695. 

3  Defect  der  Milz  bei  einem  Neugeborenen,  Arch.  f.  Heilk.,  Leipzig,  12:  1871:  190. 

4  Ziegler's  Beitrage,  20:1896:513 


INFARCTION  223 

in  the  tail  of  the  pancreas.  Irregularities  in  shape  and  position  are 
rather  common.  Rolleston  has  recorded  a  remarkable  case  in  which 
there  was  a  tongue-like  process  extending  into  the  scrotum. 

Congenital  dislocation  of  the  spleen  has  been  observed  in  cases  of 
umbilical  and  diaphragmatic  hernia.  The  so-called  "wandering  spleen" 
is  in  part  due  to  congenital  laxity  of  the  tissues  at  the  hilus,  but  perhaps 
more  important  is  increased  weight  of  the  spleen,  such  as  may  be 
brought  about  by  malaria.  In  these  cases  the  spleen  may  be  found  in 
the  pelvis.  In  transposition  of  the  viscera,  the  spleen  may  be  found  on 
the  right  side. 

Alterations  in  Position. — The  spleen  may  be  found  in  almost  any 
part  of  the  abdominal  cavity.  This  may  be  due  in  part  to  congenital 
laxness  of  its  attachments,  but  increase  in  the  weight  of  the  organ  is 
the  chief  cause.  In  other  cases  the  organ  may  be  dragged  down,  as  in 
gastroptosis  and  enteroptosis.  Occasionally,  inflammatory  adhesions 
fix  it  in  the  abnormal  position. 


CIRCULATORY  DISTURBANCES. 

Anemia. — Anemia  of  the  spleen  is  found  in  all  forms  of  generalized 
anemia  and  in  compression  of  the  organ  from  any  cause.  The  organ  is 
small,  the  capsule  somewhat  wrinkled,  and  on  section  the  tissue  is 
pale,  grayish-red,  and  less  pulpy  than  usual,  while  the  trabeculse  stand 
out  prominently. 

Hyperemia. — Active  or  congestive  hyperemia  is  found  in  cases  of 
infection  or  intoxication,  and  is  closely  allied  to  acute  inflammation, 
of  which  it  forms  the  first  stage.  It  leads  to  rapid  swelling  of  the  organ, 
with  distension  of  the  bloodvessels,  so  that  the  part  contains  more  blood 
than  normal.  The  spleen  is  usually  greatly  enlarged,  the  capsule  is 
tense  and  thin,  and  through  it  can  be  seen  the  congested  pulp.  The 
swelling  may  be  so  great  that  spontaneous  rupture  of  the  organ  may 
occur.  On  section,  it  is  soft,  pulpy,  and  intensely  reddened,  so  that  the 
corpuscles  and  trabeculse  are  quite  indistinct. 

Passive  Hyperemia. — Passive  hyperemia  is  a  common  condition  and 
is  brought  about  by  obstruction  to  the  free  exit  of  blood  from  the  spleen, 
such  as  may  result  from  valvular  disease  of  the  heart,  defective  pulmonary 
circulation,  cirrhosis  of  the  liver,  and  thrombosis  of  the  splenic  vein. 
The  affected  organ  is  somewhat  enlarged,  the  capsule  tense,  opaque, 
and  sometimes  thickened.  The  consistency  is  also  increased.  On 
section,  the  tissue  is,  as  a  rule,  dark  purple-red  in  color,  firm  and  dry,  and 
the  trabeculse  and  bloodvessels  are  thickened. 

Microscopically,  the  veins  are  congested,  even  cavernous,  and  the  pulp 
is  greatly  suffused  with. blood,  and  may  contain  blood  pigment.  The 
trabeculse  and  reticulum  generally  are  thickened,  and  the  vessels  may 
show  endarteritis.  This  condition  is  called  cyanotic  induration. 

Infarction. — This  is  a  fairly  common  event  in  the  spleen,  owing 
to  the  fact  that  the  splenic  artery  is  a  large  vessel  that  breaks  up  quickly 


224  THE  SPLEEN 

into  branches  forming  relatively  small  end-arteries.  The  usual  cause  of 
embolism  is  the  dislodgment  of  fine  particles  from  the  heart-valves  or 
aorta.  An  occasional  cause  is  thrombosis  of  the  splenic  artery,  more 
rarely  of  the  splenic  vein.  Osier  has  recorded  infarction  in  a  movable 
spleen  due  to  twisting  of  the  pedicle.  Embolic  infarcts  are  single  or 
multiple,  and  are  of  small  size,  or,  again,  may  involve  the  whole  thick- 
ness of  the  organ.  The  smaller  infarcts  are  more  or  less  wedge-shaped, 
with  the  apex  toward  the  hilus.  Recent  infarcts  form  a  firm  prominence 

FIG.  56 


White  infarct  of  the   spleen.      Section  was    made  through  the   infarct,  the  organ   being  thereby 
laid  open.      (From  the  Pathological  Laboratory  of  McGill  University.) 

on  the  surface  of  the  organ.  On  section,  the  affected  area  is  ivory-white 
in  color,  less  commonly  somewhat  hemorrhagic,  and  sharply  defined 
from  the  rest  of  the  splenic  tissue.  Mixed  forms  also  occur,  where  the 
centre  is  pale  and  the  periphery  infiltrated  with  blood.  According  to 
Orth,  all  infarcts  at  first  are  white,  but  later  some  may  become  hemor- 
rhagic. Beattie  and  Dixon  claim  on  the  other  hand  that  all  infarcts 
exhibit  a  preliminary  congestive  stage,  which  may  pass  on  either  to  the 
white  or  the  hemorrhagic  stage. 

Microscopically,  the  anemic  region  shows  coagulation-necrosis,  the 
cells  are  swollen  and  granular  and  their  nuclei  indistinct.  In  advanced 
conditions,  the  nuclei  have  disappeared  and  the  tissue  is  converted  into 
a  granular,  slightly  refractile,  fibrinoid  material.  In  parts,  especially 
about  the  periphery,  the  cells  show  a  certain  amount  of  fatty  degenera- 
tion. As  a  rule,  surrounding  the  infarct  there  is  a  zone  of  reactive 
inflammation.  As  the  infarct  ages,  the  necrotic  material  is  gradually 


SPLENITIS  225 

softened  and  absorbed,  while  vascular  granulation  tissue  develops  at 
th'^  periphery  and  gradually  substitutes  the  damaged  area,  until  finally 
only  a  pigmented  scar  remains.  In  the  red  infarct  the  vessels  are  greatly 
distended  and  the  pulp  is  densely  infiltrated  with  blood.  When  the 
emboli  are  infective  the  infarcts  rapidly  soften  and  are  converted  into 
abscesses.  Rarely,  these  heal  and  may  cause  local  adhesive  perisplenitis. 
Or,  again,  they  may  burst  into  the  peritoneal  cavity  and  set  up  a  fatal 
peritonitis. 

Hemorrhage. — Hemorrhage  into  the  spleen  is  of  frequent  occurrence, 
but  it  is  often  difficult  to  be  sure  of  the  condition,  since  the  spleen  pulp 
normally  contains  such  great  numbers  of  red  cells.  Apart  from  traumatism 
and  infarction,  the  usual  causes  are  malaria,  typhoid,  variola,  leukemia, 
and  purpura  haemorrhagica;  in  fact,  any  condition  that  may  set  up  acute 
splenitis.  The  hemorrhages  are  recognized  as  dark  red  spots  or  streaks 
in  the  parenchyma. 

INFLAMMATIONS. 

Splenitis. — Acute  Splenitis. — Acute  splenitis  is  closely  associated 
with  congestive  hyperemia,  and,  indeed,  can  hardly  be  considered  apart 
from  it.  The  condition  arises  in  the  course  of  a  variety  of  infective 
fevers,  such  as  typhoid,  pneumonia,  scarlatina,  diphtheria,  septicemia, 
malaria,  and  relapsing  fever,  and  is  characterized  in  the  main  by  hyper- 
plasia.  The  spleen  is  enlarged,  sometimes  to  several  times  its  usual  size, 
soft,  and  the  capsule  is  tense.  On  section,  the  organ  in  the  earlier  stages 
is  intensely  hyperemic  and  firm.  Later,  it  becomes  pulpy,  almost 
diffluent,  and  of  a  grayish-red  color.  The  follicles  are  usually  not  prom- 
inent, but  may  be  noticeably  enlarged  in  scarlatina.  Not  infrequently 
on  the  capsule  is  a  fibrinous  or  fibrinopurulent  exudation  (perisplenitis). 

Microscopically,  there  is  a  numerical  increase  chiefly  of  the  lymphoid 
cells,  many  of  which  show  fatty  change,  while  large  multinucleated  cells 
are  to  be  seen  containing  pigment,  broken-down  blood-corpuscles,  and 
lymphoid  cells.  The  vascular  sinuses  and  lymphatics  are  dilated  and 
their  endothelium  shows  signs  of  proliferation  and  fatty  degeneration. 
Small  hemorrhages  into  the  splenic  substance  are  common.  In  certain 
cases  the  follicles  also  participate  in  the  acute  hyperplasia.  In  connec- 
tion with  scarlatina,  Klein1  has  described  hyaline  degeneration  of  the 
smaller  vessels. 

Besides  the  appearances  just  described,  there  are  certain  others  that 
deserve  mention.  Rarely,  the  follicles  are  found  to  be  of  a  yellow  color 
in  the  centre  and  softened,  having  undergone,  not  suppurative,  but  rather 
colliquative,  necrosis.  This  has  been  described  particularly  in  connec- 
tion with  typhus  exanthematicus  and  relapsing  fever.  More  common 
are  areas  of  necrosis  which  may  be  merely  of  microscopic  size,  but 
which  occasionally  reach  that  of  a  cherry.  These  are  found  chiefly 
in  typhoid,  diphtheria,  scarlatina,  and  relapsing  fever,  either  in  the  pulp 
or  follicles.  The  affected  cells  are  found  in  all  stages  of  disintegration, 

1  Trans.  Path.  Soc.  Lond.,  28:1877:430. 
15 


226  THE  SPLEEN 

so  that  in  the  affected  area  we  find  fragments  of  nuclei  and  cellular 
debris.  About  the  periphery  is  an  extravasation  of  leukocytes.  The 
condition  is  due,  in  part,  to  the  direct  action  of  bacterial  toxins,  but 
chiefly  to  anemia  brought  about  by  the  obstruction  of  the  bloodvessels 
and  lymphatics  through  cell  proliferation  and  the  accumulation  of  debris. 
The  larger  necrotic  foci  may  soften  or  suppurate  and  may  burst  through 
the  capsule,  thus  setting  up  purulent  peritonitis. 

The  results  of  acute  splenitis  are  various.  Commonly,  the  process 
subsides,  the  hyperemia  gradually  disappears,  the  hyperplastic  cells  are 
disintegrated  and  absorbed,  and  complete  resolution  takes  place.  Rarely, 
the  simple  splenitis  may  become  suppurative,  or  the  process  may  become 
chronic. 

Suppurative  Splenitis. — In  this  form  the  whole  spleen  may  be  diffusely 
infiltrated  with  pus,  but  it  is  more  common  to  find  multiple  abscesses. 
The  condition  is  usually  due  to  a  hematogenic  infection  with  pyogenic 
microorganisms,  such  as  occurs  in  ulcerative  endocarditis  and  septicemia. 
Here  the  process  is  frequently  combined  with  hemorrhagic  infarction. 
In  other  cases  the  inflammation  may  extend  from  "neighboring  parts, 
as,  for  instance,  in  ulcerating  carcinoma  of  the  stomach,  abscess  of  the 
pancreas,  perinephritic  abscess,  and  purulent  peritonitis.  The  abscesses 
may  rupture  into  the  peritoneal  cavity,  the  left  pleura,  left  lung,  stomach, 
or  intestines.  Should  the  patient  live,  the  abscesses,  when  small,  may  be 
absorbed  and  become  fibroid;  the  larger  ones  finally  become  enclosed 
in  a  fibrous  capsule,  while  the  contents  become  inspissated  or  calcareous. 
Calling  to  mind  the  relative  frequency  of  pyemic  and  bacteriemic  states, 
the  rarity  of  suppurative  disturbances  in  the  spleen  is  very  remarkable. 
It  can  only  be  explained  on  the  assumption  of  strong  bactericidal  prop- 
erties on  the  part  of  this  organ. 

Chronic  Indurative  Splenitis. — This  may  be  the  result  of  acute  splenitis 
or  may  be  an  insidious  process  from  the  first.  It  is  found  chiefly  in 
malaria,  cirrhosis  of  the  liver,  rickets,  and  late  syphilis.  In  malaria 
the  process  may,  for  a  time,  run  an  acute  course,  just  as  in  other  infective 
fevers,  but,  as  a  rule,  the  spleen,  in  addition  to  the  signs  of  active  inflam- 
mation, contains  yellow,  brownish,  or  black  pigment  in  the  pulp  and  in 
the  centre  of  the  follicles.  In  relapsing  cases  and  chronic  malarial 
cachexia,  the  spleen  becomes  permanently  enlarged  and  firm  in  consist- 
ence, owing  to  the  overgrowth  of  fibrous  connective  tissue  (ague  cake). 
The  capsule  is  usually  thickened  and  the  organ  is  more  or  less  firmly 
attached  to  the  diaphragm.  The  enlargement  may  be  so  great  that  the 
spleen  may  become  dislocated,  or  grow  until  it  reaches  the  pelvis.  In  the 
malarial  spleen  it  is  not  uncommon  to  find  amyloid  change.  The  over- 
growth of  fibrous  tissue  is  permanent,  but  the  malarial  spleen  may 
occasionally  diminish  in  size,  owing  to  the  destruction  of  the  lymphoid 
elements. 

Another  form  of  splenomegaly  that  has  been  confounded  with  malaria, 
associated  with  febrile  disturbance,  is  found  in  kala-azar.  Here  the 
parasites  are  enclosed  in  the  smaller  endothelial  cells.  The  spleen  is 
enlarged  and  firm,  but  friable  and  not  sclerotic. 


TUBERCULOSIS  227 

In  cirrhosis  of  the  liver  the  spleen  is  often  noticeably  enlarged  and 
this  both  in  the  portal  and  the  biliary  forms  (Hanot's  type),  the  enlarge- 
ment being  more  marked  in  the  former.  It  used  to  be  thought  that 
this  was  due  to  stasis  in  the  portal  circulation,  but  this  is  undoubtedly 
not  the  whole  explanation,  for  the  spleen  may  be  enlarged  and  hyper- 
plastic  in  cases  where  portal  obstruction  is  not  present,  while  its  substance 
may  be  pale  red  and  soft,  quite  unlike  the  hard  cyanotic  spleen  of  passive 
congestion.  It  is  more  probable  that  infective  microorganisms,  or  circu- 
lating toxins,  are  at  work  here  as  well.  The  hyperplasia  affects  the  pulp, 
which  contains  numerous  red  and  white  cells  without  any  special  change 
in  structure.  Later,  the  arrangement  of  the  pulp  becomes  confused 
and  there  is  a  cellular  transformation  of  the  reticular  connective  tissue. 

FIG.  57 


Caseous  tuberculosis  of  the  spleen  (miliary  type).      Leitz  obj.  No.  7,  without  ocular.      (From 
the  collection  of  Dr.  A.  G.  Nicholls.) 

Tuberculosis. — This  is  rarely  primary  in  the  spleen.  Acute  miliary 
tuberculosis  occurs  in  systemic  dissemination  of  the  tuberculous  infec- 
tion. The  spleen  is  enlarged,  the  parenchyma  on  section  is  soft,  swollen, 
and  dark  red  in  color,  and  is  universally  scattered  over  with  minute 
round  grayish  tubercles. 

Microscopically  the  miliary  nodules  may  be  purely  lymphoid  in  type, 
but  more  commonly  endothelioid,  and  giant  cells  are  present  as  well. 
Slight  caseation  is  often  observed. 

In  another  form  the  tubercles  may  be  fewer,  rarer,  and  caseous. 
These  are  found  in  the  Malpighian  bodies  and  about  the  bloodvessels. 
There  is  apt  to  bp  jppfe  or  less  adhesive  perisplenitis. 


228  THE  SPLEEN 

Syphilis. — Miliary  gummas  are  rare,  but  are  met  with  in  both 
inherited  and  acquired  syphilis.  Large  gummas,  varying  in  size  from 
that  of  a  pea  to  that  of  an  egg,  have  been  described. 

Diffuse  hyperplasia  is  by  far  the  most  common  and  important  syphilitic 
manifestation.  The  condition  may  be  acute,  affecting  the  lymphoid  cells, 
or  may  lead  to  chronic  thickening  of  the  reticulum  and  trabeculae.  In 
long-standing  disease  amyloid  change  is  also  met  with.  In  some  cases 
of  old  lues  the  spleen  contains  abundant  pigment. 

Leprosy. — The  spleen  is  enlarged  and  presents  numerous  granulomas, 
which  contain  the  specific  bacillus. 

Glanders. — The  organ  is  enlarged,  soft,  greatly  reddened,  and 
studded  with  multiple  abscesses,  containing  thick  yellow,  rather  viscid 
pus,  in  which  the  specific  bacillus  may  be  demonstrated.  In  chronic 
cases  amyloid  deposit  may  also  be  observed. 

Actinomycosis. — This  is  rare  in  the  spleen  and  generally  a  secondary 
manifestation.  It  leads  to  the  formation  of  isolated  abscesses  filled  with 
glairy  pus,  in  which  can  be  recognized  the  actinomycosis  grains. 

Parasites. — Echinococcus,  Cysticercus,  and  the  Pentastomum  den- 
ticulatum  have  been  met  with. 


RETROGRESSIVE   METAMORPHOSES. 

Atrophy. — Atrophy  of  the  spleen  is  found  in  old  people  and  in  those 
suffering  from  long-standing  disease.  It  may  follow  acute  splenitis 
and  may  be  produced  by  general  anemia  or  by  any  obstruction  of  the 
splenic  artery  leading  to  local  anemia.  The  spleen  is  small,  firm,  and 
the  capsule  wrinkled.  On  section,  the  pulp  is  noticeably  diminished, 
the  trabeculse  are  prominent,  and  the  organ  contains  but  little  blood. 

Hyaline  Degeneration. — Hyaline  degeneration  has  been  observed  in 
the  vessels  and  reticulum.  It  is  sometimes  superadded  to  amyloid  disease. 

Amyloid  Infiltration. — This  is  a  fairly  common  occurrence.  There  are 
two  main  types,  the  "sago"  spleen  and  the  diffuse  amyloid  or  "bacony" 
spleen,  but  these  forms  are  frequently  combined.  In  the  first  type,  the 
spleen  is  not  enlarged  to  any  extent  and  its  consistency  is  but  little  altered. 
On  section,  the  pulp  is  red  and  thickly  studded  with  firm,  translucent, 
gelatinous-looking  bodies,  varying  in  size  from  a  millet  seed  to  a  pin's 
head  or  somewhat  larger.  These  are  the  amyloid  Malpighian  bodies, 
and  they  bear  a  close  resemblance  to  grains  of  boiled  sago,  whence  the 
name.  Microscopically,  the  amyloid  material  is  laid  down  in  the  walls 
of  the  smaller  arterioles  and  capillaries,  chiefly  in  the  intermediate  zone 
of  the  Malpighian  bodies,  so  that  the  appearance  presented  is  that  of  a 
ring  of  amyloid  material.  In  more  advanced  conditions,  the  whole 
corpuscle  may  be  thus  transformed.  The  vessels  are  of  course  thickened 
and  the  lymphoid  elements  tend  to  atrophy.  It  may  happen,  however, 
that  the  vessels  are  not  particularly  involved. 

In  the  diffuse  form,  which  is  less  common,  the  organ  is  moderately 
enlarged,  the  capsule  distended,  and  the  edges  rounded.  The  tissue 


REGENERATIVE  HYPERPLASIA  229 

is  firm  like  rubber  and  is  translucent  when  a  thin  section  is  held  up 
to  the  light.  On  section,  by  reflected  light,  the  surface  is  of  a  peculiar 
semitransparent,  reddish  color,  as  if  covered  with  a  thin  coating  of 
gelatin,  whence  it  has  been  compared  to  ham  or  bacon  (Speckmilz). 

Microscopically,  the  amyloid  change  affects  the  walls  of  the  venous 
radicles  and  the  reticulum  of  the  pulp.  The  endothelial  lining  of  the 
vessels  is  unaffected  and  the  lymphoid  cells  show  merely  the  effects 
of  pressure.  The  Malpighian  bodies,  as  a  rule,  escape  in  this  form. 

The  causes  of  amyloid  disease  are  the  usual  ones,  chronic  wasting 
affections  and  cachexias,  osteomyelitis,  suppuration,  septicemia,  tuber- 
culosis, syphilis,  intermittent  fever,  chronic  glanders,  carcinoma,  and 
the  like.  Associated  with  the  disease,  and  depending  equally  upon 
the  initial  cause,  are  pigmentation,  infarction,  and  fibrous  hyperplasia. 

Pigmentary  Infiltration. — This  is  met  with  in  cases  of  passive 
congestion,  the  cachexias,  leukemia,  and  all  diseases  associated  with 
blood  destruction.  It  is  well  marked  in  malaria  and  in  hemochromatosis ; 
rarely,  particles  of  coal  dust  may  reach  the  spleen.  According  to  its 
nature,  the  pigment  is  golden,  reddish,  reddish-brown,  through  all 
shades  of  brown  to  black.  It  is  laid  down  chiefly  about  the  bloodvessels 
in  the  trabeculse,  but  may  lie  free  or  within  phagocytic  cells. 

Necrosis. — Necrosis  may  involve  the  spleen  in  cases  in  which  the 
blood  supply  is  suddenly  cut  off. 


PROGRESSIVE  METAMORPHOSES. 

Regenerative  Hyperplasia. — Regenerative  hyperplasia  has  been 
observed  in  cases  where  a  portion  of  the  spleen  has  been  destroyed. 
Both  the  Malpighian  bodies  and  the  pulp  participate  in  the  process. 
Nodular  hyperplasia,  the  so-called  "splenadenoma,"  which  perhaps 
ought  to  be  classed  with  the  tumors,  is  met  with  in  dogs  and  has  occa- 
sionally been  seen  in  man.  It  forms  a  nodular  mass  the  size  of  a  cherry 
or  less,  of  a  bright  grayish-red  color,  fairly  well  defined  from  the  rest  of 
the  spleen.  It  is  occasionally  surrounded  by  a  fibrous  capsule. 

Microscopically,  there  is  hyperplasia  not  only  of  the  lymphoid  cells, 
but  also  of  the  fibrous  stroma. 

As  in  the  case  of  the  lymph-nodes,  it  is  difficult  to  draw  the  line  between 
inflammatory  hyperplasia  of  the  spleen  and  tumor  formation,  and  there 
are  certain  enlargements  of  the  organ,  notably  in  leukemia,  pseudo- 
leukemia,  and  Hodgkin's  disease,  which  belong  to  this  debatable  ground. 

The  changes  found  in  the  spleen  in  leukemia  and  pseudoleukemia  are 
practically  identical.  The  organ  may  be  greatly  enlarged,  so  that  it 
reaches  even  into  the  pelvis,  and  may  weigh  as  much  as  3  kg.  or  more. 
The  capsule  is  thickened  in  places  and  covered  with  numerous  shaggy 
adhesions,  binding  it  to  the  diaphragm  and  the  neighboring  structures. 
Two  main  forms  are  recognizable:  the  first  in  which  the  hyperplasia  is 
confined  to  the  spleen  pulp,  and  the  second  where  the  Malpighian 
corpuscles  are  involved.  In  the  first,  the  parenchyma  is  soft  and  bright 


230  THE  SPLEEN 

red  or  reddish-gray,  while  the  corpuscles  show  little  or  no  change. 
Hemorrhagic  infarcts  of  considerable  size  are  not  uncommon.  Micro- 
scopically, one  sees  hyperemia  with  hyperplasia  both  of  thel  ymphoid 
cells  and  the  connective-tissue  stroma.  In  the  second  variety,  the  Mal- 
pighian  bodies  are  greatly  enlarged,  forming  whitish,  tumor-like  masses 
arranged  in  nodules  or  bands.  They  vary  in  size  from  that  of  a  pea 

FIG.  58. 


The  spleen  in  three  different  conditions,  to  illustrate  variations  in  size.  From  left  to  right. 
a,  normal  spleen;  b,  typhoidal  spleen;  c,  leukemic  spleen.  (From  the  Pathological  Museum  of 
McGill  University.) 

to  that  of  a  walnut.  In  long-standing  cases,  owing  to  the  overgrowth  of 
fibrous  tissue,  the  organ  becomes  firm  and  the  color  gray,  often  streaked 
with  brown  or  brownish-black  pigment.  In  these  diseases  the  spleen 
may  be  the  first  and  only  organ  to  be  affected,  but,  as  a  rule,  analogous 
changes  are  to  be  found  in  the  lymph-nodes  and  bone-marrow. 

Tumors. — Tumors  do  not  commonly  involve  the  spleen.  Among 
the  primary  benign  growths  may  be  mentioned  fibroma  and  lymphangi- 
oma.  Langhans1  has  recorded  a  curious  case  of  angioma  cavernosum 
(angiosarcoma  ?)  in  which  small  secondary  masses  were  present  in  the  liver. 
Dermoid  cysts  are  rare.  Simple  cysts,  containing  clear  or  blood-stained 
fluid,  may  arise  from  degeneration  of  the  parenchyma,  the  dilatation  of 
the  lymph-spaces  (Fink  and  Aschoff),  or  from  the  inclusion  of  peritoneal 
endothelium  (Rengghi2). 

Sarcoma.3— -Sarcoma  occurs  occasionally  as  a  primary  growth,  but 
is  usually  secondary.  A  form  arising  from  the  endothelial  plates  has 

1  Virch.  Archiv,  85:  1879-  273;  see  also  Thiele,  Virch.  Arch.,  178:  1904. 

2  Inaug.  Diss  Zurich,  1894. 

3  See  Jepson  and  Albert,  Annals  of  Surgery,  40 : 1904 : 80. 


THE  BONE-MARROW  231 

been  described.1     Melanotic  sarcoma  not  uncommonly  leads  to  metastases 
in  the  spleen,  which,  however,  are  not  necessarily  pigmented. 

Carcinoma. — Carcinoma  is  invariably  secondary  and  arises  either  by 
metastasis  or  direct  extension  from  the  stomach. 


THE  BONE-MARROW. 

Anatomically  speaking,  the  marrow  forms  an  integral  portion  of  the 
bones,  so  that  it  is  difficult  to  conceive  of  it  as  detached  from  this  associa- 
tion. Nevertheless,  the  marrow  can  hardly  be  regarded  as  forming  a 
part  of  the  supporting  structure  of  the  body,  except  in  so  far  as  it  contrib- 
utes to  the  nourishment  of  bone.  Histologically,  its  structure  is  closely 
akin  to  that  of  the  spleen  and  lymph-nodes,  and  as  its  main  function 
undoubtedly  is  to  produce  certain  types  of  blood-cells,  it  seems  more 
logical  to  consider  it  in  its  physiological  rather  than  its  anatomical 
relationships. 

The  bone-marrow  is  in  its  young  state  soft,  cellular,  and  well  supplied 
with  bloodvessels.  The  stroma  is  composed  of  branching  connective- 
tissue  cells  forming  a  fine  reticulum,  in  the  meshes  of  which  lie  a  great 
variety  of  cells,  namely,  red  blood-cells,  hematoblasts,  lymphocytes, 
eosinophiles,  pigmented  cells,  and  multinucleated  giant  cells  (myeloplaxes ; 
megacaryocytes).  The  bloodvessels  are  wide,  thin-walled,  and  arranged  so 
that  collapse  cannot  occur.  On  account  of  its  appearance  and  structure, 
this  form  is  called  the  red  or  lymphoid  bone-marrow.  Red  bone-marrow 
is  present  in  all  the  bones  at  birth,  but  gradually  changes  its  character, 
the  cells  of  the  supporting  stroma  being  transformed  into  fat  (lipoid 
atrophy).  The  color  thus  changes  from  red  to  yellow,  hence  the  term 
"yellow"  bone-marrow.  At  about  the  age  of  puberty  all  the  long  bones 
contain  this  yellow  marrow.  The  red  marrow,  however,  persists  into 
advanced  life  in  the  sternum,  ribs,  vertebrae,  and  skull.  With  old  age  the 
yellow  fatty  marrow  exhibits  serous  atrophy. 

The  function  of  the  bone-marrow  is  to  produce  blood-corpuscles  and 
to  absorb  or  otherwise  render  harmless  foreign  substances  in  the  blood. 
The  giant  cells  just  referred  to  can  also  on  occasion  act  as  osteoclasts. 


CIRCULATORY  DISTURBANCES. 

Anemia. — Anemia  occurs,  but  is  so  closely  associated  with  regenera- 
tion of  the  medulla  that  it  is  better  dealt  with  under  the  Progressive 
Metamorphoses. 

Hyperemia. — In  hyperemia  the  yellow  marrow  assumes  a  reddish- 
yellow  color. 

Hemorrhage. — Hemorrhage  into  the  medulla  occurs  from  trauma- 
tism  and  from  obstruction  to  the  free  exit  of  blood  from  the  part. 

1  Bunting,  Univ.  of  Penna.  Med.  Bull,  16: 1903: 188. 


232  THE  BLOOD-FORMING  ORGANS 


INFLAMMATIONS. 

Inflammatory  infections  so  commonly  involve  the  bone  as  well,  that 
they  are  better  considered  under  affections  of  the  bones  (see  p.  1019). 
The  usual  forms  are  osteomyelitis,  met  astatic  abscesses,  tuberculosis, 
syphilis,  and  leprosy.  Litten  and  Orth1  have  pointed  out  that  in  many 
infectious  diseases  associated  with  acute  splenic  tumors,  such  as  sepsis, 
typhoid  fever,  fibrinous  pneumonia,  an  analogous  hyperplasia  is  present 
in  the  bone-marrow.  This  has  been  confirmed  for  acute  endocarditis 
by  Ponfick,  and  for  variola  by  Golgi.  In  addition  to  hyperplasia,  fatty 
degeneration  of  the  vessels  has  been  noted,  as  well  as  the  presence  of 
numerous  cells  containing  broken-down  blood-corpuscles  and  pigment. 

Metastatic  Abscesses. — Metastatic  abscesses  are  found  in  the 
marrow  not  uncommonly  in  septicemia  and  certain  other  infectious 
fevers,  notably  variola. 

Tuberculosis. — Tuberculosis  arises  as  a  hematogenic  infection  in 
most 'cases,  and  usually  begins  in  the  cancellous  part  of  the  bone.  It 
occurs  also  in  the  miliary  form,  in  general  systemic  tuberculous  infection. 

Syphilis. — Syphilis  takes  the  form  of  gummas.  It  is  rare  in  the 
marrow. 

Leprosy. — Granulomas  containing  the  characteristic  bacillus  have 
been  found. 

RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  bone-marrow  occurs  in  old  age,  in  chronic 
pulmonary  emphysema,  chronic  pulmonary  tuberculosis,  chronic  nephritis, 
and  in  death  from  starvation.  The  fat  cells  are  gradually  absorbed 
and  the  tissue  shrinks.  Its  place  is  taken  by  a  mucinous  fluid,  so  that 
the  marrow  becomes  gelatinous,  translucent,  and  somewhat  brownish 
(Gallertmark).  The  condition  is  identical  with  the  serous  atrophy  of 
fat  that  occurs  elsewhere. 

Fatty  Degeneration. — Fatty  degeneration  of  the  capillaries  and 
medullary  cells  is  met  with  in  certain  of  the  infectious  fevers,  notably 
typhoid,  typhus,  and  relapsing  fever. 

Focal  Necroses. — Focal  necroses  may  occur  under  similar  circum- 
stances. 

PROGRESSIVE  METAMORPHOSES. 

Hyperplasia. — Hyperplasia  of  the  fatty  tissue  of  the  marrow  is 
met  with  in  the  generalized  atrophy  of  the  skeleton  that  occurs  in  old 
age.  Not  only  are  the  medullary  canals  enlarged,  but  the  bone  itself 
becomes  rarefied,  cancellous,  and  infiltrated  with  fat.  Not  infrequently 

1  Ueber  Veranderungen  des  Marks  in  Rohrenknochen  unter  verschiedenen  patho- 
logischen  Verhaltnissen,  Berl.  klin.  Woch.,  14: 1877:  743. 


TUMORS  233 

the  fat  gradually  disappears  and  there  is  a  hyperplasia  of  the  marrow, 
so  that  it  reverts  to  the  more  primitive  form  of  red  or  lymphoid  marrow. 
This  occurs  in  anemia,  leukemia,  chronic  pulmonary  tuberculosis,  sup- 
purative  bone  disease,  and  cancerous  cachexia.  It  has  also  been  found 
in  many  of  the  infectious  diseases,  such  as  typhoid,  pneumonia,  septi- 
cemia,  acute  endocarditis,  and  variola,  also  in  cases  where  death  has 
occurred  after  prolonged  illness.  The  process  begins  first  in  the  long 
bones  and  involves  the  epiphyses  of  the  upper  part  of  the  bones,  grad- 
ually spreading  to  the  whole  medulla.  The  fatty  tissue  is  gradually 
reduced  and  replaced  by  lymphoid  cells  until  the  medulla  assumes  a 
reddish-gray  or  dark  red  color  and,  in  severe  cases,  the  appearance  of 
raspberry  jelly. 

Microscopically,  there  is  a  great  increase  in  the  number  of  all  the 
marrow  cells,  but  particularly  the  nucleated  red  corpuscles,  suggesting 
that  the  process  is  a  regenerative  one.  The  lymphoid  cells  are  often 
fatty,  and  there  are  numerous  phagocytic  and  pigment-bearing  cells. 
Besides  these,  there  may  be  seen  numbers  of  small  octahedral  crystals, 
the  so-called  Charcot-Neumann  crystals. 

Neumann1  was  the  first  to  draw  attention  to  that  form  of  leukemia 
in  which  the  bone-marrow  was  first  and  chiefly  affected,  hence  called 
medullary  or  myelogenic  leukemia.  The  changes  in  the  marrow  have 
been  regarded  as  the  cause  of  the  altered  blood  condition.  Pure 
myelogenic  leukemia,  that  is,  leukemia  apart  from  marked  changes  in  the 
spleen  and  lymph-glands,  is  certainly  rare,  but  one  instance  has  come 
under  our  notice  at  the  Royal  Victoria  Hospital.  This  occurred  in  a  man, 
aged  sixty-eight  years.  The  bone-marrow  presented  a  marked  rasp- 
berry-jelly appearance,  while  the  spleen  was  small.  The  retroperitoneal 
glands  were  somewhat  enlarged,  but  not  more  so  than  in  many  cases 
of  other  forms  of  disease.  The  bone-marrow  is  not  always  soft,  juicy, 
and  gelatinous  in  myelogenic  leukemia,  but,  as  Ponfick2  pointed  out, 
may  assume  a  grayish-yellow  or  even  green  color,  owing  to  the  great 
increase  in  the  numbers  of  the  colorless  cells  (pyoid  marrow).  Not 
only  this,  but  the  marrow  is  anemic  from  compression  of  the  vessels  and 
inflammation  of  their  walls.  Red  blood-cells  normal  and  nucleated, 
fattily  degenerated  leukocytes,  and  Charcot-Neumann  crystals  are  here 
found  in  varying  amounts.  The  two  forms,  therefore,  seem  to  be  but 
variations  of  the  same  condition. 

In  pseudoleukemia  the  changes  in  the  bone-marrow  are  also  variable. 
At  one  time  there  is  hyperplasia  such  as  is  found  in  anemias,  at  another 
lymphomatous  nodules. 

Tumors. — Tumors  of  the  medulla  may  arise  from  the  cellular 
elements  or  from  the  fibrous  stroma.  Many  of  these  lead  to  rarefaction 
of  the  bone  through  pressure  or  the  action  of  osteoclasts.  With  this 
there  is  frequently  a  new  formation  of  bone  from  the  periosteum.  This 
is  particularly  well  seen  in  the  giant-celled  or  "myeloid"  sarcoma,  or  more 
accurately  myeloma  (see  vol.  i,  676),  which  may  start  from  the  medulla 

1  Berl.  klin.  Woch.,  14:1877:685.  2  Virch.  Arch.,  56:1872:550. 


234 


THE  BONE-MARROW 


and  form  a  large  tumor.  On  the  surface  of  the  growth  can  be  felt  thin 
plates  of  bone  that  give  on  pressure  a  peculiar  sensation  resembling 
the  crackling  of  an  egg  shell.  There  is  in  tumors  of  this  kind  a  tendency 
to  develop  bone  in  their  interior.  Among  the  malignant  growths,  besides 
the  sarcomas,  carcinomas  are  sometimes  met  with. 

Among  benign  growths  may  be  mentioned  fibroma,  chondroma,  myx- 
oma,  and  fibromyxoma.  Virchow  has  described  a  myelogenous  angioma 
of  the  vertebrae. 

FIG.  59 


Sarcoma  of  the  shaft  of  the  humerus.       (From  the  Pathological  Museum  of  McGill  University.) 

Of  these  various  tumors,  sarcomas  are  by  far  the  most  common.  Several 
forms  are  described.  The  most  usual  site  for  their  development  is  in 
the  maxillae  and  the  epiphyses  of  the  long  bones,  especially  the  humerus 
and  tibia.  They  may,  however,  start  in  the  diaphysis.  In  the  early 
stages  they  produce  a  gradual  caries  of  the  bone,  so  that  spontaneous 
fractures  are  not  rare,  and  eventually  lead  to  great  expansion  and  rare- 
faction of  the  bone.  The  softer  growths  are  round-celled,  while  the 
firmer  are  spindle-celled,  but  mixed  forms  occur.  Of  the  mixed  type, 
the  most  interesting  is  the  giant-celled  myeloma  (tumeur  a  myeloplaxes 


MYELOMA  235 

of  Xelaton).  Here  in  the  ground  substance  are  round,  spindle,  or 
mixed  cells,  with  a  varying  amount  of  connective  tissue  and  numerous 
large,  multinucleated  cells.  These  tumors  are  often  very  vascular, 
owing  to  the  presence  of  wide  vessels.  These  may  give  way,  so  that 
hemorrhage  and  degeneration  are  not  infrequent  occurrences.  The 
presence  of  so  much  blood  gives  them  a  bright  red  appearance  that  is 
somewhat  characteristic.  They  are  relatively  less  malignant  than  other 
forms  of  sarcomas  in  that  they  do  not  tend  to  form  metastases.  Not 
infrequently,  masses  and  bands  of  osteoid  tissue  or  even  true  bone  are 
formed  (osteoid  sarcoma,  osteosarcoma).  Retrogressive  changes  are 
not  uncommon  in  this  form  of  tumor,  and  we  meet  with  fatty  degeneration 
hemorrhage,  pigmentation,  liquefaction,  and  cyst  formation.  Occasion- 
ally, the  greater  portion  of  the  tumor  may  be  destroyed.  Another  form 
is  the  alveolar  sarcoma,  in  which  the  stroma  is  arranged  in  alveoli  contain- 
ing nests  of  relatively  large  cells,  so  that  an  appearance  not  unlike 
carcinoma  is  produced.  An  alveolar  endothelioma  has  been  described 
by  Billroth,1  Hildebrand,2  and  Driessen.3  Some,  at  least,  of  these  and  of 
the  alveolar  sarcomas  of  bone  would  seem  to  be  secondary  adrenal 
growths. 

An  interesting  form  to  which  much  attention  has  been  paid  recently 
is  one  resembling  a  small  round-celled  or  lymphosarcoma,  the  so-called 
myeloma.  The  exact  etiology  of  this  growth  is  yet  a  matter  of  doubt, 
as  is  proved  by  the  great  number  of  names  that  have  been  proposed 
for  it,  namely,  angiosarcoma,  lymphosarcoma,  lymphadenia  ossium 
(Xothnagel4),  general  lymphadenomatosis  of  bone  (Weber),  myeloma 
(v.  Rustizky,  Klebs,  Herrick  and  Hektoen5),  and  plasmoma. 

The  peculiarities  of  this  growth  are  that  it  develops  usually  in  old 
persons,  forms  multiple  apparently  individual  growths  in  different  por- 
tions of  the  bony  skeleton,  and  takes  the  shape  of  nodules  or  diffuse 
infiltrations  of  soft  consistency.  The  skull,  vertebral  column,  and  ribs 
are  the  sites  of  election.  Multiple  spontaneous  fractures  of  the  bone 
frequently  result.  As  a  rule,  but  little  new  bone  is  formed,  yet  excep- 
tions to  this  statement  occur.  Microscopically,  there  is  a  delicate  con- 
nective-tissue stroma  inclosing  masses  of  small  round  cells  and  present- 
ing numerous  large  and  imperfectly  defined  bloodvessels.  Wright6 
has  pointed  out  that  most  of  the  cells  closely  resemble  "plasma"  cells. 
A  curious  point  associated  with  this  growth  is  the  excretion  of  albumose 
in  the  urine.7  As  we  have  pointed  out  in  the  first  volume,  the  multiple 
nature  and  characteristics  of  this  form  of  growth  place  it  among  the 
blastomatoid  formations,  as  a  myelomatosis  rather  than  a  myeloma. 

1  Langenbeck's  Archiv.,  11:1869:244. 

2  Deutsche  Zeitschr.  f.  Chir.,  31:1891:263. 

3  Ziegler's  Beitr.,  12: 1893:65. 

4  Internat.  Beitr.  Festschr.  f.  Virchow,  1891. 

5  Medical  News,  65 : 1894 : 239. 

6  Boston  Soc.  of  Med.  Sci.,  4: 1900: 195. 

7  See  Bence-Jones,  Phil.  Trans.  Royal  Soc.,  1848  :  Part  I  :  55. 


236 


THE  BONE-MARROW 


Carcinoma  of  the  bone-marrow  is  invariably  secondary  and  arises  by 
direct  infection  or  by  metastasis.  Nodular  and  diffuse  forms  are  met 
with. 


FIG.  60 


The  sternum  and  ribs,  showing  the  location  of  myelomatous  growths.      (Herrick  and 

Hektoen's  case.) 


Section  of  myeloma  of  vertebra.      X  600.      (S.  Saltykow.) 


SECTION  II. 
THE    RESPIRATORY  SYSTEM. 


CHAPTER    XL 

THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES. 

IN  discussing  the  broad  pathology,  as  distinct  from  the  pathological 
histology,  of  the  respiratory  system,  we  have  to  keep  constantly  before 
us  the  primary  function  of  that  system,  namely,  the  intake  of  oxygen 
and  the  discharge  of  carbon  dioxide  for  the  benefit  of  the  economy.  All 
other  functions  are  in  comparison  of  minor  importance.  Strictly  speak- 
ing, respiration  is  concerned  with  the  whole  problem  of  gaseous  inter- 
change, not  merely  between  the  system  and  the  external  medium,  but 
also  between  the  blood,  the  internal  vehicle,  and  the  tissue  cells. 

Internal  Respiration. — This  latter,  the  internal  respiration,  we 
can  but  glance  at  incidentally,  although  it  is  the  process  to  which  the 
other  is  subservient.  The  data  regarding  the  conditions  in  which  oxygen 
exists  in  the  blood,  the  avidity  for  it  exhibited  by  the  hemoglobin  of  the 
erythrocytes,  its  entrance  into  combination  with  the  same,  are  known 
to  every  first  year  student  of  medicine.  But  of  the  processes  occurring 
in  the  capillaries  governing  the  discharge  of  the  oxygen,  of  its  entrance 
into  the  tissues  and  into  the  cells,  and  of  the  changes  undergone  in  these 
cells  we  know  singularly  little. 

The  arterial  blood,  we  know,  is  almost  saturated,  but  not  quite,  with 
oxygen,  nor  is  this  wholly  used  up  in  the  circulation  through  the  tissues; 
even  in  the  last  stages  of  asphyxia  some  oxygen  can  still  be  gained  from 
the  blood.  In  other  words,  the  blood  carries  more  oxygen  than  is  needed 
by  the  tissues.  Some  of  this  oxygen,  but  only  a  small  portion,  would  seem 
to  undergo  direct  reduction  in  the  red  corpuscles,  with  production  of 
carbon  dioxide.  This,  however,  is  but  an  inconsiderable  fraction  of 
the  gaseous  interchange.  We  possess  abundant  evidence  that  the  active 
interchange  occurs  in  the  tissue  cells.  These  have  an  intense  avidity 
for  oxygen,  and  take  it  up  from  the  surrounding  blood  and  lymph — 
nay,  more,  are  capable  of  storing  it  to  some  extent,  for  it  has  been  shown 
that  muscle  and  other  tissues  are  capable  of  active  metabolism  for  some 
little  period,  in  an  atmosphere  deprived  of  oxygen,  or  when  transfused 
with  oxygen-free  saline  solution,  and  during  this  metabolism  they  dis- 


238     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

charge  abundant  CO2.  The  appearance  of  glucose,  lactic  acid,  etc., 
in  the  urine  of  animals  kept  upon  an  insufficient  oxygen  supply,  indi- 
cates that  the  oxygen  thus  taken  into  the  cell  in  excess  enters  there  into 
loose  chemical  combinations,  and  that  when  this  store  of  oxygen  is  needed, 
and  there  is  continued  discharge  of  CO2,  these  bodies  also  become 
discharged  from  the  cells.  Ultimately  the  amount  of  oxygen  removed 
from,  and  of  CO2  afforded  to  the  capillary  blood,  depends  upon  the  rela- 
tive tension  of  the  two  gases  in  the  blood  and  tissue  cells  respectively; 
but  of  the  stages  whereby  the  combined  oxygen  of  the  erythrocytes 
becomes  liberated  into  the  surrounding  plasma,  and  thence  passes  to  the 
lymph,  and  thence  to  the  tissue  cells,  our  knowledge  is  minimal.1 

External  Respiration. — In  connection  with  this  we  have  to  pass 
in  review:  (1)  Disturbances  in  the  mechanism  whereby  air  gains 
entrance  into  the  lungs,  and  their  effects.  (2)  Disturbances  in  the  air 
sacs  of  the  lungs,  the  medium  of  gaseous  interchange  between  the  air 
and  the  blood,  and  their  effects.  (3)  Disturbances  in  the  composition 
of  the  air  entering  the  lungs. 


THE  RESPIRATORY  MECHANISM. 

This  may  be  divided  into  four  parts:  (1)  The  air  passages;  (2)  the 
muscles  of  respiration;  (3)  the  pleural  cavities;  and  (4)  the  nervous 
mechanism. 

The  Air  Passages. — Under  these  are  to  be  included  the  whole 
tract  from  the  external  nares  to  the  terminal  bronchioles.  These,  it 
will  be  recognized,  are  of  considerable  length,  and  their  effect  upon  the 
air  passing  down  them  is  that  with  normal  respiration  it  enters  the  air 
sacs  (a)  at  the  body  temperature  instead  of  at  that  of  the  external  air; 
(6)  impregnated,  if  not  saturated  with  moisture;  and  (c)  devoid  of  dust 
and  foreign  particles,  and,  as  a  consequence,  sterile. 

A  multiplicity  of  mechanisms  bring  about  these  results,  and  disturb- 
ances of  any  one  of  them  tends  to  modify  the  quality  of  the  air  gaining 
entrance  to  the  air  sacs.  The  nasal  passages  are  factors  of  high  impor- 
tance; the  wide  surfaces  of  the  turbinated  bones  are  of  peculiar  service, 
both  in  warming  the  inspired  air  after  the  manner  of  radiators,  and  in 
imparting  moisture  to  the  same.  To  a  considerable  extent,  also,  these 
moistened,  somewhat  glairy  surfaces  detain  foreign  particles  present  in 
the  air  passing  over  them.  It  cannot  be  too  strongly  emphasized  that 
persistent  mouth-breathing  is  harmful  in  that  it  favors  the  air  entering 
the  air  sacs  being  deficient  in  each  of  these  respects.  Hence,  it  favors 
irritation  of  the  lower  air  passages  and  of  the  lungs,  with  resultant  inflam- 
mation. Such  mouth-breathing  results  from  nasal  obstruction  of  several 
orders — congenital  narrowness  of  the  nares,  acquired  stenosis  of  these 
passages,  from  trauma,  chronic  syphilitic  and  other  inflammations, 

1  For  the  main  data  bearing  upon  the  internal  respiration,  see  Pembrey's  article 
on  Respiration,  in  Schafer's  Physiology,  vol.  1 :  p.  780. 


THE  PHARYNX  239 

acute  and  subacute  catarrh  with  excretion  of  abundant  thick  mucus, 
the  presence  of  overgrowths  and  tumors  within  the  nasal  passages,  most 
commonly  of  mucous  polyps  in  the  posterior  nares  and  of  adenoids 
in  the  nasopharynx.  These  last,  occurring  in  childhood  and  arresting 
the  function  of  the  nose,  arrest  also  its  due  development,  so  that  even 
after  removal  it  may  happen  that  the  passages  remain  abnormally  small, 
and  nasal  respiration  is  not  as  free  as  it  should  be.  The  result  in  all 
these  cases  is  a  peculiar  liability  to  inflammation  of  the  throat  and  lower 
respiratory  passages,  increased  susceptibility  to  infection,  and  in  children 
a  distinct  delay  in  bodily  and  even  in  mental  development.  Your 
mouth-breathing  child  not  only  looks,  but  actually  is,  more  stupid  than 
the  average  of  normal-breathing  children. 

The  condition  of  the  inspired  air  may  also  affect  the  nasal  passages, 
and,  influencing  their  function,  adversely  affect  the  lower  respiratory 
passages  and  the  lungs.  The  nasal  mucosa  is  distinctly  sensitive,  with 
abundant  vascular  supply  and  rich  supply  of  mucous  glands.  Extremes 
of  temperature  of  the  air,  the  presence  of  irritant  gases  and  particles 
induce  congestion  and  inflammation  with  relative  facility,  an  inflamma- 
tion characterized  by  abundant  mucous  discharge.  That  discharge 
would  appear  to  have  several  functions;  it  protects  the  underlying  epi- 
thelium; washes  off  and  dilutes  irritant  substances,  and  is  to  some  extent 
bactericidal,  added  to  which,  by  its  physical  constitution,  it  arrests  the 
passage  and  spread  of  bacteria. 

If  chronic  and  long-continued,  catarrh  is  apt  to  be  followed  by  atrophy 
of  the  nasal  mucosa.  Such  atrophic  rhinitis  being  accompanied  by 
reduction  of  discharge  and  direct  lodgement  of  irritant  particles  on  the 
nasal  mucosa,  favors  ulceration  and  deep  inflammation,  and  as  regards 
the  lower  respiratory  passages,  from  pressure  of  irritant  bodies  in  the 
inhaled  air,  and  from  relative  lack  of  moistening  of  that  air,  tends  to  pro- 
duce results  similar  to  those  brought  about  by  mouth-breathing. 

The  Pharynx. — The  moist  surface  of  the  pharynx,  together  with  the 
sudden  bend  in  the  direction  of  the  stream  of  inhaled  air,  converts  this 
more  particularly  into  an  adjuvant  apparatus  for  removal  of  dust  and 
other  particles.  It  can  be  demonstrated  experimentally  that  if  a  current 
of  air  impinges  upon  a  moist  surface  at  an  angle  of  45°  or  thereabout, 
it  delivers  up  to  that  surface  the  majority  of  its  contained  solid  particles. 
The  mucous  glands  of  the  pharyngeal  mucosa  provide  a  mechanism 
whereby  this  surface  is  kept  continually  moistened,  and  the  abundant 
lymph-glands  of  the  tonsils  and  pharyngeal  roof  constitute  a  further 
protective  mechanism.  That  they  function  in  arresting  pathogenic 
bacteria  is  indicated  by  the  acute  tonsillitis  which  is  the  first  active 
symptom  in  so  many  diseases  which  we  regard  as  air-borne,  notably  the 
acute  exanthemata.  Although  the  diphtherial  membrane  may  show 
itself  primarily  in  the  nasal  passages,  or  upon  the  back  of  the  pharynx, 
it  is  over  the  tonsils  that  it  most  often  makes  its  first  appearance. 
Adenoids  and  other  growths  of  the  upper  part  of  the  pharynx,  inflam- 
matory and  neoplastic  growth  of  the  tonsils,  of  necessity  obstruct  the 
passage  of  air  to  a  greater  or  less  extent. 


240     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

Stertor. — Yet  another  form  of  obstructed  breathing  is  brought  about 
by  loss  of  tone  or  actual  paralysis  of  the  muscles  of  the  soft  palate — that 
form  of  stertor  known  as  snoring.  As  the  reader  may  determine  for 
himself,  three  factors  are  necessary  to  produce  this,  namely,  a  flaccid, 
soft  palate,  a  combination  of  mouth-  and  nose-breathing,  and  relatively 
deep  inspirations. 

The  other  forms  of  stertor  may  here  be  noted  in  passing.  They  are : 
(2)  Nasal  stertor,  seen  in  apoplexy,  with  paralysis  of  the  muscles  of 
the  alse  nasi,  the  inhaled  air,  as  in  sniffing,  drawing  the  alse  against  the 
septum.  (3)  Buccal  stertor,  or  puffing  out  and  flapping  of  the  cheeks. 
This  may  occur  in  cases  of  paralysis  of  the  facial  nerves,  or  again,  as  in 
the  snoring  sleeper,  from  flaccidity  of  the  muscles  with  partial  closure 
of  the  mouth.  (4)  Pharyngeal  stertor,  due  to  falling  back  of  the  base  of 
the  tongue  with  obstruction  to  the  laryngeal  entrance.  (5)  Laryngeal 
stertor,  heard  most  commonly,  according  to  Lister,  during  chloroform 
inhalation,  and  also  in  paralysis  of  some  of  the  muscles  influencing  the 
vocal  cords;  and  lastly,  (6)  Mucous  stertor,  due  to  the  bubbling  of  air 
through  mucoid  fluid  in  the  larger  air  tubes.  Of  this  nature  is  the 
"death  rattle."  As  pointed  out  by  Bowles,  most  of  these  can  be  pre- 
vented by  altering  the  posture  of  the  individual. 

The  Larynx. — Two  main  functions  are  to  be  ascribed  to  the  larynx — 
phonation  and  the  provision  of  a  firm  base  for  attachment  of  the  epi- 
glottis and  control  of  its  movements  in  the  act  of  swallowing.  As 
regards  the  former,  it  has  to  be  admitted  that  the  physiology  and  pathology 
of  voice  production  lead  us  so  far  from  the  main  object  of  this  chapter 
— that  of  discussing  the  disturbances  of  respiration  and  their  nature — 
and  constitute  in  themselves  so  large  a  subject,  that  here  we  can  at  most 
call  attention  to  them  parenthetically.  We  would  recall  that  the  larynx, 
essential  for  the  singing  voice,  is  not  essential  for  speech;  that  speech 
is  possible  after  the  complete  extirpation  of  the  larynx,  although  then 
it  is  hollow,  and  little  more  than  a  whisper;  that  variation  in  the  "note" 
of  the  voice  is  determined  by  the  tension  and  consequent  rate  of  vibration 
of  the  vocal  cords  and  communication  of  the  waves  thus  set  up  to  the 
issuing  air;  that  thus  to  this  extent  the  voice  is  affected  by  pathological 
conditions  of  the  vocal  cords;  that  these  pathological  conditions  range 
themselves  into  two  groups :  (1)  The  nervous,  excessive  stimuli  leading 
to  spasmodic  contraction  of  the  intrinsic  laryngeal  muscles,  with  closure 
of  the  laryngeal  aperture ;  defective  or  arrested  stimuli,  leading  to  flaccidity 
and  paralysis  of  the  vocal  cords  with  lack  of  the  finer  vibrations;  and 
(2)  intrinsic  disturbances  of  the  cords,  either  diffuse  or  localized  inflam- 
matory thickening  of  the  same,  or  the  development  of  tumors  upon  them; 
by  each  of  these  disturbances  the  cords  become  "muted;"  that  the 
"timbre"  of  the  voice  depends  upon  the  various  resonating  cavities 
communicating  with  the  main  air  stream,  upon  their  size  and  the  freedom 
of  communication,  upon  the  lungs  and  thoracic  cavity,  the  laryngeal 
sacculi,  the  sphenoidal,  frontal,  ethmoidal,  and  antral  sinuses,  etc.,  as 
also  upon  the  extent  of  development  of  the  palatal  arch;  that  variation 
in  the  development  of  these,  obstruction  of  their  orifices,  inflammatory 


THE  TRACHEA  AND  BRONCHI  241 

and  other  conditions  leading  to  the  accumulation  of  liquid  or  solid  matter 
within  the  cavities,  materially  affect  the  resonance  of  the  voice;  that 
articulation  is  dependent  upon  the  lips,  teeth,  palate,  and  tongue,  and 
this  is  materially  affected  either  by  congenital  or  acquired  defects  in  these 
organs,  or  by  paralysis  of  the  muscles  of  the  lips,  tongue,  and  lower  jaw. 

We  are  apt,  on  first  thought,  to  cite  the  crossing  of  the  respiratory  and 
alimentary  tracts  as  an  example  of  evolutionary  imperfection.  Un- 
doubtedly it  has  its  disadvantages.  With  imperfect  action  of  the 
larynx  or  disease  of  the  epiglottis,  foodstuffs  may  gain  entrance  into  the 
trachea  and  lungs;  with  paralysis  of  the  soft  palate,  such  as  is  apt  to  occur 
after  diphtheria,  fluids,  instead  of  being  swallowed,  may  pour  into  and 
through  the  nares;  the  infant  suffering  from  obstructive  nasal  catarrh 
cannot  suckle.  But  this  same  infant,  under  the  same  conditions,  were 
there  no  alternative  respiratory  channel  through  the  mouth,  could  not 
breathe;  and  when  we  come  to  consider  the  secondary  employment 
of  the  organs  of  mastication,  of  the  mouth  and  mouth  parts  for  articula- 
tion and  speech,  it  may  be  regarded  as  at  least  doubtful  whether  man, 
as  a  communicative  animal,  does  not  to  a  very  great  extent  owe  his  posi- 
tion in  nature  to  the  utilization  of  this  apparent  imperfection. 

The  more  recent  studies  upon  deglutition  indicate  that  the  epiglottis 
is  to  be  regarded  as  an  adjuvant  rather  than  the  essential  organ  in  pre- 
venting the  entrance  of  foodstuffs  into  the  larynx  during  the  act  of  swallow- 
ing. This  view  is  supported  by  the  fact  that  in  tertiary  syphilis  more 
particularly,  as  again  after  extensive  tuberculous  ulceration,  there  may  be 
most  extensive  erosion  and  destruction  of  the  epiglottis,  and  this  evidently 
of  long-standing,  and  without  observed  suffocative  attacks.  During 
swallowing,  that  is,  the  laryngeal  entrance  is  raised  under  the  backward 
projecting  root  of  the  tongue,  so  that  the  bolus  of  food  or  stream  of 
fluid  passes  well  behind  it. 

Lastly,  it  has  to  be  noted  that  the  passage  through  the  vocal  cords 
constitutes  the  region  of  greatest  narrowing  of  the  main  respiratory 
passage.  Here,  therefore,  the  slightest  grade  of  narrowing  by  any  cause 
— spasmodic  contraction,  inflammatory  deposits,  new-growth,  etc. — 
produces  relatively  the  most  obstruction  to  the  respiratory  act.  But 
the  larynx  as  a  whole  is  a  region  of  narrowing  of  the  passage,  and  thus 
affections  of  the  glottis  in  general  are  liable  to  induce  grave  obstruction. 
More  especially  the  loose  attachment  of  the  mucosa  (save  over  the  vocal 
cords)  renders  congestion  and  oedema  a  not  uncommon  event,  either 
as  the  result  of  trauma  or  other  irritation,  of  infection,  or  rarely  of 
angioneurotic  type,  as  one  manifestation  of  the  tendency  to  local  cedema 
of  sudden  development  and  unknown  causation  which  may  attack 
individual  portions  of  the  digestive  and  respiratory  tracts.  In  all  these 
cases,  the  upper  opening  of  the  larynx,  the  epiglottic  and  aryteno- 
epiglottic  folds  are  most  involved,  and  may  by  their  swelling  cause  such 
narrowing  of  the  passage  that,  unless  intubation  or  tracheotomy  be  per- 
formed, death  may  ensue  from  asphyxia. 

The  Trachea  and  Bronchi. — Beyond  the  larynx  the  respiratory  passage 
widens  again  into  the  trachea,  which,  it  may  be  noted,  is  not  of  uniform 
16 


242     THE  RESPIRATORY  FUNCTION   AND  ITS  DISTURBANCES 

diameter,  but  according  to  the  measurements  of  Braune  and  Stahel1 
is  at  its  broadest  about  half-way  between  the  larynx  and  the  point  of 
bifurcation,  slowly  narrowing  below  this.  The  transverse  section  of  the 
larger  bronchi  is  again  greater  than  that  of  the  lower  end  of  the  trachea, 
and  greater  than  that  of  the  combined  smaller  bronchi.  The  result  of 
these  variations,  both  in  the  shape  and  the  transverse  section  volume  of 
the  respiratory  passages,  must  necessarily  be  to  intermingle  the  different 
portions  of  the  inspired  air-stream,  and  so  favor  all  portions  of  that  stream 
coming  into  contact  at  one  or  other  point  with  the  walls  of  the  channel. 
There  are,  indeed,  indications  that  the  stream  eventually  acquires  a  spiral 
instead  of  a  direct  motion.  The  moist  ciliated  surface  which  extends 
from  the  larynx  to  the  terminal  bronchioles  thus  tends  to  arrest  such 
solid  particles  as  have  managed  to  pass  the  upper  respiratory  passages, 
and  through  the  action  of  the  cilia  these  are  passed  upward  to  be  expelled 
through  the  larynx  by  an  act  of  coughing.  An  adjuvant  in  the  removal 
of  matter  are  leukocytes  which  migrate  on  to  the  free  surface,  and  either 
become  expectorated  or  wander  back  into  the  lymph-glands,  notably 
into  the  group  below  the  bifurcation  of  the  trachea. 

Despite  the  mechanism  of  cartilaginous  bars,  by  means  of  which  the 
trachea  and  bronchi  are  kept  patent  arid  at  the  same  time  mobile,  there 
may  be  hindrance  to  passage  of  air  through  them  either  from  (1)  the 
entrance  of  foreign  bodies,  (2)  inflammatory  deposits  or  contraction, 
(3)  new-growths,  or  (4)  pressure  from  without.  The  last  may  be 
variously  produced  by  aneurism,  goitre,  mediastinal  tumors,  enlarged 
tuberculous  lymph-glands,  and,  very  possibly,  by  the  enlarged  thymus. 
Regarding  the  capacity  of  the  enlarged  thymus  to  produce  direct  com- 
pression of  the  trachea  in  the  striking  and  fatal;  condition  known  as 
thymic  asthma,  there  is  still  debate.  The  sudden  onset  of  the  dyspnoea 
and  the  stridor  suggest  spasm  of  the  glottis,  and  although  in  these  cases 
postmortem  examination  reveals  an  enlarged  thymus,  there  is  no  sign 
of  narrowing  or  distortion  of  the  trachea.  Nevertheless,  it  may  be 
urged  that  the  thymus  of  young  children  is  a  very  vascular  organ;  that 
a  sudden  congestion  may  greatly  increase  its  size  and  the  pressure  that 
it  exerts  upon  the  trachea  where  this  passes  through  the  narrow  orifice 
of  the  chest ;  and  that  such  sudden  compression  cannot  be  expected 
to  show  permanent  effects  upon  the  tracheal  tube.  Certain  it  is  that 
recent  cases  are  on  record  in  which  the  operative  removal  of  the  thymus 
during  paroxysms  of  this  form  of  spasmodic  dyspnoea  have  been  followed 
by  marked  disappearance  of  symptoms.  We  have  encountered  similar 
paroxysmal  attacks  of  dyspnoea  in  cases  of  goitrous  enlargement  of  the 
middle  lobe  of  the  thyroid  which  could  only  be  ascribed  to  congestion 
and  accompanying  increase  in  size  and  pressure  of  the  goitrous  mass. 

Of  peculiar  interest  are  those  forms  of  obstruction,  whether  of  the 
larynx,  trachea,  or  bronchi,  which  assume  a  valvular  nature  permitting 
free  inspiration  or  expiration,  but  not  both.  Thus,  oedema  of  the 
glottis  or  a  polypoid  tumor  in  the  glottis  will  obstruct  inspiration,  as 

1  Berichte  d.  K.  Sachs.  Gesell.  d.  Wiss.,  Math.  Phys.  Cl.,  1885:  326. 


ASTHMA  243 

will  also  a  growth  of  the  walls  projecting  from  a  smaller  into  a  larger 
bronchus.  On  the  other  hand,  a  diphtheritic  membrane,  or  a  polyp 
attached  below  the  vocal  cords,  will  permit  inspiration,  but  act  as  a  val- 
vular plug  preventing  expiration,  as  may  also  an  enlarged  and  partly 
detached  tuberculous  gland  or  other  tumor  in  this  region  projecting  into 
one  of  the  larger  bronchi  at  the  root  of  the  lungs. 

Yet  another  form  of  obstruction  is  associated  with  bronchial  narrow- 
ing, namely,  asthma.  The  symptoms  here  point  very  definitely  to  a 
spasmodic  narrowing  of  the  bronchi;  the  extreme  grade  of  distension 
of  the  lungs  developed  during  the  attack  indicate  that  through  the  active 
inspiratory  efforts  air  is  sucked  into  the  alveoli,  but  with  expiration 
cannot  be  expelled  to  the  same  extent.  Evidently,  also,  the  whole 
bronchial  tree  is  simultaneously  involved,  for  all  the  lobes  of  both  lungs 
are  simultaneously  expanded.  The  sudden  onset  of  the  condition,  and 
sudden  departure,  indicate  a  nervous  origin,  that  the  condition  is  a  neuro- 
sis ;  and,  as  a  matter  of  fact,  it  has  been  observed  that  in  many  susceptible 
subjects  particular  odors  and  surroundings  induce  the  condition,  while 
there  is  in  some  a  small  area  of  the  posterior  and  upper  portion  of  the 
nasal  septum,  irritation  of  which  induces  an  asthmatic  attack.  Various 
theories  have  been  adduced  to  explain  the  development  of  the  condition — 
that  it  is  due  to  spasm  of  the  bronchial  muscle  sheath;  that  it  is  due  to 
hyperemia  and  swelling  of  the  bronchial  mucous  membrane  (Traube, 
Weber,  Clark);  that  in  many  cases  it  is  a  special  form  of  inflammation 
of  the  bronchioles,  bronchiolitis  exudativa  (Curschmann) ;  that  it  is  due  to 
a  reflex  spasm  of  the  respiratory  muscles.  This  last  may  be  dismissed; 
it  does  not  explain  the  overfilling  of  the  lungs.  Curschmann's  theory 
also  is  untenable;  the  onset  is  too  sudden  for  any  known  inflammation. 
Brodie  and  Dixon1  have  brought  forward  strong  evidence  in  favor  of 
the  bronchial  spastic  theory.  It  had  already  been  shown  (MacGillavry, 
Einthoven,  Beer)  that  the  vagus  contains  motor  fibers  for  the  bronchial 
muscles.  By  oiicometric  records  of  the  lobe  of  the  dog's  lung  under 
artificial  respiration,  they  proved  that  vagus  stimulation  could  produce 
rapid  arrest  both  of  entry  into  and  of  exit  of  air  from  the  air  sacs, 
as  again,  the  state  of  overdistension  of  the  organ.  They  point  out  that 
those  drugs  which  are  of  service  in  asthma  are  just  those  which  by  experi- 
ment they  determined  cause  paralysis  of  the  nerve  endings,  and  point 
out  that  ether,  which  is  of  material  benefit  in  asthma,  notoriously  causes 
hyperemia  of  the  mucous  membrane  and  increased  secretion  from  the 
bronchial  glands.  Nevertheless,  it  does  not  seem  to  us  that  Brodie  and 
Dixon  have  adequately  explained  all  the  symptoms  of  all  cases.  They 
pass  too  slightingly  over  the  discharge  which,  while  not  primary,  never- 
theless soon  makes  its  appearance  in  a  larger  proportion  of  cases,  and 
this  even  when  there  has  been  no  attack  for  a  long  period.  As  is  well 
known,  this  discharge  is  characterized  by  the  presence  of  Charcot-Leyden 
crystals  and  Curschmann's  spirals  (p.  278).  And  they  depreciate 
the  vascularity  of  the  bronchial  mucous  membrane.  But,  as  every 

1  Trans.  Path.  Soc.  London,  54:  1903:17. 


244     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

one  knows  who  has  studied  sections  of  the  lungs  in  acute  bronchitis, 
that  mucous  membrane  is  highly  vascular.  Admitting  freely  that  the 
condition  is  brought  about  by  reflex  nervous  stimulation,  as  also  the 
value  of  Brodie  and  Dixon's  observations,  we  still  cannot  but  consider 
that,  in  one  order  of  cases  at  least,  the  abundant  exudate,  and  the  similar- 
ity of  the  causative  factors  to  those  seen  operating  in  hay-fever  suggest 
the  presence  of  a  similar  angioneurotic  oedema. 

The  General  Effects  of  Obstruction  to  the  Air  Passages. — It  is 
scarce  necessary  to  state  that  complete  obstruction  of  the  air  passages 
for  more  than  a  few  minutes  ends  in  death  by  asphyxia.  If  the  obstruction 
be  incomplete,  then  according  to  its  nature  do  we  find  either  inspiratory 
or  expiratory  dyspnoea,  or  both,  and  according  to  its  degree  do  we  have 
either  death  by  progressive  slow  suffocation,  or  a  remarkable  adaptation 
of  the  respiration  and  of  the  bodily  activities  in  general  to  the  lessened 
supply  of  oxygen.  With  obstruction  to  inspiration,  the  respiratory  acts 
become  slow  and  labored,  and  not  only  are  the  diaphragmatic  contrac- 
tions deeper,  but  also  the  accessory  muscles  of  respiration  are  called 
into  play,  so  as  to  secure  the  greatest  extent  of  thoracic  enlargement. 
In  marked  contrast,  the  expirations  are  short  and  not  labored.  The  swift 
passage  of  the  air  through  a  narrowed  passage  leads  to  inspiratory  stridor. 
The  reverse  is  the  case  in  expiratory  obstruction.  Here  it  is  the  expiration 
that  is  slow  and  labored;  the  accessory  muscles  called  into  play  are  those 
which  most  reduce  the  thoracic  capacity,  notably  the  abdominal  muscles 
and  those  bending  the  vertebral  column,  bringing  the  ribs  more  close  to 
each  other. 

Where  only  one  main  bronchus  is  obstructed  the  results  are  different; 
a  period  of  rapid,  not  to  say  tumultuous  respiration,  is  followed  by 
indications  of  adaptation,  the  one  lung  being  capable  of  performing 
the  functions  of  both.  The  rapid  irregular  respiration  may  be  ascribed 
to  the  different  periods  at  which  the  vagus  inspiratory  and  expiratory 
stimuli  are  initiated  in  the  two  lungs  (see  p.  248  et  seq.). 


THE  MUSCLES  OF  RESPIRATION. 

In  ordinary  quiet  breathing  the  expansion  of  the  lungs  in  inspiration 
is  an  active  process,  expiration  being  largely,  if  not  entirely,  passive, 
due  to  relaxation  of  the  diaphragm,  and  pressure  upward  of  the  abdom- 
inal viscera  under  the  normal  abdominal  tension,  and  to  the  elastic 
reaction  of  the  distended  lungs  and  of  the  thoracic  musculature.  And 
in  children  of  both  sexes,  and  in  the  adult  male,  the  distension  of  the 
lungs  in  inspiration  is  in  the  main  brought  about  by  contraction  of  the 
diaphragm.  The  use  of  corsets,  and  that  only,  would  seem  to  reduce 
the  diaphragmatic  activity  and  cause  inspiration  of  the  costal  type. 
Women  who  do  not  employ  corsets  are  found  to  exhibit  the  diaphrag- 
matic type  of  inspiration  indistinguishable  from  that  of  men. 

But  while  the  contraction  of  the  diaphragmatic  muscle,  by  separating 
the  circumferential  portion  of  this  septum  from  the  costal  wall,  is  the 


THE  PLEURAL  CAVITIES  245 

main  factor  in  increasing  the  thoracic  cavity,  it  must  be  kept  in  mind 
that  in  inspiration  the  thorax  increases  in  every  direction — in  lateral  and 
fore  and  aft  diameters,  as  well  as  in  the  vertical.  It  is  thus  obvious  that 
the  costal  musculature  is  also  a  factor  even  in  pronounced  cases  of  dia- 
phragmatic breathing.  According  to  Mosso,  during  natural  sleep  this 
thoracic  breathing  is  the  normal  condition.  Of  the  muscles  causing  eleva- 
tion of  the  ribs  and  lateral  and  anterior  expansion  of  the  thoracic  cage, 
the  external  intercostals  are  the  most  important.  In  forced  inspiration  a 
large  number  of  other  muscles  are  called  into  play;  the  head,  shoulder, 
and  arm  are  all  called  upon  to  form  fixed  supports  for  muscles  which, 
passing  from  them  to  the  thorax,  elevate  and  rotate  the  ribs  forward  and 
outward,  that  the  thoracic  cage  may  attain  its  greatest  capacity;  as 
again,  that  the  increase  in  the  negative  pressure  within  the  thorax  may 
cause  most  forcible  inhalation.  The  more  important  of  these  muscles 
are  the  sternomastoids,  the  pectoralis  minor  and  lower  part  of  the  pec- 
toralis  major,  and  the  lower  segments  of  the  serratus  magnus.  In 
normal  inspiration  also  the  central  tendon  of  the  diaphragm  does  not 
alter  its  position;  with  forced  inspiration,  with  more  vigorous  contraction 
of  the  diaphragmatic  muscle,  it  is  pulled  downward,  thus  raising  very 
materially  the  negative  intrathoracic  pressure.  Yet  other  accessory 
muscles  must  be  noted.  Even  in  ordinary  respiration,  the  larynx 
moves  downward  during  inspiration  through  the  contraction  of  the  sterno- 
hyoid  and  sternothyroid  muscles,  the  upward  movement  in  expiration 
being  aided  by  the  thyrohyoid  muscles.  In  forced  inspiration  the 
larynx  is  to  some  extent  pulled  downward  by  the  descent  of  the  lungs 
and  trachea.  In  forced  inspiration,  also,  and  where  there  is  nasal  ob- 
struction (and  in  the  quiet  breathing  of  children,  also,  with  their  smaller 
nasal  passages),  the  dilator  naris  comes  into  play,  widening  the  nostrils. 

Expiration. — Normal  expiration  is  wholly,  or  almost  wholly,  a 
passive  process.  As  Starling  expresses  it,  the  inspiratory  enlargement 
of  the  lungs  not  only  acts  against  gravity  in  raising  the  ribs,  but  also 
stores  up  potential  energy  in  consequence  of  a  stretching  of  the  rib 
cartilages  and  of  the  elastic  lungs.  Thus  it  follows  that  as  the  muscles 
relax,  gravity  and  the  elasticity  of  the  cartilage  and  lungs  come  into 
play,  and  restore  the  thorax  to  its  resting  state  and  original  size.  Where, 
however,  there  is  obstruction  and  forced  expiration,  there  muscular  aid 
is  given  to  lessening  of  the  thoracic  cavity,  more  particularly  by  contrac- 
tion of  the  abdominal  wall,  whereby  the  abdominal  contents  force  the 
diaphragm  upward,  while  at  the  same  time  the  ribs  and  sternum  are 
pulled  downward.  The  lower  ribs  are  further  pulled  downward  by 
the  serratus  posticus  inferior  and  the  sacrolumbalis. 


THE  PLEURAL  CAVITIES. 

Intimately  associated  with  the  muscular  apparatus  of  the  thorax  in 
bringing  about  the  distension  of  the  lungs  we  have  to  consider  the  pleurae. 
These  form  closed  cavities  around  either  lung.  Were  the  lungs  directly 


246      THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

attached  to  the  parietes  (as  happens  in  cases  of  universal  pleural  adhe- 
sions), expansion  and  contraction  could  occur,  but  the  extent  would  vary 
greatly  in  different  regions.  With  diaphragmatic  contraction  there 
would  be  great  expansion  of  the  lower  lobes,  whereas  the  apices  would  be 
scarcely  influenced,  and,  as  a  consequence,  there  would  be  little  inter- 
change of  air  occurring  in  them.  The  pleural  cavity,  with  the  free  move- 
ment of  the  visceral  over  the  parietal  pleura,  insures  that  with  inspiration 
the  lungs  undergo  uniform  expansion;  if  the  excursion  of  the  lower  parts 
of  the  lower  lobes  is  the  greatest,  this  at  the  same  time  causes  the  expan- 
sion of  the  whole  organ  and  of  its  individual  air  sacs. 

According  to  Donders,  the  elastic  pull  of  the  lungs  in  expiration — and 
this  is  equivalent  to  the  negative  pressure  in  the  pleural  cavities — is 
7.5  mm.  Hg.  in  the  expiratory  phase;  it  is  increased  to  about  9  mm. 
in  ordinary  inspiration,  and  to  30  mm.  in  the  deepest  inspiration. 

Pneumothorax. — This  negative  may  be  converted  into  a  positive 
pressure  in  either  or  both  cavities  by  the  entrance  of  air  or  gas  into  them, 
whether  by  traumatic  or  other  communication  with  the  exterior,  or  with 
gas-containing  abdominal  viscera;  by  rupture  of  the  visceral  pleura, 
and  communication  between  the  air  sacs  and  the  pleural  cavity;  or 
thirdly,  by  the  generation  of  gases  in  the  cavity  through  the  activity  of 
gas-producing  bacteria.  Contrary  to  the  general  teaching,  we  would 
point  out  that  a  small  opening  into  the  healthy  pleural  cavity  is  not 
necessarily  followed  by  pneumothorax.  We  have  made  such  an  opening 
in  the  dog  (without  artificial  respiration)  and  seen  the  visceral  pleura 
move  across  it  with  each  breath  without  the  lung  undergoing  collapse, 
the  viscous  adhesion  of  the  two  layers  of  the  pleura  surrounding  the 
opening  being  sufficient  to  neutralize  the  positive  pressure  upon  the 
exposed  surface.  This,  it  must  be  added,  is  exceptional;  ordinarily, 
air  rushes  into  the  cavity,  and  the  pressure  it  exerts  upon  the  lung  causes 
collapse  of  the  same.  Nay,  more,  where  the  orifice,  either  in  the  thoracic 
wall  or  in  the  lung,  is  of  a  valvular  nature,  the  air  drawn  into  the  pleural 
cavity  during  inspiration,  and  unable  to  escape  during  expiration,  may 
accumulate  and  come  to  exert  a  pressure  upon  the  lung  in  excess  of  the 
atmospheric  pressure,  causing  an  extreme  filling  of  the  side  of  the  chest, 
even  to  the  extent  of  displacing  the  heart  and  compressing  largely  the 
other  lung,  thus  rendering  adequate  respiration  and  continued  existence 
impossible. 

Two  forms  of  pneumothorax  may  be  distinguished,  the  open  and  the 
closed.  In  the  former  there  is  free  communication  with  the  external 
air,  either  through  the  thoracic  wall,  or  an  abdominal  viscus,  or  most 
often  through  the  lung;  here  of  necessity  the  pressure  within  the  cavity 
is  positive,  and  the  lung  undergoes  complete  collapse,  unless  there  be 
pleural  adhesions  which  keep  portions  of  the  organ  distended.  In 
closed  pneumothorax  the  communication  has  become  occluded,  or,  as  in 
gaseous  pneumothorax,  may  never  have  existed.  In  such  cases,  according 
to  the  amount  of  contained  air  or  gas,  we  may  have  merely  diminution 
of  the  negative  pressure  or  low  positive  pressure;  and  here  respiration 
is  not  wholly  arrested  in  the  affected  lung.  There  is  considerable 


PLEURAL  EFFUSIONS  247 

divergence  of  opinion  regarding  the  effect  of  these  two  forms  upon 
respiration.  In  general  it  would  seem  that  in  both  there  is  a  tendency 
to  deeper  respiration,  with  increased  rapidity  in  the  open  form  and  some 
slowing  in  the  closed.1 

Pleural  Effusions. — The  mechanics  and  the  effects  upon  respira- 
tion of  the  accumulation  of  fluid  in  a  pleural  cavity  form  an  interesting 
study.  Upon  first  consideration  we  would  imagine  that  the  effect  of 
accumulation  of  fluid  in  a  closed  cavity  is  to  diminish  the  space  that 
can  be  occupied  by  the  lung;  that  the  lung  in  consequence  cannot  fully 
expand,  and  that  thus  the  effects  are  similar  to  those  of  a  positive  pressure 
exerted  upon  the  lung,  which,  as  a  matter  of  fact,  in  extreme  cases  be- 
comes completely  collapsed,  lying  against  the  vertebral  column.  Gar- 
land, however,  urges  (1)  that  a  pleuritic  exudation  does  not  compress 
the  lung  as  universally  taught,  but,  on  the  contrary,  by  virtue  of  its  weight 
exerts  a  negative  pressure;  (2)  that  the  lung  does  not  swim  upon  the 
effusion,  but  by  virtue  of  its  retractility  it  supports  the  entire  body 
of  the  effusion,  together  with  the  diaphragm,  until  the  weight  of  the  fluid 
exceeds  the  lifting  force  of  the  lung;  (3)  that  the  diaphragm  does  not 
bag  down  until  the  weight  of  the  fluid  exceeds  the  lifting  force  of  the 
lung,  and  (4)  that  the  heart  is  not  pushed  out  of  place  by  an  effusion, 
whether  of  air  or  fluid,  but  that  those  parts  are  drawn  over  by  the  negative 
pressure  in  the  other  pleural  cavity.  Enormous  effusions  increase  this 
displacement.  While  there  is  much  that  we  must  accept  in  these  con- 
clusions of  Garland,  it  is  difficult  to  accept  them  in  their  entirety.  Let 
us  begin  by  considering  the  normal  lung  at  the  end  of  expiration.  That 
organ  is  still  in  a  state  of  distension — of  distension  so  considerable  that 
its  elasticity,  or  the  force  necessary  to  keep  it  distended,  is,  as  already 
stated,  equivalent  to  a  negative  pressure  of  about  7  mm.  Hg.  With 
ordinary  inspiration  that  force  is  only  increased  by  about  2  mm.  Hg. 
Suppose  that  now  the  pleural  cavity  became  filled  in  the  expiratory 
phase  either  with  fluid  or  with  a  solid  growth.  The  lung  would  thereby 
become  collapsed  and  airless,  and  with  ordinary  inspiration,  instead  of 
expanding  to  the  extent  of  bringing  about  a  negative  pressure  of  9  mm., 
the  total  amount  that  it  could  exert  would  be  only  2  mm.  (i.  e.,  9- 
7  mm.).  In  other  words,  the  elasticity  of  the  lung  which  causes  the 
negative  pressure  to  come  into  play  is  exerted  to  a  very  slight  extent 
when  the  organ  is  collapsed;  just  as  a  rubber  band  exerts  very  little  pull 
when  it  is  flaccid  compared  to  the  pull  exerted  when  it  is  taut.  This 
progressive  reduction  of  the  pull  exerted  by  the  lung,  due  to  the  elasticity 
of  the  same,  would  seem  to  have  been  largely  neglected  by  Garland. 
Xor  is  it  merely  the  intrathoracic  negative  pressure  that  keeps  the  dia- 
phragm bowed  upward  in  spite  of  extensive  fluid  in  the  pleural  cavity. 
A  more  important  factor  is  the  upward  pressure  of  the  abdominal 
viscera  under  the  influence  of  the  muscles  of  the  abdominal  wall.  It  is 
very  rarely  that  we  find  the  diaphragm  bulging  actually  downward,  and 

1  For  a  full  study  of  the  mechanics  of  pneumothorax,  see  Emerson,  Johns  Hopkins 
Hospital  Reports,  11 : 1903: 1. 


248     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

then  in  conditions  of  marked  abdominal  flaccidity.  When  this  occurs 
it  is  to  be  noted  that  each  contraction  of  the  diaphragm,  instead  of  pro- 
ducing a  negative,  must  materially  increase  the  positive  pressure  in  the 
pleural  cavity. 

Thus,  our  opinion  is  that  Garland's  conclusions  are  only  valid  for 
conditions  where  there  is  but  relatively  small  accumulation  of  fluid  in 
either  cavity;  any  considerable  quantity  so  hinders  the  expansion  of 
the  lung  that  its  elasticity  cannot  be  effective,  while  the  very  weight  of 
the  fluid,  if  it  does  not  materially  lower  the  diaphragm,  and  so  lessen  its 
excursion  and  with  that  the  distension  of  the  lung,  must  at  least  interfere 
with  the  excursion  of  the  thorax  by  increasing  the  load  which  the  costal 
muscles  of  inspiration  have  to  carry.  Whether  the  diaphragm  or  the 
costal  muscles  bear  the  brunt  of  the  increased  load  will  depend  materially 
upon  the  position  of  the  patient.  Here  we  would  add  that  the  accumula- 
tion of  fluid  below  the  lung  in  the  lower  part  of  the  thorax  can  only 
exert  a  negative  pressure  upon  the  lung  when  its  weight  has  become 
sufficient  to  neutralize  the  upward  pressure  of  the  abdominal  organs  upon 
the  diaphragm.  As  a  matter  of  fact,  the  singular  freedom  with  which, 
in  general,  fluid  escapes  from  the  chest  in  thoracocentesis,  and  this 
during  both  the  inspiratory  and  the  expiratory  act,  and  the  absence  of 
suction  of  air  into  the  chest,  demonstrate  that  this  fluid  is  under  a  positive, 
and  not  under  a  negative  pressure.  It  is  only  when  the  amount  of  fluid 
is,  or  has  become,  small  that  there  is  danger  to  be  anticipated  of  suction, 
or,  more  accurately,  of  forcible  entrance  of  air  into  the  cavity. 


THE  NERVOUS  MECHANISM  OF  RESPIRATION. 

The  acts  of  inspiration  and  expiration  being  determined  by  muscular 
activity,  and  the  skeletal  muscles  not  acting  automatically,  but  contracting 
under  nervous  stimuli,  it  is  clear  that  we  have  in  the  first  place  to  look 
to  the  nervous  system  for  the  initiation  of  active  breathing,  and  that 
disturbances  affecting  the  centres  controlling  the  work  accomplished 
by  the  individual  muscles  which  bring  about  the  acts  of  inspiration  and 
expiration  must  materially  affect  those  acts. 

There  must,  in  the  first  place,  be  as  many  centres  as  there  are  individual 
muscles.  We  know,  in  the  first  place,  that  the  intercostal  muscles  have 
their  innervation  through  the  series  of  dorsal  motor  roots,  and  conclude 
that  the  neurons  that  cause  these  muscles  to  contract  originate  in  the 
anterior  horns  of  the  dorsal  cord.  The  diaphragm  is  innervated  by  the 
phrenic  nerves,  and  these  have  their  origin  from  the  fourth  and  fifth 
cervicals;  the  alse  nasi  are  innervated  by  the  seventh  cranial;  the  muscles 
of  the  vocal  cords  by  the  recurrent  laryngeal  branches  of  the  vagus. 
It  follows  from  this  that  the  performance  of  normal  acts  of  respiration 
depends  upon  the  coordinated  stimulation  of  a  series  of  centres  situated 
in  the  medulla  and  cervical  and  dorsal  cord,  as  also  that  destruction  of 
particular  centres,  or  of  the  nerves  passing  from  them  to  particular 
muscles,  must  modify  the  respiratory  act  in  particular  directions;  that 


THE  NERVOUS  MECHANISM  OF  RESPIRATION  249 

section  of  one  phrenic,  for  example,  must  arrest  diaphragmatic  respira- 
tion upon  one  side.     But  if  these  centres  are  coordinated,  there  must 
be  some   supreme  or  coordinating   centre.     The   striking   fact  is  that 
after  all  these  years  we  still  are  unable  to  state  exactly  what  particular 
group  of  cells  forms  this  centre.     We  know  that  if  the  brain  be  separated 
from  the  cord  by  section  through  the  upper  part  of  the  medulla  oblongata, 
respiration  is  unaltered;  that,  therefore,  the  centre  is  not  in  the  brain. 
The  observations  of  Flourens  and  several  others  indicate  that  it  is  bilateral 
and  situated  in  the  medulla;  but  the  experiments  of  Gad  and  Marinesco 
show  that  the  localization  given  by  Flourens,  Gierke,  and  Mislawsky 
is  either  incorrect  or  too  limited;  while  the  very  extent  of  the  area  which 
they,  in  their  turn,  lay  down,  namely,  the  cells  of  the  formatio  reticularis 
upon  either  side  of  the  median  line,  would  seem  too  vague  and  widespread 
to  constitute  what  is  generally  considered  a  spinal  or  cranial  centre. 
Granted  that  there  be  such  a  coordinative  respiratory  centre — which 
some  still  deny — how  does  it  work?    Does  it  send  down  automatic  and 
rhythmic  impulses,  or,  on  the  contrary,  is  it  only  stimulated  by  afferent 
impulses,  or,  thirdly,  is  its  activity  rhythmic,  but  capable  of  material 
modification  by  afferent  impulses  reaching  it  from  other  regions  of  the 
organism,  as  again  by  nutritional  influences  due  to  alteration  in  the 
gaseous  contents  of  the  blood  circulating  through  it?     These  questions 
also  cannot  be  said   to  have  gained  an  absolutely  uncertain  answer. 
This  much  is  clear:  (1)  That  stimulation  of  several  regions  of  the  brain, 
as  again  of  many  peripheral  nerves,  modifies,  at  least  temporarily,  the 
respiratory  act,  and  that  thus  the  respiratory  rhythm  is  influenced  by 
afferent  impulses;  (2)  that  the  pulmonary  branches  of  the  vagus  nerves 
stand  out  preeminently  in  exerting  an  influence  upon  the  respiratory 
rhythm;  they  are  the  foremost  afferent  nerves  of  respiration;  and  (3) 
that  the  activity  of  the  centre  is  materially  affected  by  the  blood  passing 
through  the  medulla,  being  stimulated  by  increased  presence  of  CO2, 
and  depressed,  not  so  much  by  over-oxygenation,  as  used  to  be  taught, 
as  by  reduced  CO2  tension.     The  brilliant  studies  carried  out  by  Head1 
in  Hering's  laboratory  established  firmly  the  second  of  these  conclusions, 
studies  in  which  he  was  able  to  observe  the  uncomplicated  movements 
of  the  main  muscle  of  respiration — the  diaphragm — by  recording  the 
contractions  of  a  small  isolated  band  of  the  same,  which,  in  the  rabbit, 
passes  to  the  ensiform  cartilage,  and  is  capable  of  isolation  along  with 
its  nerve  and  blood  supply  without  disturbing  the  thorax  or  its  contents. 
The  extent  of  the  contractions  of  this  muscle  and  variation  in  the  rate  of 
its  contraction  can  be  recorded  without  being  disturbed  by  passive  move- 
ments of  the  thorax.     By  this  means  the  observations  of  Hering  and 
Breuer  were  confirmed,  that  the  normal  stimulus  to  both  the  inspira- 
tory  and  the  expiratory  act  is  due  to  influence  of  the  intrapulmonary 
conditions  upon  the  nerve  endings  of  the  vagus.     Collapse  of  the  lung, 
for  example,  induces  a  prolonged  contraction  of  the  diaphragm — as  it 
were,  an  intense  inspiratory  effort  on  the  part  of  this  muscle;  inflation  of 

1  Jour,  of  Physiol.,  10:  1889:  279. 


250     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

the  lung,  on  the  contrary,  relaxation  of  the  diaphragm  such  as  accom- 
panies the  passive  expiratory  act  in  the  rabbit.  The  immediate  effect  of 
section  of  both  vagi  is  to  produce  inspiratory  tone,  or  partial  contraction 
of  the  diaphragm,  and  to  lengthen  individual  inspiratory  contractions 
of  the  same;  the  individual  contractions  are  slower  and  more  violent. 
What  is  the  meaning  of  these  phenomena  is  still  a  matter  of  debate — 
whether,  for  example,  with  relaxation  of  the  lung,  stimuli  pass  up  the 
vagi  which  set  in  action  the  inspiratory  muscles,  or,  on  the  contrary, 
inhibit  the  expiratory  mechanism;  or  whether,  again,  the  only  impulses 
which  pass  up  the  nerve  are  expiratory  in  nature';  or,  lastly,  whether,  as 
Meltzer1  has  urged,  the  vagus  contains  both  positive,  inspiratory  and 
inhibitive,  expiratory  fibers.  Further,  it  is  to  be  kept  in  mind  that 
vagus  stimulation  is  not  necessary  for  respiration;  after  division  of  both 
vagi  respiration  gradually  assumes  a  regular  although  slower  rate, 
with  long  and  powerful  inspirations  with  intervening  complete  expira- 
tions. These  may,  it  is  true,  be  brought  about  by  impulses  reaching 
the  coordinating  centres  through  other  afferent  nerves.  They,  at  the 
same  time,  raise  the  question  whether  the  centre  may  not  possess  an  auto- 
matic rhythm  of  its  own,  normally  always  in  action,  but  modified  by 
impulses  proceeding  from  the  lungs  and  other  cerebral  and  spinal  centres. 
The  existence  of  this  automatic  rhythm  is  inferred  from  several  considera- 
tions. Thus,  more  particularly,  several  observers  have  shown  that  if 
the  medulla  be  separated  from  the  brain  above,  and  the  lower  cervical 
cord  be  cut  across,  along  with  all  sensory  nerves  reaching  the  cord  be- 
tween these  two  sections,  a  rhythmical  contraction  of  the  diaphragm 
is  still  in  evidence,  slow  but  definite.  It  may,  however,  be  objected  that 
the  trauma  of  the  cut  ends  of  the  sensory  nerves  still  acts  as  a  sensory 
stimulus.  The  problem  thus  is  one  that  it  is  practically  impossible  to 
solve  by  direct  means.  We  can  only  repeat  that  the  bulk  of  evidence 
is  in  favor  of  this  automatic  rhythmic  action  of  the  coordinating  centre. 
And  further,  we  must  imagine  that  the  condition  of  the  blood  raises 
or  depresses  the  activity  of  the  main  respiratory  centre,  thereby  increasing 
or  decreasing  both  the  rate  of  the  rhythm  and  the  force  of  the  individual 
respiration.  Both  Miescher  and  Head  have  shown  that  by  over- 
ventilation  of  the  lungs,  and,  therefore,  of  the  blood,  a  condition  of 
apncea  or  arrest  of  the  respiratory  acts  can  be  produced  even  when  both 
vagi  are  cut;  when,  therefore,  the  arrest  is  not  due  to  afferent  impulses 
from  the  lungs,  but  presumably  is  due  to  the  influence  of  the  blood 
upon  the  respiratory  centre.  Similarly,  asphyxia  may  be  brought  about 
by  cutting  off  the  blood  supply  to  the  medulla,  or  by  bleeding  an  animal 
so  as  to  produce  universal  anemia,  and  such  asphyxia,  as  will  be  presently 
noted,  is  characterized  in  its  earlier  stages  by  the  contrary  stage  of 
excessive  force  of  the  respiratory  movements.  The  researches  of  Haldane 
and  Priestley  show  that  it  is  not  want  of  oxygen  but  increased  amount  of 
carbon  dioxide  that  is  the  influence  exciting  the  respiratory  centre  to 
increased  activity. 

1  Arch.  f.  Physiol,  Leipzig,  1898:  340. 


DYSPNCEA  251 

With  this  general  and  brief  review  of  what  is  a  most  complicated  sub- 
ject, we  may  now  pass  on  to  consider  the  more  characteristic  disturbances 
of  the  respiratory  process. 

Sneezing. — Sneezing  is  characteristically  a  reflex  act;  the  usual  cause 
is  from  nasal  irritation,  by  stimulation  of  a  branch  or  branches  of  the 
fifth  nerve;  it  may  be  initiated,  however,  by  exposure  to  intense  light, 
and  thus  by  stimulation  of  the  optic  nerve.  The  act  consists  of  a  spas- 
modic, deep  inspiration,  followed  by  a  strong  expiratory  effort.  While 
this  last  is  proceeding,  the  mouth  passage  is  at  first  closed  by  approxima- 
tion of  the  dorsum  of  the  tongue  to  the  soft  palate.  The  first  portion  of 
the  expired  air  passes,  therefore,  with  considerable  force  through  the 
nostrils,  tending  to  dislodge  irritant  particles  if  they  be  present.  Almost 
immediately  the  tongue  is  slightly  depressed,  so  that  the  remainder  of 
the  air  is  forced  through  a  relatively  narrow  passage  between  the  tongue 
and  the  upper  jaw,  producing  a  characteristic  sound. 

Coughing. — Coughing  may  be  either  a  voluntary  or  a  reflex  act, 
the  reflex  originating  either  from  irritation  in  the  larynx  (most  common), 
in  the  lungs,  or  of  the  pleural  surfaces.  Here  a  deep  inspiration  is  fol- 
lowed by  closure  of  the  glottis,  which  continues  during  the  first  part  of 
the  strong  expiratory  act,  so  that  with  sudden  opening  of  the  glottis, 
the  air  under  considerable  pressure  in  the  main  air  passages  is  liberated, 
and  escapes  with  much  force,  carrying  with  it  mucus  or  other  matter 
in  the  bronchi,  trachea,  or  larynx. 

Dyspnoea. — The  term  dyspnoea  is  used  in  two  senses,  to  indicate 
both  the  sensation  of  air-hunger  and,  more  commonly,  and  we  think 
more  correctly,  the  condition  of  labored  respiration  brought  about  by 
obstruction  to  the  entrance  of  an  adequate  amount  of  air  into  the  lungs, 
and  broadly  by  all  conditions  of  accumulation  of  increased  amounts  of 
CO2  in  the  blood,  with  or  without  deficiency  of  oxygen.  The  condition 
may  or  may  not  be  accompanied  by  cyanosis,  according  to  the  activity 
of  gaseous  interchange  possible  in  the  lungs.  Here  we  have  to  recognize 
the  existence  of  a  protective  mechanism;  whenever  there  is  a  tendency 
to  deficient  ventilation  in  the  lungs,  then  constantly  the  general  metabo- 
lism becomes  lessened  by  all  possible  means;  the  individual  indulges  in 
the  least  possible  muscular  and  other  exercise,  whereby  both  the  call  for 
oxygen  is  brought  to  a  minimum,  as  is  also  the  discharge  of  carbonic 
acid.  Coincidently,  even  in  the  absence  of  sensation  of  air-hunger, 
there  may  be  increase  in  the  rate  of  the  respiratory  act,  the  more  rapid 
if  shallow  inspirations  leading  to  increased  respiratory  interchange, 
and  favoring  thus  a  better  condition  of  the  blood.  When  the  venous 
condition  of  the  blood  becomes  more  aggravated,  such  rapid  respiration  is 
replaced  by  deeper  inspirations  and  slow,  labored  breathing,  correspond- 
ing to  the  first  stage  of  asphyxia.  But  at  the  same  time  it  is  evident  that 
prolonged  relative  venosity  of  the  blood  dulls  the  respiratory  centre;  in 
other  words,  the  subject  of  respiratory  obstruction  may  endure  with 
little  respiratory  distress  a  state  of  venosity  of  the  blood,  which,  sud- 
denly produced  in  a  healthy  person,  would  be  accompanied  by  the  gravest 
respiratory  distress.  This  is  equivalent  to  recognizing — as  from  other 


252     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

considerations  we  must  recognize — that  the  sensitiveness  of  the  respira- 
tory centre  is  capable  of  considerable  variation;  and,  as  a  matter  of  fact, 
there  exists  the  opposite  condition  of  hypersensitiveness  of  the  centre. 
Thus,  we  occasionally  encounter  a  pronounced  dyspnoeic  condition  in 
connection  with  hysteria  and  melancholia,  in  states,  that  is,  in  which 
there  is  no  evidence  of  any  modification  in  the  gaseous  tension  in  the 
blood. 

Keeping  these  matters  in  mind,  we  may  sum  up  the  conditions  under 
which  dyspnoea  may  manifest  itself.  They  are: 

1.  Conditions  of  severe  hindrance  to  entrance  of  normal  amount  of  air: 
(a)  In  the  air  passages,  leading  to  diminished  ingress   and  egress  of 
air  (foreign  bodies,  inflammatory  and  other  narrowings  of  the  larynx, 
trachea,    or   bronchi,   compression    from    without),     (b)  In    the    lungs 
themselves — collapse,  diminution  of  the  ventilating  surface  by  exudates 
into  the  alveoli,  tuberculous  and  other  growths  in  the  lung  substance, 
destruction  of  the  lung  substance  (gangrene,  cavitation,  etc.),  fibroid 
induration,  atrophy,  emphysema. 

2.  Conditions    affecting    the    muscular    mechanism    of    respiration: 
(a)  Diseased  conditions,  inflammation,  etc.,  of  the  diaphragm  or  other 
respiratory  muscles,  more   particularly   the   former.      (6)   Pathological 
condition  of  the  main  and  secondary  respiratory  centres  and  of  the 
nerves  forming  the  respiratory  arc,  paralysis  or  irritation  of  the  centres, 
trauma  or  destruction  of  the  afferent  (vagus)  nerves,  as  again  of  the 
efferent  nerves  (more  particularly  the  phrenics). 

3.  Conditions  obstructing  the  circulation  of  blood:  (a)  Through  the 
lungs  (obstructive  heart  disease,  emphysema,  chronic  interstitial  pneu- 
monia, etc.);  (6)  through  the  medulla. 

4.  Conditions  modifying  the  constitution  of  the  inspired  air — rarefac- 
tion of  the  air  as  at  high  altitudes,  reduction  of  the  amount  of  contained 
oxygen,  increased  CO2  content,  presence  of  carbon  monoxide,  which, 
uniting  with  the  hemoglobin,  permits  less  oxygen  to  be  taken  up,  and 
reduces  thus  the  gaseous  interchange  in  the  medulla  and  tissues  generally. 

Asphyxia. — Dyspnoea  connotes  the  existence  of  at  least  the  minimum 
gaseous  interchange  necessary  to  maintain  life,  the  obstruction  to  that 
interchange  being  overcome  either  by  increased  rate  or  increased  ampli- 
tude of  the  respiratory  acts,  and  the  calling  into  play  of  the  accessory 
muscles  of  the  respiration.  Asphyxia  or  suffocation,  on  the  other  hand, 
connotes  a  condition  in  which  that  minimum  cannot  be  attained,  and 
in  which,  as  a  consequence,  the  progressive  accumulation  of  carbon 
dioxide  in  the  blood  circulating  through  the  medulla  eventually  arrests 
the  action  of  the  respiratory  centre,  and  brings  about  death  unless  the 
obstruction  to  the  gaseous  interchange  be  rapidly  removed.  It  is  true 
that  the  progressive  exhaustion  of  the  respiratory  centre  in  dyspnoea 
through  overstimulation  may  usher  in  a  quiet  death  with  progressive 
cyanosis,  but  little  or  no  struggle.  But  more  often  in  dyspnoea  when  the 
venosity  of  the  blood  becomes  excessive,  and  always  when  the  hindrance 
to  respiration  is  of  sudden  development,  we  obtain  that  intense  respiratory 
struggle  which  forms  the  typical  picture  of  asphyxia.  In  this  acute 


CHEYNE-STOKES  RESPIRATION  253 

condition  three  stages  may  be  recognized :  a  first,  of  increased  amplitude, 
as  well  as  rate  of  the  respiratory  movements;  a  second,  with  increase  of 
the  expiratory  movements  out  of  all  proportion  to  the  inspiratory,  the 
expiration  becoming  prolonged,  the  inspirations  short  and  convulsive. 
To  produce  these  violent  expirations  the  whole  musculature  of  the  body 
appears  to  be  called  into  play.  In  the  final  stage  these  violent  expirations 
reuse  almost  suddenly,  and  now  slow,  deep  inspirations  manifest  them- 
selves. The  mouth  is  widely  open,  the  head  is  stretched  back,  so  as  to 
gain  the  fullest  freedom  for  entrance  of  air  into  the  trachea,  the  body 
stretched,  the  arms  raised.  There  is  now  complete  insensibility;  the 
dilated  pupils  do  not  react,  the  inspiratory  movements  become  farther 
and  farther  apart,  become  weaker  and  weaker,  until,  in  the  course  of 
a  few  minutes  (the  time  varying)  the  last  gasp  is  taken. 

These  stages  are  accompanied  by  changes  in  the  circulation,  of  which 
the  most  marked  is  rise  in  the  blood  pressure.  It  is  evident  that  the 
venous  blood  stimulates  the  vagus  and  vasomotor  centres  in  the  medulla, 
so  that  there  is  both  slowing  of  the  heart  beat  and  contraction  of  the  arteri- 
oles.  With  the  advent  of  the  third  stage,  the  heart  poisoned  with  venous 
blood  begins  to  fail;  its  beats  become  weaker  and  ineffective,  and  death 
occurs  with  all  the  chambers  of  both  sides  hugely  distended,  and  with  the 
blood  pressure  rapidly  sinking.  It  may  be  noted  in  passing  that  during 
the  second  stage,  if  the  blood  pressure  be  taken,  large  secondary  waves 
show  themselves  of  progressive  increase  and  decrease  of  the  general 
blood  pressure,  embracing  many  pulse  waves.  •  These,  the  "Traube- 
Hering  curves,"  indicate  the  existence  of  a  rhythmic  action  of  the 
vasomotor  centres  in  the  medulla.  They  are  of  no  small  interest,  as 
being  analogous  to  the  phenomenon  to  be  immediately  discussed. 

Cheyne-Stokes  Respiration.— The  first  clear  description  of  this 
type  of  respiration  was  given  by  Cheyne,  of  Dublin,  in  1818;  the  first 
full  study  by  Stokes  of  the  same  city  in  1854.  Both  these  physicians 
observed  it  in  connection  with  cases  of  fatty  degeneration  of  the  heart. 
To  quote  Stokes:  "It  consists  in  the  occurrence  of  a  series  of  inspira- 
tions increasing  to  a  maximum,  and  then  declining  in  force  and  length 
until  a  state  of  apparent  apncea  is  established.  In  this  condition  the 
patient  may  remain  for  such  a  length  of  time  as  to  make  his  attendants 
believe  that  he  is  dead,  when  a  low  inspiration,  followed  by  one  more 
decided,  marks  the  commencement  of  a  new  ascending  and  then  descend- 
ing series  of  inspirations.  This  symptom,  as  occurring  in  its  highest 
degree,  I  have  only  seen  during  a  few  weeks  previous  to  the  death  of 
a  patient."  Herein  fuller  study  has  shown  that  although  frequently 
seen  in  association  with  fatty  degeneration  of  the  heart,  and  although 
most  frequently  manifested  in  the  last  few  days  of  life,  periodic  breathing 
of  the  same  order  may  present  itself  in  several  morbid  states,  and  not 
necessarily  as  a  penultimate  event.  As  Traube  pointed  out,  there  are 
two  main  groups  of  conditions  in  which  these  respirations  make  their 
appearance — circulatory  disturbances  with  no  obvious  brain  disease,  and 
intracranial  diseases  without  heart  disease.  Of  the  first  group,  the  most 
common  disorders  are  those  associated  with  chronic  interstitial  nephritis 


254     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

and  arteriosclerosis — cardiac  degeneration,  aortic  and  mitral  stenosis 
and  incompetence,  etc. ;  of  the  second,  cerebral  hemorrhage  and  tumors, 
tuberculous  meningitis,  hydrocephalus,  and  other  conditions  leading  to 
increased  intracranial  pressure,  with  (presumably)  compression  and 
lowered  blood  supply  to  the  medulla.  Yet  a  third  group  should  be 
added,  that  of  the  intoxications,  including  (rare)  cases  of  infection 
(typhoid,  diphtheria,  smallpox,  pneumonia),  and  the  narcotic  (morphine, 
ether,  and  chloroform  in  association  with  morphine,  chloral).  A  mild 
form  of  the  same  type  of  ascending  and  descending  periodic  breathing, 
with  brief  apnoeic  pause,  may  not  infrequently  be  heard  during  the 
afternoon  siesta  of  the  middle-aged  who  have  many  years  of  life  still 
before  them.  According  to  Ede,  the  breathing  of  certain  individuals 
during  sleep  is  constantly  periodic,  and  Gibson1  suggests  that  this  is  a 
familial  peculiarity.  We  encounter  grades  of  the  periodic  breathing 
from  those  in  which  ascent  follows  descent  with  no  absolute  pause,  to 
others  in  which  the  apnoea  may  persist  for  half  a  minute  and  more. 
There  may  or  may  not  be  associated  changes  in  the  general  blood 
pressure,  unconsciousness  during  the  apnoeic  pause,  contraction  and 
dilatation  of  the  pupil. 

In  attempting  to  elucidate  this  peculiar  phenomenon,  Gordon  Douglas 
and  Haldane,2  as  also  Haldane  and  Poulton,3  found  they  could  produce 
it  experimentally  by  breathing  deeply  and  rapidly  for  about  two  minutes. 
Whenever  any  desire  to  breathe  returned,  the  process  was  allowed  to 
take  its  own  course,  with  the  result  that  the  Cheyne-Stokes  type  of 
respiration  was  induced.  The  first-mentioned  observers,  in  studying 
farther  the  intra-alveolar  air  in  regard  to  oxygen  and  carbonic  dioxide 
pressure,  conclude:  (1)  that  the  periodic  breathing  is  excited  by  the 
periodic  occurrence  and  disappearance  of  the  (indirect)  stimulating 
effect  of  want  of  oxygen  on  the  respiratory  centre;  (2)  the  want  of 
oxygen  may  be  due  to  an  abnormal  deficiency  in  the  intra-alveolar 
oxygen  pressure  or  to  the  effect  on  the  circulation  of  changes  in  the 
breathing,  or  to  both  causes  combined. 

There  have  been  abundant  theories,  and  at  times  angry  debate  as 
to  the  meaning  of  this  type  of  respiration;  more  particularly  the  studies 
and  views  of  Traube,  Filehne,  Wellenbergh,  Luciani,  Rosenbach, 
Mosso,  Murri,  and  Gibson  stand  out  prominently.  But  notwithstanding 
all  this  work,  it  cannot  be  said  that  any  consensus  of  opinion  has  been 
reached.  Without  attempting  to  lay  down  any  full  hypothesis,  we  could 
point  out: 

1.  That  the  phenomena  and  the  records  taken  of  those  phenomena 
have  essentially  the  characters  of  what  physicists  term  interference 
curves,  i.  e.,  of  a  series  of  waves  of  one  rhythm  modified  by  the  super- 
position of  waves  of  another  rhythm,  which  at  one  period  augment, 
at  another  neutralize,  each  other. 

1  Cheyne-Stokes  Respiration,  Edinburgh,  Oliver  and  Boyd,  1892:  122.    This  work 
gives  a  full  bibliography  of  the  subject  up  to  date,  with  discussion  of  the  many 
theories. 

2  Journ.  of  Physiol.,  38 : 1909 : 401 .  3  Medico-Chir.  Trans.,  40 : 1907 : 49. 


THE  AIR  SACS  255 

2.  That   there  is  a  curious  likeness  between    these  Cheyne-Stokes 
curves  and    those  of   certain  forms  (the  so-called  "extra -systolic")  of 
periodic  cardiac  irregularity;  and  that,  if  in  the  latter  case  we  con- 
clude that  in  addition  to  evidence  of  the  manifestation  of  an  automatic 
rhvthm,  there  are  manifested  opposite  phases  of  muscular  contraction 
and  dilatation,  so  in  connection  with  the  respiratory  act  we  have  similar 
indications. 

3.  To  the  existence  of  an  automatic  rhythm  reference  has  already 
been  made  (p.  250).     Whether  this  is  truly  automatic,  or  whether  it  rep- 
resents the  rhythmic  resultant  of  stimuli  other  than  those  proceeding 
from  the  lungs  must,  for  the  present,  be  left  an  open  question.     What 
is  of  import  is  that  in  the  "absence  of  normal  vagus  stimuli  the  centre  has 
been  demonstrated  to  initiate  rhythmic  contractions. 

4.  The  respiratory  cycle  is  what  may  be  termed  a  double-phase  act: 
inspiration  demands  that  expiratory  stimuli  be  inhibited,  and  vice  versa. 
The  inspiratory  contraction  with  the  succeeding  expiratory  contraction 
may  be  compared  with  the  cardiac  muscular  contraction  with  the  suc- 
ceeding  refractory   period,   which,   as   we  have  pointed   out,   may  be 
regarded  as  indicating  active  dilatation  of  the  cardiac  fibers. 

5.  As  in  connection  with  cardiac  irregularity,  so  here,  we  may  presume 
that  want  of  coordination  between  these  factors  and  the  establishment 
of  interfering  rhythms  is  at  the  bottom  of  the  production  of  these  periodic 
alterations  in  rate  and  volume  of  the  respiratory  curves. 

6.  There   are   other   forms   of   irregular   breathing,    not   possessing, 
on  the  one  hand,  the  marked  periodic  character  of  the  Cheyne-Stokes 
type,  and  on  the  other  hand,  not  manifesting  the  progressive  ascent  and 
descent  in  the  amplitude  of  the  contractions.    Thus,  some  would  separate 
what  is  termed  "Biot's  respiration."    This  is  seen  in  meningitis  and  some 
other  cases  of  brain  disease,  and  is  characterized  by  irregularly  periodic 
periods  of  apnoea,  lasting  for  many  seconds  up  to  half  a  minute.     These 
may  be  encountered  in  uremia,  diabetes,  and  sundry  nervous  states, 
as  again  under  the  action  of  certain  drugs.     Here  presumably  stimuli 
of  another  type  act  upon  the  main  respiratory  centre.     Still  less  is  known 
regarding  the  mode  of  development  of  these  than  of  the  Cheyne-Stokes 
type. 

THE  AIR  SAGS. 

We  shall  in  the  next  chapter  describe  in  detail  the  various  disorders 
which  may  affect  the  air  sacs.  Here  we  have  to  consider  the  broad 
effects  of  these  disorders  upon  the  work  of  the  lung  and  upon  the  system 
in  general.  Speaking  generally,  these  disorders  range  themselves  into 
two  classes :  those  in  which  more  particularly  the  entrance  into  and  egress 
from  the  air  sacs  of  the  proper  amount  of  air  is  prevented,  and  those  in 
which  changes  occurring  in  the  walls  of  the  air  sacs  prevent  the  air 
which  has  entered  them  from  being  properly  utilized  for  purposes  of 
gaseous  interchange.  There  are  not  a  few  cases  in  which  diseases  of  the 
first  group  lead  to  the  development  of  disorders  of  the  second ;  we  might 


256     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

thus  constitute  a  third  group  of  the  combined  disorders.  It  will,  how- 
ever, be  simpler  to  consider  the  cases  according  as  one  or  other  of  the 
two  main  orders  is  primary.  Leaving  out  of  consideration  those  cases 
in  which  obstruction  occurs  in  the  air  passages,  the  entrance  of  a  due 
amount  of  air  into  the  sacs  may  be  prevented  either  by  want  of  distension 
of  the  sacs,  as  in  atelectasis  and  collapse,  or  by  the  collection  within 
them  of  fluid  or  solid  matter.  By  atelectasis  is  understood  the  condition 
of  primary  imperfect  expansion  of  the  air  sacs  (dre^'ci  incomplete; 
ixrdmz,  expansion),  by  collapse,  the  closure  of  air  sacs  which  have 
previously  been  expanded,  although — we  are  inclined  to  think  incorrectly 
— the  term  atelectasis  is  coming  to  be  used  for  both  conditions.  The 
former  condition  thus  dates  from  the  moment  of  birth,  the  latter  may  be 
brought  about  either  by  complete  bronchial  obstruction  with  subsequent 
absorption  of  the  air  already  contained  in  the  air  sacs,  or  more  commonly 
by  compression  from  without,  as  by  the  pressure  of  air  (pneumo thorax), 
fluid  matter,  the  contraction  of  inflammatory  organized  products  either 
on  the  surface  or  within  the  tissues  of  the  lungs,  the  pressure  of  granulo- 
matous  and  other  new-growths.  If  of  limited  extent,  neither  condition 
produces  any  recognizable  disturbance;  the  surrounding  unobstructed 
air  sacs  undergo  a  compensatory  enlargement;  nay,  more,  the  whole  of 
one  lung  may  undergo  collapse,  and  if  the  other  be  healthy  it  is  able  to 
suffice  for  the  needs  of  the  organism.  Only,  as  already  noted,  if  the  one 
lung  be  put  out  of  action  suddenly,  there  is  a  period  of  tumultuous 
breathing  and  dyspnoea  before  adaptation  to  the  changed  circumstances 
becomes  complete,  while,  further,  the  respiratory  system  is  working 
perilously  near  to  the  limit  of  its  reserve  force,  so  that  bronchial  and 
other  disturbances,  of  little  import  when  both  lungs  are  functional,  now 
assume  a  serious  aspect. 

The  air  sacs  may  become  filled,  and  the  air  they  should  contain  be 
replaced,  (1)  by  serous  fluid,  as  in  cases  of  acute  or  chronic  congestion; 
(2)  by  blood,  as  in  rupture  of  an  aneurism  of  one  of  the  branches  of  the 
pulmonary  artery  in  a  tuberculous  cavitation,  or  of  an  aortic  aneurism 
into  the  trachea,  from  an  infarct,  etc. ;  (3)  by  water  or  other  fluid  from 
without,  as  in  cases  of  drowning;  (4)  by  inflammatory  exudates  and 
migrated  cells,  as  in  pneumonia.  The  effects  upon  respiration  are  of  the 
same  order  as  in  the  former  group  of  cases;  whether  there  be  no  noticeable 
results,  or  rapid  respiration,  dyspnoea,  and  eventual  adaptation,  or 
the  development  of  asphyxia  and  death,  depends  primarily  upon  the 
extent  of  lung  tissue  involved,  secondarily,  upon  the  causative  agent. 
The  diffusion  of  the  products  of  coagulation  and  death  of  the  erythrocytes 
in  cases  of  hemorrhage,  and  of  toxins  and  cytolytic  products  in  cases  of 
pneumonia,  sets  up  a  febrile  state,  and  doing  this,  inter  alia,  directly 
affects  the  respiratory  centre,  inducing  an  increased  rate  of  breathing 
out  of  relationship  to  the  extent  of  lung  substance  involved.  Further, 
it  must  be  remembered  that  an  acute  lobar  pneumonia  is  always  a  pleuro- 
pneumonia,  and  that  the  painful  irritation  of  the  pleural  surface  inhibits 
the  excursion  of  the  thorax;  here,  again,  is  a  cause  of  the  rapid  and 
shallow  respirations  present  in  this  condition.  But  even  in  acute  lobar 


EMPHYSEMA  257 

pneumonia  the  exudate  may  completely  distend  all  the  air  sacs  of  a 
whole  lung,  or  the  lower  lobes  of  both  lungs,  and  bring  about  complete 
displacement  of  the  air  in  the  same,  without  a  fatal  result,  or,  more 
accurately,  at  autopsy  wre  may  encounter  cases  showing  a  distribution 
of  the  pneumonic  disturbance,  which  has  evidently  been  of  some  days' 
duration,  and  in  which  the  lethal  event  is  seen  to  be  due  to  recent  and 
further  extension  of  the  process  beyond  these  limits.  Certain  distinctions 
of  some  practical  importance  must  be  drawn  between  the  fluid  agents 
and  those  which,  like  blood  and  inflammatory  exudates,  are  liable  to 
coagulation  and  solidification.  In  the  first  place  the  site  of  accumulation 
of  fluid  depends  upon  the  position  of  the  individual;  these  naturally 
accumulate  in  the  more  dependent  parts  of  the  lungs,  and,  indeed,  by  turn- 
ing the  individual  upon  one  or  other  side,  they  may  drain  from  one  side 
to  the  other,  often  with  grave  disturbance  of  the  respiratory  act  in  the 
process.  Or,  as  in  Sylvester  and  Schafer's  methods  of  resuscitation  from 
drowning,  the  fluids  can  be  drained  out  through  the  trachea.  In  the 
second  place,  when  the  air  sacs  become  distended  with  solid  contents, 
there  is  the  greater  interference  with  the  circulation.  In  collapse  and 
atelectasis  the  absence  of  the  air  pressure  within  the  alveoli,  as  again 
the  lack  of  distension  of  the  same,  and  of  consequent  flattening  and 
elongation  of  the  capillaries  within  their  walls,  induces  a  dilatation  of  the 
bloodvessels  of  the  affected  area,  and  consequent  free  flow  of  blood 
through  the  same.  When  serous  fluid  percolates  into  the  air  sacs,  then 
also  we  find  a  congestion,  either  active  or  passive.  When  passive,  it 
is  not  the  lung  condition,  but  obstruction  to  the  onflow  of  blood  from 
mitral  or  other  obstructive  disease  in  the  left  heart  that  is  the  cause  of 
the  great  dilatation  of  the  capillaries,  and  this  dilatation  continues, 
however  advanced  the  serous  accumulations  within  the  alveoli.  In 
pneumonia  and  hemorrhagic  infarcts,  with  coagulation  arid  progressive 
passage  of  more  material  into  the  air  sacs,  these  become  overdistended, 
and  the  result  now  is  that  eventually,  from  great  congestion,  we  pass  to 
a  state  of  narrowing  and  compression  of  the  interalveolar  capillaries, 
and  when  the  conditions  affect  any  large  portion  of  the  lungs,  the  result 
i  s  well-marked  increase  in  the  work  of  the  right  heart,  with  dilatation  of 
the  same,  and  some  incompetence.  It  is  remarkable  how  relatively 
rare  are  local  necrosis  and  gangrene  of  the  affected  lung  as  the  result  of 
this  induced  anemia;  nevertheless  these  conditions  occasionally  show 
themselves. 

Changes  in  the  Walls  of  the  Air  Sacs  Hindering  Due  Aeration. 
—These  changes  may  be  of  two  orders,  either  atrophic,  or  of  the  nature 
of  interstitial  deposits  in  the  alveolar  walls.  The  first  of  these  leads  to 
or  accompanies  the  not  uncommon  condition  of  either  localized  or  gener- 
alized vesicular  emphysema.  As  will  be  more  fully  described  in  the 
next  chapter,  we  find  in  these  states  a  great  dilatation  of  the  air  sacs, 
with  pronounced  thinning  of  the  interalveolar  septa,  leading  to  atrophy 
of  the  same,  with  fusion  of  contiguous  air  sacs  into  large  chambers, 
a  process  inevitably  accompanied,  not  only  by  reduction  of  the  respiratory 
surface,  but  also  by  a  harmful  alteration  in  the  relationship  between  the 
17 


258      THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

mass  of  air  within  the  alveoli,  and  the  surface  of  the  same,  and  so  hin- 
drance of  the  gaseous  interchange  with  the  blood  in  the  alveolar  walls. 
Emphysema. — To  understand  the  ill  effects  of  the  emphysematous  con- 
dition, it  is  necessary  to  realize  the  mechanics  of  its  production.     Let  us 

FIG.  62 


Emphysema  of  lung.      Leitz  obj.  No.  7,  without  ocular.      The  atrophied  alveolar  walls  and  the 
rupture  of  several  of  them  are  well  shown.      (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

FIG.  63 


Diagram  to  demonstrate  the  effect  of  emphysematous  dilatation  of  the  air  sac  upon  the  epi- 
thelial lining  of  the  air  sac  (a)  and  upon  the  capillary  network  of  the  alveolar  walls  (6).  A, 
normal  air  sac;  B,  expanded  air  sac. 

take  first  the  condition  of  localized  emphysema,  in  which  the  change 
affects  one  small  bronchial  tree  and  its  associated  air  sacs,  the  rest  of  the 
lung  being  in  a  normal  state.  It  is  obvious  here  that  the  primary  con- 
dition has  involved  the  bronchus.  We  can  best  explain  the  development 


EMPHYSEMA  259 

by  presuming  a  narrowing  of  the  lumen  of  the  bronchus,  whether  con- 
genital or  induced  by  inflammatory  thickening,  or  by  the  presence  of  thick 
exudate  within  it.  In  such  a  case  the  forcible  nature  of  the  inspiratory 
act  draws  air  into  the  associated  air  sacs ;  the  passive  nature  of  the  expira- 
tory act  may  prevent  an  equal  amount  of  air  becoming  expired.  The 
result  will  be  that  with  successive  acts  of  inspiration,  the  air  sacs  will 
become  more  and  more  distended,  as  demonstrated  by  Brodie  and 
Dixon's  experiments,  already  noted  (p.  243).  There  is,  it  is  true,  an 
active  force  opposing  overdistension,  namely,  the  fairly  abundant  elastic 
tissue  in  the  alveolar  walls.  This  force  is  most  effective  when  the  air 
sacs  are  in  a  state  of  distension;  it  is  of  little  effect  when  the  alveoli  are 
only  moderately  filled.  Time  and  again  at  autopsy  we  notice  that  a  very 
slight  grade  of  bronchitis  suffices  to  prevent  the  postmortem  collapse  of 
the  lungs.  Thus,  under  the  condition  postulated,  both  the  expiratory 
act  and  the  elasticity  of  the  alveolar  walls  are  unable  to  reduce  the 
air  sacs  to  the  collapsed  expiratory  state  (Fig.  63).  If  the  state  of 
distension  be  kept  up,  it  materially  affects  the  abundant  capillary  net- 
work of  the  alveolar  wall.  When  that  wall  is  collapsed,  the  pressure 
from  without  upon  the  capillaries  is  reduced,  they  are  circular  in 
section,  and  there  is  a  minimal  obstruction  to  the  onflow  of  blood.  When, 
on  the  contrary,  the  air  sac  is  distended,  not  only  do  the  capillaries  become 
stretched,  but,  in  addition,  they  are  subjected  to  increased  pressure  from 
the  contained  air.  The  result  is  that  the  individual  capillaries  become 
flattened  and  elliptical  in  section;  not  only  can  they  contain  less  blood 
but  that  smaller  amount  of  blood  is  exposed  to  a  larger  surface,  and 
consequently  to  increased  friction.  Thus,  if  the  distension  be  continued, 
the  result  is  continued  malnutrition  of  the  alveolar  wall,  reduced  gaseous 
interchange,  atrophy  of  the  wall,  diminished  elastic  tissue,  and  diminished 
elasticity;  yet  further  dilatation,  with  fusion  of  the  air  sacs,  until  now, 
even  if  the  obstruction  be  removed  and  the  ingress  and  egress  of  air  be 
restored,  the  atrophy  of  the  tissue,  and  the  want  of  proportion  between 
the  volume  of  the  air  in  the  greatly  dilated  system  and  the  cross-section 
of  the  emergent  bronchioles  render  the  emphysematous  condition 
permanent,  so  that  in  an  otherwise  healthy  lung  we  may  encounter  thin- 
walled  translucent  sacs  with  occasional  thin  septa  crossing  them  which 
may  attain  to  the  size  of  a  pigeon's  egg  or  larger,  and  project  prominently 
from  the  surface  of  the  organ.  A  similar  state  may  be  brought  about  by 
somewhat  different  means  in  the  condition  of  compensatory  emphysema. 
Here,  with  collapse  of  the  air  sacs  connected  with  certain  bronchi,  the 
force  of  the  inspiratory  negative  pressure  leads  to  an  overdistension  of 
air  sacs  connected  with  other  bronchi,  and  especially  of  such  air  sacs 
— e.  g.,  those  at  the  edges  of  the  lobes — as  are  not  supported  by  mutual 
compression.  Such  extreme  distension  is  apt  to  overstrain  the  elastic 
tissues  of  the  alveolar  walls  and  bring  about  atrophy. 

Now  these  same  conditions  must  be  operative  in  causing  generalized 
emphysema.  In  perhaps  the  majority  of  cases  microscopic  examination 
reveals  the  existence  of  a  chronic  bronchitis  and  peribronchitis,  i.  e., 
suggests  that  the  main  causative  factor  has  been  a  diminution  of  the 


260     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

lumen  of  entrance  and  exit  of  air  to  and  from  the  air  sacs.  But  in  other 
cases,  as  in  children  after  prolonged  whooping-cough,  in  glassblowers, 
players  upon  wind-instruments,  the  obstruction  has  been  situated 
higher  up  in  the  respiratory  passages.  But  in  many  of  these  cases  also  it 
is  to  be  noted  that  the  condition  develops  with  advancing  life;  i.  e., 
just  as  in  the  vessels,  so  in  the  lungs,  there  is  a  progressive  diminution 
in  the  elasticity,  and  an  atrophy  of  the  elastic  connective  tissues,  so  that 
the  lung-tissue  does  not  sufficiently  aid  the  thoracic  wall  in  bringing 
about  expiratory  contraction  of  the  lungs. 

The  effects  of  such  generalized  emphysema  are  thus:  generalized 
expansion  of  the  lungs,  coincident  enlargement  of  the  thorax,  which 
assumes  a  characteristic  barrel  shape  (the  "air  hunger"  demands  con- 
stantly the  entrance  of  more  air  into  the  already  overfilled  air  sacs,  and 
therewith  increased  inspiratory  expansion  of  the  thorax),  prolonged 
expiration,  diminished  gaseous  interchange,  obstruction  to  the  passage  of 
blood  through  the  lungs,  hypertrophy  and  dilatation  of  the  right  heart, 
and  eventually  dyspnoea  and  cardiac  failure.  , 

How,  it  may  be  asked,  does  this  conception  agree  with  the  various 
views  that  have  been  enunciated  regarding  the  etiology  of  vesicular 
emphysema?  Laennec  was  of  opinion  that  mucus  in  the  lumen  of  the 
bronchi  exercised  a  ball-valve  action  admitting  the  entry,  but  preventing 
the  discharge  of  air  from  the  air  sacs.  This,  it  will  be  recognized,  is  a 
possible  cause  of  one  series  of  cases.  Gairdner,  in  1857,  held  that  col- 
lapse, either  complete  or  partial,  of  one  portion  of  the  lung  was  a 
necessary  antecedent  of  emphysema  in  another  part;  that  the  condition 
was  compensatory.  This,  again,  can  be  applied  to  only  one  series  of 
cases,  and  cannot  explain  diffuse  emphysema.  Of  more  importance 
are  the  views  of  Mendelssohn,  followed  by  Jenner,  that  obstructed 
expiration  is  the  essential  cause.  They  showed  clearly  that  the  emphy- 
sematous  condition  followed  violent  expiratory  effort,  and  pointed  out 
that  the  increased  pressure  in  the  alveoli  must  tend  to  affect  most  those 
portions  of  the  lung  which  have  least  support  from  their  surroundings, 
and  that  so,  as  a  matter  of  fact,  emphysema  shows  itself  most  extensively 
along  the  edges.  This  view,  however,  leaves  out  of  account  the  nature 
of  the  lung  substance  as  a  factor  in  the  development  of  the  condition. 
J.  Jackson,  Jr.,  of  Boston,  was  the  first  to  direct  attention  to  an  inherited 
liability  to  emphysema,  and  to  the  fact  that  it  is  more  common  in  the 
young  than  is  usually  supposed.  This  seemed  to  point  to  some  de- 
velopmental defect  in  the  lung  tissue  as  a  factor.  Cohnheim  concluded 
that  the  explanation  was  to  be  sought  in  a  congenital  imperfect  formation 
of  the  elastic  tissue,  basing  the  view  upon  experiments  which  demon- 
strated the  singular  loss  of  elasticity  of  the  lung  in  advanced  emphysema. 
Eppinger,  admitting  this  loss  of  elasticity,  held  that  in  the  majority 
of  cases  it  was  a  secondarily  induced,  and  not  a  primary  condition. 
Lastly,  Isaaksohn  regarded  the  disease  as  essentially  due  to  vascular 
disturbance.  As  we  have  pointed  out,  the  vascular  disturbance  naturally 
follows  persistent  distension  of  the  alveoli.  It  will  be  seen  that  our  con- 
ception of  the  nature  of  the  process  takes  into  account  both  the  factors 


MODIFICATIONS  IN  THE  CONSTITUTION  OF  INSPIRED  AIR    201 

which  may  be  regarded  as  clearly  established,  namely,  (1)  increased 
difficulty  of  expiration,  leading  to  raised  intra-alveolar  pressure,  and 
(2)  diminished  elasticity  of  the  alveolar  wall.  It  must  be  freely  admitted 
that  these  interact,  and  that  one  or  the  other  may  be  the  more  prominent 
in  the  early  stage.  Thus  a  congenital  deficiency  in  elasticity  will  render 
a  relatively  slight  obstruction  to  expiration  the  more  effective,  while, 
contrariwise,  in  the  lung  possessing  well-marked  elasticity,  prolonged 
distension  of  the  alveoli  eventually  brings  about  atrophy  of  the  elastic 
tissue.  Further,  it  has  to  be  noted  that  the  elastic  tissues  of  the  body 
are  liable  to  lose  their  elasticity  with  advancing  age,  and  that  so,  condi- 
tions which  have  little  effect  in  early  life  favor  the  development  of  em- 
physema at  a  later  period;  this  loss  of  elasticity  is  a  characteristic  feature 
of  arteriosclerosis;  we  are  thus  prepared  to  find  that  emphysema  and 
arteriosclerosis  are  very  commonly  associated  conditions. 

Bronchiectasis. — Here,  in  passing,  may  be  noted  the  contrasted  condi- 
tion of  bronchiectasis,  in  which,  through  atrophy  of  their  walls  or,  again, 
through  tension  exerted  upon  them  from  without  by  the  contraction 
of  interstitial  fibrous  tissue,  the  bronchi  and  bronchioles  undergo  dila- 
tation. Whether,  as  in  the  latter  category  of  cases,  the  alveoli  have 
undergone  a  coincident  compression  by  the  interstitial  tissue  or  not,  the 
dilated  bronchi  take  the  space  that  should  be  occupied  by  functionating 
air  sacs.  Here  also  the  result  is  a  diminished  aeration  of  the  blood. 
The  different  forms  will  be  found  described  on  p.  281. 

Interstitial  Deposits. — New  tissue,  whether  fibrous,  as  in  chronic 
interstitial  pneumonia;  granulomatous,  as  in  tuberculosis,  syphilis, 
actinomycosis ;  or  neoplastic,  leads  to  compression  and  diminution,  if  not 
absolute  occlusion  of  the  air  sacs,  and  in  addition  affords  a  mechanical 
resistance  to  their  expansion  during  inspiration.  According,  therefore, 
to  the  extent  of  the  development  of  these  interstitial  deposits  within  the 
lung  substance,  so  do  we  obtain  greater  or  less  reduction  in  the  functional 
capacity  of  the  lungs.  Nor  has  the  amount  only  of  the  new  tissue  to  be 
taken  into  consideration,  and  the  lessened  amount  of  air  which  in  conse- 
quence can  be  inspired.  Such  interstitial  deposits  gravely  obstruct  also 
the  pulmonary  circulation;  interstitial  fibrous  tissue,  as  it  contracts, 
obliterates  largely  the  capillaries  of  the  alveolar  walls;  the  granulomas 
and  tumors,  as  they  grow,  obliterate  the  surrounding  air  sacs,  and  if 
situated  within  the  lung  substance  rather  than  at  the  surface,  obstruct  the 
blood  supply  of  the  tissue  lying  between  them  and  that  surface. 

Modifications  in  the  Constitution  of  the  Inspired  Air.— This  is 
a  subject  that  is  treated  so  fully  in  the  larger  text-books  of  physiology 
that  here  it  is  only  necessary  to  recall  the  main  facts  that  have  been 
ascertained.  Oxygen  is  the  essential  constituent  of  the  inhaled  air. 
In  the  normal  animal  this  may  be  materially  increased  in  amount  without 
modification  of  the  respiratory  exchange;  nay,  if  the  animal  breathe 
pure  oxygen  instead  of  air,  there  is  little  immediate  effect,  metabolism 
being  regulated  by  the  needs  of  the  tissues,  and  not  by  the  amount 
of  oxygen  presented.  When,  however,  the  blood  comes  to  contain  one- 
third  more  oxygen  than  normal,  metabolism  is  arrested  and  the  animal 


262     THE  RESPIRATORY  FUNCTION  AND  ITS  DISTURBANCES 

dies.  Such  increase  may  be  brought  about  by  inhalation  of  the  gas  under 
pressure,  and,  as  shown  by  Paul  Bert,  if  this  pressure  be  increased  to 
six  atmospheres,  the  animal  dies  in  violent  convulsions.  The  same 
effects  may  be  induced  by  breathing  air  under  pressure,  although  here 
the  pressure  has  to  be  raised  to  a  much  greater  extent  in  order  to  attain 
a  partial  pressure  of  the  contained  oxygen  of  the  same  value.  As  shown 
recently  by  Leonard  Hill,  where  the  individual  through  muscular  exercise 
— as  in  runners  and  those  engaged  in  football  matches — is  in  need  of 
oxygen,  the  effects  of  inhaling  pure  oxygen  are  immediate,  relieving  the 
hyperpnoea  (i.  e.,  rapid,  labored  breathing)  and  fatigue.  The  same  is  true 
when  the  respiratory  surface  is  diminished,  as  in  pneumonia.  The  oppo- 
site condition  of  diminished  atmospheric  pressure  produces  its  results  by 
diminution  of  the  partial  pressure  of  the  oxygen,  and  so  of  the  amount 
of  this  gas  which  can  diffuse  or  otherwise  gain  entrance  into  the  blood. 
When  the  atmospheric  pressure  is  reduced  to  half  an  atmosphere,  marked 
discomfort  is  felt,  with  dyspnoea  and  rapid  breathing;  with  reduction  to 
250  mm.,  the  symptoms  become  violent,  with  profound  diminution  of 
the  oxygen  in  the  arterial  blood,  convulsions,  insensibility,  and  death. 
Similar  results  are  produced  by  maintaining  the  normal  atmospheric 
pressure,  while  reducing  the  amount  of  contained  oxygen,  i.  e.,  reducing 
the  partial  pressure  of  the  oxygen.  An  air,  for  example,  that  contains 
only  5  per  cent,  of  oxygen  produces  insensibility  in  man  within  a  minute. 

The  nitrogen  of  the  air  is  wholly  inert,  and  variations  in  its  amount  are 
without  effect  so  long  as  the  partial  pressure  of  the  oxygen  is  maintained. 
Though  here,  again,  as  in  caisson  disease,  with  increase  in  atmospheric 
pressure,  an  increased  amount  is  taken  up  by  the  blood,  and  if  the  press- 
ure be  suddenly  removed,  the  liberation  of  the  gas  in  the  capillaries  in 
gaseous  form  leads  to  profound  and  often  fatal  results.  Hydrogen 
is  similarly  inert;  animals  can  breathe  without  apparent  ill  effects 
a  mixture  of  equal  parts  of  oxygen  and  hydrogen.  Argon  is  also  stated 
to  be  inactive. 

Carbon  Dioxide. — Excess  of  this  gas  in  the  air  produces  effects 
more  rapidly  than  does  deficiency  in  oxygen,  or  otherwise,  to  this  gas 
more  particularly  are  due  the  deleterious  effects  of  air  vitiated  by  being 
breathed  over  again  in  a  confined  space  without  ventilation.  The 
figures  given  by  different  observers  for  the  maximum  amount  of  this  gas 
which  can  be  inspired  without  discomfort  vary  somewhat  widely.  The 
most  accurate  appear  to  be  those  of  Haldane  and  Lorrain  Smith.  They 
found  that  the  presence  of  18.6  per  cent,  induced  in  them  hyperpnoea 
and  distress,  with  flushing,  cyanosis,  and  mental  confusion,  and  this 
within  a  minute  or  two,  and  that  when  the  carbon  dioxide  in  vitiated  air 
rose  to  from  3  to  4  per  cent.,  symptoms  of  hyperpnoea  and  distressed 
breathing  gradually  developed.  On  the  other  hand,  hyperpnoea  from 
defect  of  oxygen  only  showed  itself  when  the  oxygen  was  reduced  to  12 
per  cent,  in  one  individual,  to  6  per  cent,  in  another.  The  volatile 
substances  exhaled  or  given  off  from  the  skin,  whose  presence  is  so  pro- 
nounced in  the  air  of  crowded  and  ill-ventilated  rooms,  are  doubtfully 
toxic,  but  undoubtedly  they  cause  discomfort  in  breathing.  Pure 


CARBON  MONOXIDE  POISONING  263 

carbon  dioxide  is  irrespirable ;  it  causes  an  immediate  spasm  of  the 
glottis.  The  cyanosis  and  toxic  effects  of  the  gas  are  due  to  its  accumula- 
tion in  the  blood;  where  the  amount  in  the  inspired  air  is  excessive,  there, 
instead  of  being  discharged  from  the  blood,  it  is  absorbed  from  the 
pulmonary  air.  Speck  found  abundant  absorption  when  the  air  con- 
tained 11.51  per  cent.  CO2. 

Other  gases,  like  ammonia  and  nitric  oxide,  are  irrespirable  even  in 
small  amounts,  in  consequence  of  the  spasm  of  the  glottis  induced  by 
them.  Yet  others,  like  nitrous  oxide,  carbon  monoxide,  and  hydrogen 
sulphide,  can  be  breathed  and  undergo  absorption,  producing  specific 
effects.  Of  these,  the  most  important  is  carbon  monoxide,  and  this 
because  the  hemoglobin  of  the  corpuscles  has  an  intense  avidity  for 
the  same,  combining  with  it  to  the  exclusion  of  oxygen.  As  shown  by 
Haldane,  symptoms  manifest  themselves  when  the  corpuscles  become 
about  one-third  saturated,  and  become  urgent  when  they  are  half  satu- 
rated. These  symptoms  are  identical  with  the  effects  of  reducing  the 
amount  of  oxygen  in  the  respired  air.  With  the  presence  of  0.05  per 
cent,  of  this  gas  in  the  air,  symptoms  show  themselves;  when  the  per- 
centage rises  to  0.2  they  become  severe.  It  is  this  carbon  monoxide 
poisoning  that  is  the  cause  of  the  frequent  deaths  from  inhalation  of 
coal  gas,  from  the  fumes  of  charcoal  and  coke  fires,  kilns,  and  noxious 
gases  in  coal  mines,  more  especially  after  explosions. 


CHAPTEE    XII. 

THE  RESPIRATORY  PASSAGES. 

THE  NOSE. 
CONGENITAL  ANOMALIES. 

THE  most  common  anomaly  is  an  asymmetrical  position,  generally 
due  to  deviation  of  the  bony  septum,  a  condition  which  is  present  in 
more  than  50  per  cent,  of  individuals.  Malformations  of  high  grade, 
however,  are  almost  invariably  associated  with  other  defects  of  the  face. 
For  example,  in  the  condition  known  as  cyclops  the  nose  may  be  absent 
or  rudimentary.  One  or  more  of  the  turbinated  bones  may  be  absent, 
the  anterior  or  posterior  nares  may  be  occluded,  or  there  may  be  defect 
in  the  alse  or  the  floor  of  the  nasal  cavity.  The  last-mentioned  condition 
may  be  found  associated  with  harelip  or  cleft-palate. 

CIRCULATORY  DISTURBANCES. 

Owing  to  its  excessive  vascularity,  the  nasal  mucosa,  particularly  the 
erectile  portion  of  it  in  the  neighborhood  of  the  lowest  turbinal,  is 
specially  liable  to  sudden  and  extreme  disorders  of  circulation. 

Hyperemia. —  Passive  Congestion. — Passive  congestion  is  found  in 
heart  and  lung  diseases,  and  in  consequence  of  the  presence  of  tumors 
in  the  nasal  cavity. 

Active  Hyperemia. — Active  hyperemia  is  common  at  the  commence- 
ment of  inflammatory  processes  and  in  some  infective  fevers,  such  as 
measles,  typhoid,  and  influenza.  It  very  readily  leads  to  rupture  and 
hemorrhage  (epistaxis). 

Hemorrhages. — Hemorrhages  are  also  common  in  hemophilia,  leuke- 
mia, death  from  suffocation,  and  as  a  result  of  trauma.  Of  more  than 
ordinary  interest  are  the  cases  of  vicarious  hemorrhage  taking  the  place 
of  ordinary  menstruation.  In  fact,  there  seems  to  be  some  sympathetic 
relationship  between  the  nasal  mucosa  and  the  sexual  organs,  for  during 
sexual  excitement  the  membrane  becomes  turgescent. 

(Edema. — (Edema  is  a  frequent  result  of  inflammation. 

INFLAMMATIONS. 

Acute  Catarrh  or  Coryza  (Acute  Rhinitis).— Acute  rhinitis  occurs 
as  a  primary  affection  which  is  usually  attributed  to  the  effects  of 
exposure  to  cold  and  wet,  and  to  bacterial  influences.  It  is  probable 


RHINITIS  265 

that  individual  peculiarities  are  also  operative.  Chemical  irritants, 
such  as  ammonia,  formalin,  nitric  and  osmic  acids,  can  induce  severe 
rhinitis.  Some  people  are  very  susceptible  to  the  influence  of  certain 
volatile  substances,  like  ipecacuanha,  the  scent  of  certain  flowers  or 
anim.ils,  pollen,  etc.  To  this  group  belongs  hay  fever,  which  seems  to 
be  due  to  personal  idiosyncrasy  of  this  kind.  Coryza  is  met  with  also 
as  a  complication  of  many  infections,  as  measles,  typhus  fever,  and 
influenza. 

The  condition  is  often  important,  as  the  inflammation  may  spread  to 
the  accessory  cavities  and  sinuses,  to  the  throat,  to  the  Eustachian  tubes, 
and  middle  ear.  In  acute  coryza  the  mucosa  is  swollen,  hyperemic, 
of  a  deepened  color,  with  often  considerable  thickening,  and  feels  dry 
and  irritable.  After  this  first  stage  there  is  an  abundant  secretion  of  a 
clear,  watery,  very  slightly  viscid,  irritating  fluid,  which  contains  leuko- 
cytes and  ciliated  epithelium.  Erosion  of  the  edges  of  the  nose  and 
upper  lip  is  frequently  the  result  of  its  irritating  qualities. 

Purulent  Rhinitis. — This  form  may  develop  into  a  purulent  rhinitis. 
It  is  characterized  by  greater  inflammatory  reddening  and  swelling  of 
the  mucosa,  with  an  abundant  purulent  exudate,  often  mixed  with 
blood,  and  having  a  foul  odor.  It  may  lead  to  collections  of  pus  in  the 
various  accessory  cavities  (e.  g.,  empyema  of  the  antrum  of  Highmore). 
The  suppurative  process  may  extend  into  the  deeper  parts,  leading  to 
the  formation  of  local  abscesses  and  erosion  of  bone  or  cartilage.  Sup- 
purative rhinitis  may  occur  as  a  primary  infection,  or  may  be  a  compli- 
cation of  infectious  diseases,  such  as  scarlatina,  variola,  and  diphtheria. 

Croupous  and  Phlegmonous  Inflammation. — Croupous  and  phlegmonous 
inflammation  are  also  described. 

Chronic  Rhinitis. — Chronic  rhinitis  occurs  frequently  in  those  of  a 
phthisical  or  syphilitic  habit,  but  sometimes  in  healthy  individuals. 

In  the  productive  forms  there  is  a  generalized  cellular  infiltration  of 
the  mucosa,  particularly  that  covering  the  lowest  turbinals,  which  leads 
to  a  more  or  less  extensive  thickening  of  the  membrane  (rhinitis  hyper- 
trophica).  This  gradually  gives  way  to  a  contracting  fibrous  tissue, 
relatively  poor  in  cellular  elements,  in  which  the  glands  are  atrophic, 
that  ultimately  leads  to  atrophy  of  the  bony  parts  (rhinitis  atrophica). 
In  these  later  stages  the  mucosa  is  covered  with  a  thick  yellowish  or 
yellowish-green  purulent  secretion,  together  with  thick  scabs.  These 
have  a  very  characteristic  and  offensive  odor  (ozcend). 

Tuberculosis. — Tuberculosis  takes  the  form  of  tuberculous  granula- 
tions or  actual  ulceration.  It  is,  on  the  whole,  not  frequent,  except 
when  there  is  most  advanced  tuberculous  disease  of  the  respiratory 
passages,  the  palate,  or  the  pharynx.  Lupus  of  the  face,  however, 
frequently  extends  into  the  nose  and  causes  widespread  destruction. 

Syphilis. — Syphilis  takes  the  form  of  a  purulent  catarrh,  which  is 
very  common  in  the  eruptive  stage  of  the  disease,  and  in  the  congenital 
lues  of  the  newborn.  As  with  other  mucous  surfaces,  the  nasal  mucosa 
can  be  the  site  of  condylomata.  More  frequent  is  the  gummatous  form 
of  the  disease,  which  may  begin  either  in  the  mucosa  or  in  the  bony 


266  THE  NOSE 

and  cartilaginous  structures.  This  leads  often  to  perforation  of  the  sep- 
tum and  the  hard  palate,  with  the  production  of  the  characteristic  saddle- 
shaped  deformity.  It  is  associated  with  a  thick  purulent  secretion, 
which  dries  into  hard  scabs  and  has  a  very  offensive  odor  (ozaena 
syphilitica). 

Glanders. — Glanders  causes  a  purulent  or  a  purulent-hemorrhagic 
discharge  from  the  nose,  and  produces  in  the  mucosa  either  a  diffuse 
inflammation  or  multiple  small  abscesses.  The  process  may  lead  to 
destruction  of  the  bone. 

Leprosy. — Leprosy  is  found  in  the  form  of  nodular  granulomas, 
which  may  form  ulcers  covered  with  thick  crusts. 

Rhinoscleroma. — Rhinoscleroma  is  a  peculiar  disease  first  studied 
fully  by  v.  Frisch,  Paltauf,  v.  Eiselsberg,  and  O.  Chiari,  which  is  charac- 
terized by  the  formation  of  nodular  masses  of  almost  ivory  consistency, 
chiefly  upon  the  nasal  mucosa,  but  also  upon  the  skin  and  the  upper  lip. 
Later,  the  masses  coalesce  and  lobulated  tumors  are  produced. 

Microscopically,  in  the  submucosa  and  subcutaneous  tissues  a  round 
and  spindle-celled  infiltration  is  seen.  The  infiltration  is  perhaps 
more  marked  in  the  deeper  layers  where  the  cells  are  larger  and  often 
hyaline  or  show  mucoid  degeneration.  The  walls  of  the  vessels  are 
thickened,  and  all  parts  show  a  chronic  productive  inflammation. 
Numerous  "mastzellen"  are  found  in  the  infiltrated  areas.  The  condi- 
tion is  due  to  a  specific  microorganism  which  is  found  abundantly  in  the 
swollen  cells. 

Parasites. — A  great  variety  of  these  have  been  described.  Besides 
the  schizomycetes ,  may  be  mentioned  the  O'idium  albicans,  the  Scolo- 
pendra,  and  the  larvae  of  various  diptera  (producing  a  condition  of 
myiasis). 

Foreign  Bodies. — These  are  very  frequently  inserted  into  the  nose  by 
children.  Many  substances  have  been  found,  such  as  marbles,  peas, 
beans,  paper,  and  wood.  H.  S.  Birkett1  has  recently  recorded  a  curious 
case  in  which  a  thimble  was  embedded  in  the  nose  for  eighteen  years. 
It  was  found  to  be  incrusted  with  salts,  the  surface  being  quite  smooth. 


RETROGRESSIVE  METAMORPHOSES. 

Calcification. — Calcification  of  the  mucosa  is  recorded.  It  takes 
the  form  of  scattered  spicules  of  lime  embedded  in  the  membrane,  or  of 
actual  plates. 

Rhinoliths. — Rhinoliths,  or  concretions,  may  be  formed  in  plugs 
of  inspissated  mucus,  but  more  usually  the  salts  are  deposited  about 
foreign  bodies.  Sometimes,  instead  of  a  calcareous  coating,  a  caseous- 
looking  mass  composed  of  dead  epithelium,  leukocytes,  and  detritus 
may  be  deposited  upon  the  offending  substance. 

A  perforating  ulcer  of  the  septum   (apart  from  the  syphilitic  and 

1  Montreal  Medical  Journal,  28 : 1899 : 449. 


PROGRESSIVE  METAMORPHOSES  267 

tuberculous  variety)  is  decidedly  rare.  According  to  Zuckerkandl,  it 
does  not  necessarily  follow  inflammation,  but  may  be  a  purely  retro- 
gressive phenomenon.  It  may  perforate  or,  healing,  leave  a  dense  scar. 
The  condition  has  been  attributed  to  coagulation-necrosis,  capillary 
thrombosis,  the  action  of  bacteria,  and  to  a  trophoneurosis. 


PROGRESSIVE  METAMORPHOSES. 

The  mucous  membrane  of  the  nose  and  of  the  accessory  cavities  often 
shows  hyperplastic  growth  or  actual  tumor  formation,  which  may  be  due 
to  a  preexisting  inflammation  or  may  arise  without  discoverable  cause. 
The  lesion  is  diffuse  or  may  result  in  the  formation  of  local  excrescences 
or  polyps.  A  common  form  is  the  soft  or  myxomatous  polyp,  which  is 
of  a  grayish  semitranslucent  appearance.  Nasal  polyps  are  frequently 
multiple  and  may  attain  a  considerable  size.  They  are  composed  of 

FIG.  64 


Myxomatous  polyp  from  the  nose.      Winckel  obj.  No.  6,  without  ocular.      (From  the 
collection  of  Dr.  A.  G.  Nicholls.) 

tissue  identical  with  that  of  the  mucous  membrane,  except  that  it  is  more 
cellular.  Within  this  growth  glands  may  become  entangled,  which  some- 
times become  dilated,  owing  to  the  retention  of  secretion,  so  that  cystic 
tumors  are  the  result.  In  other  cases  the  glandular  elements  proliferate, 
giving  rise  to  adenoma.  From  the  deeper  fibrous  structures,  particularly 
from  the  bony  parts,  fibromas  often  develop.  These  often  become 
oedematous,  thus  simulating  the  myxomatous  polyp.  They  have  a 
more  yellow  color,  however.  Owing  to  excessive  development  of  the 


288  THE  LARYNX  AND  TRACHEA 

bloodvessels,  telangiectatic  or  cavernous  fibromas  are  produced.  Nasal 
polyps  may  undergo  hyaline  or  calcareous  degeneration.  Strictly  speaking, 
most  of  these  polyps  are  not  true  tumors  but  should  be  classed  with  the 
hyperplastic  formations  resulting  from  a  preexisting  inflammation. 

Carcinoma. — Primary  carcinoma  takes  the  form  of  medullary  cancer 
or  of  epitheliomatous  growths  of  the  order  of  Krompecher's  "basal- 
celled"  cancer  (vol.  i,  p.  735). 

Sarcoma. — Sarcoma,  as  a  primary  disease,  is  rare  in  the  nose,  but 
usually  extends  from  sarcoma  of  the  antrum  or  the  neighboring  parts. 
It  occasionally  forms  polypoid  excrescences. 

More  rarely,  chondroma  and  osteoma  are  met  with. 

THE  PHARYNX. 

This  will  be  considered  along  with  the  Digestive  Tract  in  Chap- 
ter XVII. 

THE  LARYNX  AND  TRACHEA. 

The  predominant  etiological  factor  in  the  production  of  diseases  of 
the  larynx  is  the  character  of  the  respired  air.  The  physical  and  chemical 
peculiarities  of  the  air;  affections  of  neighboring  organs,  particularly 
the  pharynx,  the  thyroid  gland,  and  the  lungs;  constitutional  tendencies, 
all  play  their  part.  "Catching  cold"  is  an  important  predisposing 
cause.  It  has  been  shown  experimentally  by  Rossbach  that  anemic 
and  hyperemic  conditions  of  the  larynx  can  be  induced  by  the  action 
of  heat  and  cold  upon  the  skin  of  the  body  at  some  distance  from  the 
larynx  itself. 

MALFORMATIONS. 

Complete  absence  of  the  air  passages,  in  common  with  absence  of  lungs, 
occurs  only  in  connection  with  other  grave  defects,  as  in  the  condition 
of  Acardiacus  amorphus  and  A.  acephalus.  Particular  cartilages  or 
portions  of  them  may  be  deficient  or  absent.  Hypoplasia  occurs  in 
aplasia  of  the  testicles  or  early  castration.  Partial  or  complete  fissuring 
of  the  epiglottis  has  been  observed.  There  may  be  sacculated  diver- 
ticula  of  the  sinus  of  Morgagni,  as  again  of  the  sacculi  laryngis,  recalling 
the  pouches  normally  found  in  monkeys.  The  most  important  anom- 
alies of  the  trachea  are  atresia  or  abnormal  narrowing  of  the  tube,  fistu- 
lous  communication  with  the  oesophagus,  or  imperfect  closure  of  the 
branchial  clefts. 

CIRCULATORY  DISTURBANCES. 

Owing  to  the  loose  tissue  of  the  submucosa,  circulatory  disturbances 
can  quickly  arise  and  as  quickly  disappear.  Thus  it  is,  that  when, 
for  instance,  marked  oedema  of  the  glottis  has  been  present  during  life, 
at  postmortem  examination  all  traces  of  it  may  have  passed  off. 


ACUTE  CATARRHAL  LARYNGITIS  269 

Anemia  and  Hyperemia. — Anemia  and  hyperemia  may  be  local  or 
a  part  of  a  general  condition,  and  occur  under  the  same  conditions  as 
elsewhere.  Anemia  of  the  larynx  is  well  known  to  be  an  early  mani- 
festation in  some  cases  of  tuberculosis  and  chlorosis. 

Prolonged  passive  hyperemia  may  lead  to  a  permanent  dilatation  of 
the  veins  (phlebectasia  laryngea).  Hemorrhages  may  occur  from 
traumatism,  acute  inflammation,  ulceration,  the  hemorrhagic  diathesis, 
morbus  maculosus,  scorbutus,  and  phosphorus  poisoning.  They  may 
amount  to  the  formation  of  a  hematoma  which  may  lead  to  death  from 
suffocation. 

(Edema. — The  most  important  condition  is  that  of  oedema,  which 
leads  to  partial  or  complete  obstruction  of  the  rima  glottidis.  The  swell- 
ing affects  those  parts  in  which  loose  submucous  tissue  abounds,  the 
aryepiglottic  folds,  the  epiglottis,  the  false  cords,  the  arytenoid  cartilages, 
and  more  rarely  on  the  vocal  cords.  The  condition  is  due  to  a  variety 
of  causes,  among  which  may  be  mentioned  cardiac  and  renal  dropsy, 
pressure  from  cervical  and  mediastinal  tumors,  aneurisms.  These  forms 
are  mostly  chronic.  The  acute  type  is,  as  a  rule,  inflammatory  in  origin. 


INFLAMMATIONS. 

Acute  Laryngitis. — According  to  the  character  and  intensity  of 
the  causative  agent,  acute  laryngitis  may  be  classified  as  catarrhal, 
herpetic,  fibrinous  (membranous  laryngitis),  diphtheritic,  phlegmonous, 
and  vlcerative,  in  addition  to  certain  more  specific  forms  found  in  tuber- 
culosis, syphilis,  variola,  rhinosderoma,  glanders,  and  leprosy. 

Acute  Catarrhal  Laryngitis. — Acute  catarrhal  laryngitis  is  a  very  common 
affection,  but  comes  much  more  frequently  under  the  observation  of 
the  laryngologist  than  of  the  pathologist. 

The  condition  is  usually  brought  about  by  mechanical,  chemical, 
or  thermic  irritation,  and  occurs  in  one  of  those  situations  that  is  affected 
readily  by  climatic  and  atmospheric  conditions.  It  occurs  also  in  the 
course  of  the  infective  fevers,  measles,  scarlatina,  variola,  typhoid,  while 
in  certain  others,  such  as  influenza  and  whooping-cough,  it  is  often 
the  most  prominent  feature.  The  distribution  and  intensity  of  the 
process  are  affected  mainly  by  the  nature  and  virulence  of  the  infecting 
agent.  The  epiglottis  or  the  vocal  cords  may  be  chiefly  affected,  or  else 
the  intermediate  portion.  At  first  there  is  more  or  less  redness  and 
swelling  of  the  parts.  Sooner  or  later,  a  catarrhal  secretion  makes  its 
appearance,  which  is  at  first  viscid  and  glassy,  but  soon  becomes  more 
cloudy,  grayish,  or  grayish-yellow  from  admixture  with  leukocytes. 
The  secretion  rarely  forms  hard  crusts.  From  loss  of  the  lining  epi- 
thelium small  superficial  erosions  are  produced.  From  the  irritation 
produced  by  coughing,  small  quantities  of  blood  are  often  effused. 
The  catarrh  from  influenza  usually  begins  in  the  nose  and  spreads 
downward  into  the  bronchi  and  the  lungs.  The  secretion  is  viscid, 
mucopurulent,  and  glairy,  and  of  a  pale  greenish  color.  Within  the 
leukocytes,  and  lying  free,  the  minute  bacillus  of  Pfeiffer  can  be  demon- 


270  THE  LARYNX  AND  TRACHEA 

strated.  The  process  in  whooping-cough  usually  begins  below  the  vocal 
cords,  and  thence  spreads  into  the  bronchi. 

Acute  Catarrhal  Tracheitis. — Acute  catarrhal  tracheitis  does  not  differ 
materially  in  its  etiology  and  progress  from  catarrhal  laryngitis.  Acute 
catarrh,  if  neglected,  or  if  the  cause  be  not  removed,  may  ultimately 
assume  a  chronic  course,  particularly  in  those  who  suffer  from  some 
constitutional  taint.  Catarrh  may  also  be  chronic  from  the  first  in 
those  who  use  the  voice  much  or  are  addicted  to  the  immoderate  use  of 
alcohol  or  tobacco. 

Laryngitis  Herpetica. — Laryngitis  herpetica  is  characterized  by  the 
formation  of  vesicles  on  the  edge  of  the  epiglottis  and  on  the  vocal  cords. 
Small  erosions  may  be  caused  by  the  rupture  of  the  vesicles.  The 
condition  is  rare. 

Membranous  Laryngitis. — This  form  is  characterized  by  the  production 
of  a  ^  fibrinous  membrane  of  a  gray  or  grayish-white  color,  somewhat 
elevated  above  the  general  level  of  the  mucosa  and  varying  in  extent. 
The  condition  is  usually  not  confined  to  the  larynx,  but  extends  upward 
to  the  epiglottis,  throat,  tonsils,  and  buccal  cavity,  and  downward  to 
the  trachea  and  bronchi.  The  infection  is  usually  a  descending  one, 
but  not  invariably  so,  for  the  larynx  may  be  affected  while  the  pharyngeal 
structures  remain  free.  When  the  membrane  is  removed,  which  can 
often  be  accomplished  without  difficulty,  the  surface  is  found  to  be  red- 
dened and  more  or  less  swollen.  The  thicker  the  membrane  is,  the 
firmer  and  more  elastic  it  appears  to  be.  In  the  neighborhood  of  the 
patch  the  mucosa  shows  the  usual  changes  of  a  simple  or  mucopurulent 
catarrh. 

Microscopically,  the  membrane  is  seen  to  consist  of  fine  threads 
of  fibrin,  or  sometimes  broader  bands,  which  form  a  thick  meshwork 
and  in  many  instances  have  undergone  hyaline  change,  so  that  it  presents 
a  superficial  resemblance  to  cartilaginous  tissue.  In  the  meshes  of  this 
membrane  are  numerous  cells,  chiefly  leukocytes  and  desquamated 
epithelium.  These  cells  often  show  signs  of  necrosis.  It  is  not  always 
possible  to  differentiate  macroscopically  the  true  diphtherial  from  other 
forms  of  membranous  inflammation.  A  distinction  is  drawn,  pathologic- 
ally, between  a  fibrinous  or  croupous,  sometimes  called  pseudomem- 
branous,  exudate,  which  merely  lies  upon  the  top  of  an  epithelial  surface 
and  which  can  readily  be  removed,  from  another  form,  of  which  true 
diphtheria  is  the  type,  where  the  mucosa  and  submucosa  are  the  site  of 
a  fibrinous  inflammation,  and  both  the  exudate  and  the  deeper  tissues 
have  undergone  a  form  of  coagulation-necrosis,  welding  them  into  a 
united  mass.  It  should,  however,  be  mentioned  that  the  adhesion  or 
otherwise  of  the  membrane  depends  largely  on  the  nature  of  the  under- 
lying epithelium.  Where  columnar  epithelium  exists,  the  membrane  is 
readily  removed,  but  where  the  epithelium  is  absent  or  where  the  cells 
•are  of  the  squamous  variety,  the  exudate  becomes  firmly  attached.  The 
term  "croup"  has  unfortunately  given  rise  to  much  misunderstanding  in 
this  connection.  If  we  employ  it  in  regard  to  those  forms  of  laryngitis 
characterized  by  a  stridorous  respiration,  we  have  to  recognize  with 


TUBERCULOUS  LARYNGITIS  271 

Virchow  a  catarrhal,  a  fibrinous,  and  a  diphtheritic  form.  It  is  better, 
however,  to  discontinue  the  use  of  the  word  "croup"  as  misleading,  and 
speak  of  a  fibrinous  laryngitis. 

With  regard  to  the  etiology  of  laryngitis,  a  variety  of  factors  may 
be  at  work.  Any  cause  which  destroys  the  lining  epithelium  of  the  larynx 
and  irritates  the  underlying  structures  is  sufficient.  Children  have  a 
greater  liability  to  the  affection  than  have  adults.  Chemical  and  thermic 
irritants  play  a  certain  part,  but  by  far  the  most  important  factors  are 
the  infectious  diseases,  such  as  variola,  measles,  typhoid,  pyemia,  and 
pneumonia.  The  most  frequent  causes  are  diphtheria  and  scarlatina. 
In  both  the  last-named  diseases,  the  condition  is  usually  secondary  to 
an  affection  of  the  pharynx,  but  in  some  few  cases  the  larynx  alone  is 
attacked.  It  must  be  admitted  that  whatever  the  original  cause  of  a 
laryngitis  may  be,  bacteria  sooner  or  later  play  an  important  role.  The 
germs  chiefly  concerned  are  the  diphtheria  bacillus  and  the  various  pus- 
producing  organisms,  notably  the  streptococcus.  It  should  not  be  for- 
gotten that  the  pyogenic  cocci  are  quite  competent  to  produce  an  adherent 
membrane  closely  resembling  that  of  diphtheria. 

The  forms  due  to  the  Klebs-Loeffler  bacillus  and  pyogenic  cocci  are 
much  more  intense  than  are  the  others.  Any  case  of  membranous  laryn- 
gitis that  lasts  longer  than  twenty-four  hours  should  rouse  the  suspicion 
of  diphtheria.  The  examination  of  swabs  from  the  throat,  or  of  the 
expectoration,  for  the  specific  germ  is,  however,  the  only  sure  way  of 
making  a  differential  diagnosis. 

Phlegmonous  Laryngitis. — Phlegmonous  laryngitis  is  characterized 
by  the  formation  of  a  purulent  or  fibrinopurulent  infiltration  in  the 
mucosa  and  submucosa,  and  frequently  is  a  late  complication  of  inflam- 
matory oedema  of  the  larynx.  The  parts  affected  are  the  loose  connective 
tissues,  the  epiglottis,  the  aryepiglottic  folds,  and  the  false  cords,  more 
rarely  the  under  surface  of  the  true  cords.  Local  abscess  formation 
with  ulceration  is  not  uncommon.  It  is  a  frequent  complication  of 
diphtheria,  erysipelas  of  the  face,  and  the  other  infectious  diseases. 
It  may  spread  to  the  cartilages,  causing  a  perichondritis,  that  may  lead 
to  sequestration  of  the  part  and  the  formation  of  a  fistula. 

Chronic  Catarrh. — Chronic  catarrh  of  the  larynx  leads  to  hyperemia 
of  the  parts,  with  hypertrophy  of  the  mucosa  and  submucosa,  together 
with  fibrous-tissue  proliferation.  Localized  thickenings,  either  flat  or 
warty,  are  found  not  infrequently  upon  the  vocal  cords,  of  a  gray  or 
whitish-gray  color.  They  are  often  found  in  speakers  and  singers.  In 
other  cases  the  glands  are  enlarged,  so  that  a  granular  condition  is  pro- 
duced. 

Tuberculous  Laryngitis. — This  is  a  very  frequent  complication  of 
pulmonary  tuberculosis.  Rarely,  the  larynx  is  affected  in  general  miliary 
tuberculosis,  and  more  rarely  still  primary  tuberculosis  of  the  larynx 
has  been  recorded  (Orth).  The  infection  is  usually  brought  about 
through  the  sputa.  The  lesions  generally  take  two  forms,  ulceration 
and  diffuse  infiltration. 

Tuberculous  ulcers  vary  a  good  deal  in  appearance.     They  may  be 


272  THE  LARYNX  AND  TRACHEA 

shallow  and  lenticular,  deep  and  crateriform,  few  or  numerous.  The 
lenticular  form  is  seen  most  typically  on  the  epiglottis  with  a  pale,  flat 
base  and  rounded  margin.  In  other  cases,  on  the  contrary,  hyperemia 
is  marked.  The  process  begins  with  small  foci  of  cellular  infiltration 
in  the  subepithelial  tissue,  which  gives  rise  to  minute  nodules  projecting 
above  the  surface.  These  caseate  and  produce  shallow  ulcers.  In  other 
cases  the  infiltration  is  more  extensive  and  severe,  forming  a  subepithelial 
granulation  tissue  in  which  typical  tubercles  are  found  embedded.  This 
gives  rise  to  warty  projections  of  the  epithelial  surface,  so  that  a  hyper- 
trophic  appearance  is  produced.  Through  caseation  ulcers  are  produced 
in  this  form  also. 

FIG.  65 


Chronic  tuberculosis  of  the  larynx  and  trachea.  The  thickening  of  the  epiglottis  is  well 
shown,  and  numerous  superficial  erosions  are  also  apparent.  (From  the  Pathological  Museum 
of  McGill  University.) 

On  the  primary  infection  can  supervene  a  secondary  development  of 
tubercles  in  the  neighborhood  of  the  original  lesion,  which  takes  the 
form  of  small  inflammatory  foci  in  the  mucosa,  submucosa,  and  peri- 
chondrium,  or  in  the  mucous  glands,  or  even  between  the  muscle  bundles. 
Microscopically,  the  appearances  are  those  of  a  vascular  granulation 
tissue,  with  epithelioid  and  giant  cells  and  central  caseation.  The  ulcers 
are  found  on  the  epiglottis,  the  anterior  or  posterior  portions  of  the 
thyroid  cartilage,  the  arytenoid  cartilages,  the  false  and  true  cords, 
but  the  greatest  variety  exists  in  the  distribution  of  the  lesions.  In  addi- 


SIMPLE  ATROPHY 

tion  to  the  specific  lesion,  there  is  usually  a  marked  simple  catarrh. 
Supervening  upon  the  condition,  oedema  of  the  glottis,  phlegmon,  and 
sequestration  of  cartilage  may  occur.  In  the  most  severe  cases  of  tuber- 
culous laryngitis  scattered  ulcers  are  to  be  found  for  some  distance  down 
the  trachea. 

Syphilitic  Laryngitis. — Syphilis  may  produce  a  simple  congestion 
or  catarrh  of  the  parts,  but  there  is  often  a  definite  infiltration.  Ulcera- 
tion  may  take  place,  the  base  and  edges  of  the  erosions  being  greatly 
thickened.  Owing  to  the  prolonged  irritation,  the  mucosa  is  often 
hyperplastic  and  thrown  into  polypoid  or  warty  elevations,  which  later  on 
may  ulcerate  and  finally  may  become  more  or  less  normal  from  absorp- 
tion of  the  exudate.  The  ulcers  are  found  usually  upon  the  epiglottis, 
the  vocal  cords,  and  the  back  part  of  the  larynx. 

A  second  variety  is  characterized  by  the  formation  of  gummas,  which 
are  to  be  found  usually  in  the  submucosa  of  the  epiglottis  and  on  the 
vocal  cords.  From  their  size  they  may  obstruct  the  lumen  of  the  air 
passages.  Perichondritis  and  necrosis  of  the  cartilages  and  epiglottis 
are  not  uncommon.  Small  gummas  may  be  absorbed,  but  when  there 
is  destruction  of  tissue,  scars  are  the  result.  These  are  hard,  whitish, 
and  contracted,  leading  to  great  deformity  of  the  organ  with  stenosis  of 
the  lumen.  The  mucosa  between  the  contracted  bands  often  shows 
warty  or  polypoid  hyperplastic  growth. 

Typhoid  Fever. — This  may  give  rise"  to  catarrh,  which  leads  to 
desquamation  of  the  epithelium,  hemorrhagic  infiltration,  and  super- 
ficial erosion.  The  lesions  are  usually  to  be  found  on  the  edges  of  the 
epiglottis.  There  is  very  little  exudate,  and  the  inflammation  is  apt  to  be 
desquamative  in  character.  Often  there  is  marked  oedema  of  the  parts, 
and  perichondritis  and  erosion  of  the  cartilages  are  not  uncommon. 
In  other  cases  there  is  a  fibrinous  or  so-called  "croupous"  exudate.  In 
this  case  the  mucosa  of  the  epiglottis,  the  anterior  surface  of  the  larynx, 
and  the  vocal  cords  are  covered  with  a  distinct  membrane  composed  of 
desquamated  and  necrotic  epithelium,  leukocytes,  fibrin,  and  bacteria. 

A  specific  typhoid  lesion,  however,  exists,  in  which  there  is  swelling 
of  the  lymphoid  follicles  at  the  base  of  the  epiglottis,  the  false  cords,  the 
inner  side  of  the  arytenoid  cartilages,  and  the  anterior  commissure. 
Just  as  in  the  intestine,  the  swelling  of  the  lymphoid  tissue  tends  to  the 
production  of  definite  elevations  upon  the  mucous  membrane.  These 
become  necrotic  and  may  develop  into  ulcers  with  swollen  infiltrated 
margins.  In  severe  cases  the  infiltration  can  extend  beyond  the  limits 
of  the  lymphoid  tissue  to  the  neighboring  parts.  Ulcers  analogous 
to  those  found  in  the  intestine  are  thus  produced. 

Ulceration  of  the  larynx  may  also  occur  in  glanders,  leprosy,  variola, 
and  rhinoscleroma. 

RETROGRESSIVE  METAMORPHOSES. 

Simple  Atrophy. — Simple  atrophy,  leading  to  a  thinning  of  the 
mucosa,  diminution  of  secretion,  atrophy  of  muscle  and  cartilage,  fatty 

18 


274  THE  LARYNX  AND  TRACHEA 

degeneration,  and  calcification  of  the  various  cartilages,  is  found  in  old 
age,  premature  senescence,  and  in  certain  cachexias. 

Ossification. — Ossification  of  the  cartilages  has  been  recorded  as  a 
result  of  chronic  inflammatory  processes. 

Rarely,  a  deposit  of  uric  acid  salts  has  been  observed  in  the  cartilages 
and  ligaments  in  gouty  cases. 


PROGRESSIVE  METAMORPHOSES. 

Simple  Hypertrophy. — Simple  hypertrophy  of  the  mucosa  leads  to 
the  production  of  small  local  verrucose  overgrowths,  often  taking  the  form 
of  polypi.  These  are  found  by  preference  in  those  situations  that  are 
rich  in  glandular  elements.  Polypoid  masses  are  also  met  with  in  the 
neighborhood  of  tumors  and  scars. 

FIG.  66 


Carcinoma  of  the  larynx.      (From  the  Pathological  Museum  of  McGill  University.) 

Tumors. — The  most  common  form  of  tumor  is  the  papilloma  or 
papillary  fibroma.  This  forms  a  warty  or  papillomatous  growth,  often 
resembling  the  acuminate  condyloma.  It  is  usually  situated  on  the 
vocal  cords,  and  consists  of  a  fibrous  ground  substance  covered  with 
stratified  pavement  epithelium.  Round  cells  are  often  seen  and  the 
growth  may  contain  numerous  wide  vessels.  The  diffuse  variety  is 
called  by  some  pachydermia  laryngis.  These  growths  frequently 
return  after  removal,  but  only  rarely  develop  into  carcinoma. 

Nodular  fibromas  are  found  also  on  the  vocal  cords,  particularly  in 
singers. 


THE  BRONCHI  275 

Enchondroma,  lipoma,  myxoma,  angioma,  lymphangioma,  adenoma, 
and  lymphadenoma  have  been  found.  Cysts  are  fairly  rare,  and  are 
found  in  the  epiglottis1  and  on  the  sinuses  of  Morgagrii. 

Of  malignant  growths,  the  most  frequent  is  the  carcinoma.  .  This 
is  usually  of  the  squamous  variety,  but  a  soft  glandular  carcinoma  and 
even  scirrhus  have  been  recorded. 

Carcinoma  of  the  larynx  usually  starts  from  the  true  or  false  vocal 
cords,  the  ventricles,  or  even  lower  down  (intrinsic  laryngeal  carcinoma). 
Rarely,  it  extends  from  the  epiglottis,  pharynx,  or  adjacent  parts 
(extrinsic  carcinoma).  It  is  apt  to  spread  locally,  but  distant  metastases 
may  be  formed,  particularly  in  the  liver. 

Sarcoma,  either  round  or  spindle-celled,  is  rarer. 

Secondary  growth  is  rare. 


ALTERATIONS  IN  SIZE  AND  SHAPE. 

These  affect  mainly  the  trachea,  but  the  lumen  of  the  larynx  may  be 
obstructed  either  partially  or  completely  by  cedema,  inflammatory 
infiltration,  tumors,  or  exostoses.  Atresia,  or  narrowing  of  the  lumen, 
may  be  due  to  structural  defects  or  to  a  contraction  of  scar  tissue,  but 
usually  is  due  to  pressure  exerted  upon  the  tube  from  without.  Such 
may  be  brought  about  by  tumors  of  the  thyroid  glands,  peritracheal 
abscesses,  enlarged  glands,  and  aneurisms.  Pressure,  if  long  continued, 
may  lead  to  atrophy  of  the  cartilages  and  give  rise  to  the  clinical  symp- 
toms of  asthma,  as  in  a  case  under  our  own  observation  at  the  Royal 
Victoria  Hospital,  where  the  obstruction  was  brought  about  by  an  en- 
larged middle  lobe  of  the  thyroid  and  led  to  acute  bronchitis  and  death 
from  atelectasis  of  the  lungs  and  secondary  pneumonia.2  Perforation  of 
the  trachea  may  be  due  to  ulcerating  tumors,  peritracheal  suppuration, 
and  aneurisms. 

Dilatation. — Dilatation,  either  complete  or  partial,  in  the  latter  case 
leading  to  the  formation  of  saccular  diverticula,  is  occasionally  found. 


THE  BRONCHI. 

The  pathological  changes  that  are  apt  to  be  met  with  in  the  bronchial 
tree  are  somewhat  diverse,  the  details  of  the  processes  being  considerably 
modified  by  the  anatomical  peculiarities  of  the  parts  affected.  Thus, 
the  larger  bronchi  which  have  cartilaginous  walls  bear  a  closer  relation, 
pathologically  speaking,  to  the  larynx  and  trachea  than  to  the  lungs, 
while  the  terminal  bronchioles  are  in  such  intimate  connection  with  the 
lung  substance  that  lesions  of  the  one  structure  profoundly  affect  the 
other. 

1  See  H.  D.  Hamilton,  Montreal  Med.  Jour.,  28: 1899:  602. 

2  Adami,  Canada  Lancet,  35  :  1902  :  373, 


276  THE  BRONCHI 


CONGENITAL  ANOMALIES. 

Supernumerary  bronchi  have  been  met  with.     Congenital  atresia  and 
bronchiectasis  are  more  frequent. 


CIRCULATORY  DISTURBANCES. 

Passive  Congestion. — Passive  congestion  is  frequently  met  with,  and 
is  found  typically  in  connection  with  valvular  diseases  of  the  heart. 
The  mucous  membrane  is  swollen  and  deep  red  in  color.  When  catarrhal 
inflammation  supervenes,  as  it  so  frequently  does,  there  is  a  slight  sticky 
mucoid  exudate.  The  congestion  affects  not  only  the  mucosa,  but  the 
deeper  structures  as  well.  In  other  cases  small  ecchymoses  are  produced 
or  even  extravasation  of  blood  into  the  lumen  of  the  bronchi.  Congestion 
is  a  constant  accompaniment  of  bronchitis. 

Hemorrhage. — Hemorrhage  into  a  bronchus  does  not  often  occur, 
unless  from  erosion  a  communication  is  made  with  some  branch  of  the 
pulmonary  artery,  or  when  an  aneurism  ruptures  into  the  tube.  Petechice 
are  seen  also  in  the  hemorrhagic  diatheses  and  in  many  of  the  infective 
fevers. 

Vicarious  menstruation  from  the  bronchi  is  also  recorded. 


INFLAMMATIONS. 

Bronchitis. — Bronchitis  is  one  of  the  most  frequent  pathological 
changes  that  we  meet  with.  The  inflammation  may  be  restricted  to  the 
bronchial  structure,  but  it  is  frequently  associated  with  other  and  more 
serious  distui  bances.  The  condition  is  usually  bilateral,  but  not  in- 
variably co-extensive  with  the  whole  bronchial  tree.  Certain  portions 
seem  to  be  more  often  affected  than  others.  Bronchitis  affecting  the 
larger  bronchi  is  always  associated  with  tracheitis  and  often  with  laryn- 
gitis. A  similar  condition  of  the  minuter  bronchioles  is  apt  to  be 
associated  with  peribronchitis  and  bronchopneumonia. 

Bronchitis  arises  from  many  causes  and  assumes  various  forms.  It 
may  result  from  the  inhalation  of  irritating  substances  derived  from  the 
air  or  the  nasopharyngeal  cavity,  and  is  a  frequent  accompaniment  of 
the  infective  fevers,  particularly  typhoid,  measles,  diphtheria,  tubercu- 
losis, influenza,  and  variola.  Bronchitis  is  a  constant  accompaniment 
of  emphysema  of  the  lungs,  bronchiectasis,  pneumonia,  and  abscess 
of  the  lung.  In  bronchiectasis  it  is  very  apt  to  assume  the  putrid  type. 
Passive  congestion  of  the  lungs,  especially  when  due  to  a  valvular  heart 
lesion,  favors  the  development  of  bronchial  inflammation. 

The  macroscopic  appearances  of  the  lungs  in  this  condition  vary  some- 
what according  to  the  type  of  the  affection  present,  but  in  the  simple 
catarrhal  form  the  mucous  membrane  is  reddened,  swollen,  and  covered 


PURULENT  BRONCHITIS  277 

with  more  or  less  sticky  exudate.  In  the  simpler  forms,  this  is  of  a  clear 
grayish  appearance  and  largely  mucoid  in  nature,  but  when  the  exudate 
is  more  cellular,  and  tends  to  approach  the  mucopurulent  or  purulent 
type,  the  secretion  is  thicker  and  more  opaque.  When  a  section  is  made 
through  the  lung,  small  drops  of  muco-pus  may  often  be  squeezed  out 
of  the  openings  of  the  bronchioles.  Usually  the  lung  substance  is  more 
or  less  reddened. 

Acute  Bronchitis. — Acute  bronchitis  may  be  divided  into  the  follow- 
ing forms :  Simple  catarrhal,  purulent,  membranous,  putrid  or  gangrenous, 
and  specific. 

FIG.  67 


Acute  purulent  bronchitis  with  early  bronchopneumonia.      Leitz  obj.  No.  7,  without  ocular. 
(From  the  private  collection  of  Dr.  A.  G.  Nicholls.) 

In  catarrhal  bronchitis  the  character  of  the  secretion  may  vary  con- 
siderably. As  a  rule,  desquamation  of  the  epithelium  is  not  such  a 
prominent  feature  as  the  conversion  of  the  epithelial  into  muciniparous 
cells,  resulting  in  hypersecretion.  Sometimes  the  secretion  is  only 
scanty,  transparent,  and  adherent,  the  so-called  "dry  catarrh."  In 
other  cases  it  is  more  abundant,  mucoid,  or  even  purulent.  In  still 
others,  it  is  very  abundant,  thin,  and  watery  (serous  catarrh).  The  last 
form  is  apt  to  occur  in  passive  congestion  of  the  lungs.  A  few  instances 
have  been  met  with  where  several  liters  of  fluid  have  been  expectorated 
daily. 

Purulent  Bronchitis  (Bronchoblennorrhoea). — This  is  a  more  serious  affec- 
tion and  is  apt  to  be  found  in  the  smaller  bronchi  associated  with 
certain  chronic  pulmonary  affections,  such  as  bronchiectasis,  tuberculosis, 
abscess,  and  the  like.  When  the  secretion  is  retained  and  becomes 
decomposed  from  the  action  of  putrefactive  microorganisms,  it  becomes 


278  THE  BRONCHI 

altered  in  color  and  very  fetid.  In  such  cases  the  wall  of  the  bronchus 
may  be  destroyed.  This  is  putrid  or  gangrenous  bronchitis.  It  is  often 
the  result  of  abscess  of  the  lung,  but  may  be  primary. 

Microscopically,  sections  through  an  affected  bronchus  show  oedema, 
congestion,  and  cellular  infiltration  in  the  mucosa,  and  in  severe  cases 
in  the  submucosa.  There  is  a  certain  amount  of  desquamation  of  the 
lining  epithelium,  with  an  abundance  of  mucin-containing  goblet  cells. 
On  the  free  surface  is  a  certain  amount  of  exudate  made  up  of  mucin, 
fibrin,  and  leukocytes. 

Under  the  term  "  bronchiolitis  exudativa,"  Curschmann1  has  de- 
scribed a  form  of  bronchial  catarrh  that  is  by  some  regarded  as  the 
main  lesion  of  bronchial  asthma.  In  this  form  the  exudate  is  scanty, 
clear  and  glassy,  and  very  viscid.  It  is  coughed  up  in  small  pearls  that 
are  characteristic.  When  these  are  spread  out  on  a  glass  plate  on  a  black 
background  a  very  peculiar  appearance  can  be  observed.  This  con- 
sists in  the  arrangement  of  the  sputum  in  a  spiral  form  about  a  central 
fine  thread.  These  are  the  so-called  "  Curschmann's  spirals,"  and  may 
often  be  made  out  with  the  help  of  a  hand-lens.  They  are  not  absolutely 
pathognomonic  of  asthma,  as  they  have  been  described  as  present  occa- 
sionally in  lobar  and  lobular  pneumonia.  In  the  secretion,  octahedral 
crystals,  the  so-called  "  Charcot-Leyden  crystals,"  have  been  met  with 
in  asthma,  but  are  found  in  fibrinous  bronchitis  also.  A  striking 
feature  of  asthmatic  sputum  is  the  presence  of  considerable  numbers  of 
eosinophile  cells.  They  are  found  in  other  conditions  as  well. 

Membranous  Bronchitis. — Membranous  bronchitis  is  nearly  always 
associated  with  a  similar  affection  of  the  trachea.  It  is  most  often  due 
to  the  diphtheria  bacillus,  but  is  occasionally  due  to  the  inhalation  of 
septic  microorganisms  from  the  mouth.  The  mucosa  is  covered  with 
a  membrane  of  varying  thickness,  usually  most  extensive  in  the  larger 
bronchi.  The  bronchioles  may  be  blocked  with  leukocytes  and  fibrin 
intermingled. 

Chronic  Bronchitis. — Chronic  bronchitis  is  practically  always  purulent, 
and  while,  in  most  particulars,  it  closely  resembles  the  acute  form,  it 
differs  from  it  in  the  presence  of  a  more  deeply  penetrating  inflammation 
and  in  the  production  of  fibrous  tissue.  It  is  common  in  emphysema 
and  bronchiectasis.  The  mucous  membrane  is  swollen,  reddened, 
infiltrated,  and  covered  with  purulent  secretion.  Leukocytic  infiltra- 
tion can  be  made  out  in  all  the  layers  of  the  bronchi,  the  walls  of  which 
are  also,  according  to  Orth,  hypertrophic.  Not  infrequently  the  mucosa 
is  thrown  into  little  polypoid  excrescences,  partly  due  to  contraction 
and  partly  to  fibrous  proliferation.  The  walls  of  the  bronchi  become 
thickened  and  there  is  often  a  fibrous  peribronchitis,  which  in  time  may 
lead  to  induration  of  the  lung  (indurative  pneumonia). 

Under  the  specific  forms  are  included  tuberculosis  and  syphilis. 

Tuberculous  Bronchitis. — Tuberculous  bronchitis  is  a  very  frequent 
occurrence  in  the  course  of  pulmonary  tuberculosis,  and  may  be  found 

1  Deut.  Archiv  f.  klin.  Med.,  32: 1882 : 1. 


HYPERTROPHY  279 

as  a  secondary  affection  in  the  aspiration  form  of  the  disease.  In  other 
cases  the  bronchus  is  involved  by  the  extension  of  a  caseous  focus  from 
without.  In  the  first  form  little  tubercles  develop  in  the  mucosa  and 
submucosa  and  by  degeneration  lead  to  the  formation  of  small  ulcers. 
These  may  coalesce  and  in  some  cases  the  whole  bronchial  wall  becomes 
caseous. 

Primary  tuberculosis  of  the  trachea  and  bronchi  has  been  observed.1 

Syphilis. — Syphilis  of  the  bronchi  resembles  the  same  affection  of  the 
larynx  and  trachea.  From  fibrous  contraction,  great  distortion  of  the 
bronchi  may  occur.  It  is,  however,  a  rare  condition. 

Foreign  Bodies  and  Parasites. — Foreign  bodies,  such  as  corks, 
particles  of  bone,  slate-pencils,  etc.,  are  occasionally  inhaled  into  the 
bronchi.  They  are  most  frequently  found  in  the  right  side,  the  right 
bronchus  being  wider  and  more  vertically  situated  than  the  left.  The 
symptoms  produced  depend  largely  on  the  nature  of  the  substance 
inhaled.  If  this  be  septic,  putrid  bronchitis  and  abscess  of  the  lung 
are  the  results.  In  other  cases  a  mild  bronchitis  or  pneumonia  is  the 
result.  When  the  obstruction  of  the  branch  of  the  bronchial  tree  is 
complete,  collapse  of  the  lung  occurs. 

Broncholiths. — Broncholiths  formed  of  inspissated  secretion  in  which 
lime  salts  have  been  deposited  are  occasionally  met  with,  especially 
in  cases  of  bronchiectasis. 

Apart  from  the  various  schizomycetes,  parasites  are  not  commonly 
found  in  the  human  lung.  In  the  lower  animals  a  very  common  parasite 
is  the  Strongylus,  of  which  several  varieties  have  been  noted. 

Mycosis  Aspergillina. — A  mycosis  aspergillina,  due  to  the  growth  of  the 
Aspergillus  fumigatus,  has  been  described  in  connection  with  bronchiec- 
tasis. Rarely  echinococci  and  nematode  worms  have  been  met  with. 


RETROGRESSIVE  METAMORPHOSES. 

These  are  not  of  much  practical  importance.  Atrophy  and  degenera- 
tion of  the  muscle  of  the  bronchi  occur  in  chronic  bronchitis,  as  well  as 
fatty  degeneration  of  the  cartilaginous  rings.  More  noteworthy  are  the 
cases  of  generalized  calcareous  degeneration  of  the  bronchi,  analogous 
to  the  changes  in  the  cartilages  elsewhere,  that  are  met  with  in  old  people. 


PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Hypertrophy  of  the  muscular  tissue  is  frequently 
found  in  chronic  bronchitis.  A  general  hyperplasia  of  the  bronchial 
wall  has  been  described  by  Heller  in  cases  of  bronchitis,  and  this  seems 
to  form  a  starting  point  for  many  varieties  of  tumors,  such  as  the  chon- 
droma,  osteoma,  myxoma,  papilloma,  lipoma,  adenoma,  and  mixed 

1  See  Hedinger,  Verb.  d.  deutsch.  path.  Ges.,  1904. 


280  THE  BRONCHI 

growths,  for  it  has  long  been  known  that  these  are  relatively  more 
frequent  in  connection  with  bronchiectasis  than  in  affections  of  the  lung 
proper. 

Tumors. — The  benign  growths  are  exceedingly  rare.  Malignant 
growths,  originating  as  primary  tumors  in  the  larger  bronchi,  are  uncom- 
mon. Those  beginning  in  the  finer  branches  cannot  be  distinguished 
from  those  beginning  in  the  lung  itself.  Carcinomas  either  originate 
from  the  lining  columnar  epithelium  or  from  the  peribronchial  mucous 
glands.  Squamous  epithelioma  of  a  bronchus  has  been  reported.1  This 
is  probably  due  to  metaplasia  of  the  lining  columnar  epithelium  of  the 
mucosa.  Sarcoma  and  lymphosarcoma  are  rare.  Metastatic  growths, 
however,  are  common  enough. 


ALTERATIONS  IN  THE  CONDITION  OF  THE  LUMEN. 

Perforation. — Perforation  of  the  bronchial  wall  may  arise  from  a 
variety  of  causes.  Tuberculous  or.  purulent  inflammation,  parasites, 
or  foreign  bodies  may  erode  through  the  bronchial  wall  into  the  lung 
substance.  Caseous  lymph-glands,  cancer  of  the  oesophagus,  abscesses, 
and  more  rarely  aneurisms,  may  burst  in  from  without. 

Bronchial  Occlusion. — Bronchial  occlusion  may  be  brought  about 
by  foreign  bodies,  such  as  corks,  slate-pencils,  or  bits  of  bone,  that 
have  been  accidentally  inhaled.  Exudates  and  excretions  sometimes 
also  collect  in  the  tubes,  and,  while  they  are  often  got  rid  of  by  coughing, 
they  may,  owing  to  inspissations  or  caseation,  form  a  permanent  obstruc- 
tion. Such  exudations  may  become  calcified  and  form  broncholiths. 
In  rare  instances,  as  in  a  case  recorded  by  one  of  us,2  the  occlusion  is 
brought  about  by  intrabronchial  tumors.  Pressure  from  without  is  a 
not  infrequent  cause,  and  is  usually  due  to  inflammatory  processes  in 
the  neighborhood  of  the  bronchus,  tumors  of  the  lung  or  esophagus, 
and  aortic  aneurisms.  By  far  the  most  potent  cause  is  inflammation 
of  the  bronchial  wall,  which  is  often  associated  with  inflammatory 
changes  in  the  lung  substance  in  the  immediate  neighborhood.  In 
such  cases  secretion  and  exudation  are  usually  present.  When  tuber- 
culosis is  the  underlying  condition,  the  bronchial  wall  is  often  caseous 
and  the  lumen  plugged  with  a  more  or  less  dry,  cheesy  mass.  In  some 
forms  of  inflammation,  notably  the  syphilitic,  the  process  as  it  heals 
leaves  a  contracting  scar-tissue,  that  gradually  squeezes  in  the  bronchial 
wall  and  thus  produces  distortion,  or  even  complete  occlusion.  In  those 
cases  where  the  obstruction  of  the  bronchus  is  complete,  the  corre- 
sponding lobule  of  the  lung  becomes  in  time  quite  collapsed,  owing  to 
the  absorption  of  the  contained  air.  When  the  obstruction  is  only  par- 
tial, the  alveoli  become  dilated  and  a  form  of  emphysema  is  the  result. 
Much  depends,  too,  upon  the  nature  of  the  obstructing  substance. 

1  P.  Ernst,  Ziegler's  Beitrage,  20:1896:155. 

2  Adami,  Montreal  Medical  Journal,  24:1895-96:510. 


BRONCHIECTASIS  281 

Tuberculosis  is,  of  course,  a  progressive  affection,  and  may  lead  to  a 
local  destruction  of  a  bronchus  and  of  the  lung  itself.  Even  when  the 
offending  substance  is  of  a  benign  nature,  secondary  infection  is  apt  to 
occur,  so  that  septic  pneumonia,  abscess,  and  even  gangrene  are  not 
infrequent  results.  In  many  instances  the  trouble  is  only  temporary, 
for  if  the  obstructing  substance  be  coughed  up,  restoration  to  the  normal 
condition  is  more  or  less  complete. 

Bronchiectasis. — Bronchiectasis  is  the  condition  in  which  the  bronchi 
are  dilated  and  often  distorted.  The  factors  concerned  in  its  production 
are  increased  intrapulmonary  pressure,  disease  of  the  bronchial  walls, 
pressure  of  accumulated  secretion  within  the  bronchi,  and  certain  chronic 
affections  involving  the  parenchyma  of  the  lung.  Usually  more  than 
one  cause  is  at  work  in  any  given  case.  According  to  the  appearance  of 
the  dilatations,  the  following  varieties  have  been  recognized :  the  saccular, 
the  cylindrical,  the  fusiform,  and  the  varicose.  The  condition  may  be 
localized  to  one  region  of  the  lung  or  may  be  disseminated.  In  the  sac- 
cular  form,  the  dilated  portion  is  somewhat  globular  in  shape  and  often 
more  or  less  eccentrically  situated;  in  the  cylindrical,  the  whole  tube 
is  evenly  dilated.  The  fusiform  and  the  varicose  are  sufficiently  de- 
scribed in  their  designations.  The  condition  of  the  bronchial  wall 
varies  a  good  deal  according  to  the  etiological  factor  at  work,  being  at 
one  time  dilated  and  thinned  (atrophic  form),  at  another  dilated  and 
thickened  (hypertrophic  form).  When  the  bronchial  wall  is  weakened, 
as,  for  instance,  from  long-continued  inflammation,  increased  pressure 
of  air  within  the  tube  will  naturally  produce  simple  dilatation  and  atrophy. 
In  the  atrophic  form  the  dilatation  is  usually  cylindrical,  but  if  the  pressure 
has  been  unevenly  exerted,  the  sacculated  form  will  be  produced. 
Within  the  lumen  the  wall  is  seen  to  be  encircled  by  a  number  of  sharp 
ridges  which  represent  the  muscular  and  elastic  constituents  of  the  wall, 
while  the  more  widely  dilated  portion  is  the  connective  tissue. 

The  hypertrophic  form  is  most  frequently  found  in  those  cases  where 
the  lung  is  fibroid  and  certain  portions  are,  as  a  consequence,  imperme- 
able to  air.  The  dilatation  is  not  so  much  due  to  weakness  of  the  walls 
as  to  irregular  pressure.  As  can  be  readily  understood,  when  a  portion 
of  the  lung  is  cut  off  in  any  way  from  its  air  supply  the  bronchi  of  the 
unaffected  portion,  are  subjected  to  increased  pressure  and  therefore 
dilate.  In  chronic  indurative  pneumonia  bronchiectasis  is  not  uncommon 
where  there  are  pleural  adhesions,  owing  to  traction  of  the  fibrous  bands 
upon  the  bronchi.  Great  dilatation  and  deformity  may  thus  result. 

We  may,  further,  recognize,  with  Orth,  a  primary  and  a  secondary 
bronchiectasis. 

In  the  primary  form,  chronic  catarrh  and  productive  inflammation  are 
the  most  potent  causes.  Owing  to  the  cellular  infiltration  and  the  exu- 
dative and  productive  processes,  the  bronchial  wall  becomes  less  resist- 
ant, so  that  it  gives  way  before  any  increased  demand  upon  it,  such  as 
is  made  by  prolonged  coughing,  and  difficult  respiration.  The  secondary 
variety  is  due  to  some  cause  apart  from  the  condition  of  the  bronchial 
wall.  The  forms  described  are  the  compensatory,  the  atelectatir, 


282  THE  BRONCHI 

the  cirrhotic,  and  the  paretic.  The  compensatory  form  is  seen  where 
a  portion  of  the  lung  is  compressed,  collapsed,  or  indurated,  so  that  the 
bronchioles,  in  part,  are  obstructed;  those  in  the  more  healthy  portion 
may  then  become  dilated.  This  affords  the  most  typical  example  of 
atrophic  bronchiectasis.  The  atelectatic  form  is  met  with  in  cases  where 
a  lobe  of  the  lung  is  collapsed  but  not  compressed,  as  in  pleural  effusions 
and  in  congenital  atelectasis.  The  bronchi  of  the  affected  portion  may 
dilate  and  their  walls  become  hypertrophic.  Cirrhotic  dilatation  is 
found  in  fibroid  lungs.  Here  the  condition  is  due  not  only  to  increased 
intrabronchial  pressure,  but  also  to  traction  upon  the  bronchial  wall 
by  the  newly-formed  fibrous  tissue.  In  the  paretic  form,  none  of  the 
ordinary  causes  are  at  work,  but  the  most  important  factor  is  the  pressure 
of  secretion  which  has  stagnated  in  some  portion  of  the  bronchial  tree. 

The  appearance  of  the  lung  in  bronchiectasis  will,  of  course,  vary 
according  to  the  causes  at  work.  The  condition  is  usually  recognized 
from  the  fact  that  the  bronchi  are  dilated  and  consequently  occupy 
more  than  they  should  of  the  pulmonary  area.  The  dilated  bronchioles 
can  often  be  traced  to  the  very  periphery  of  the  lung.  The  mucous 
membrane  is  reddened  and  there  is  more  or  less  secretion.  The  paren- 
chyma of  the  lung  may  present  the  appearance  of  compression,  collapse, 
or  fibroid  induration.  In  all  forms  of  bronchiectasis  the  mucous  mem- 
brane is  more  or  less  atrophic  and  infiltrated  with  inflammatory  products. 
The  cartilaginous  plates  are  degenerated  and  often  invaded  by  vascular 
newly-formed  fibrous  tissue.  The  ducts  of  the  mucous  glands  are  dilated. 
The  lining  ciliated  epithelium  is  sometimes  intact,  but  more  often 
desquamated  or  in  a  state  of  hypersecretion,  goblet  cells  being  quite 
numerous.  Very  rarely,  polypoid  outgrowths  of  the  mucosa  have  been 
met  with. 


CHAPTER    XIII. 

THE  LUNGS. 
CONGENITAL  ANOMALIES. 

The  anomalies  of  development  are  of  little  practical  importance. 

Complete  absence  of  both  lungs  has  been  found  in  acephalic  monsters, 
and  is,  of  course,  a  condition  quite  incompatible  with  life.  Complete 
absence  (agensia)  of  one  lung  and  imperfect  development  (hypoplasia) 
are  occasionally  met  with,  but  still  are  rare.  Again,  a  lobe  may  be 
rudimentary.  With  some  of  these  defects  bronchiectasis  may  be 
associated.  More  common  is  abnormal  lobation  of  the  lung,  which  is 
rather  more  frequent  on  the  right  side.  In  addition  to  the  usual  fissures, 
there  may  be  several  extra  ones  more  or  less  perfect  or,  again,  the  right 
lung  may  consist  of  only  two  lobes.  In  1500  autopsies  at  the  Royal  Vic- 
toria Hospital  abnormal  lobation  was  met  with  71  times;  on  the  right 
side,  54  times;  and  on  the  left  11;  on  both,  6. 

An  interesting  condition  is  that  in  which  the  right  apex  is  cleft  by 
a  fissure  passing  from  above  downward  and  inward.  In  the  cleft  is 
usually  a  fold  of  the  parietal  pleura,  along  the  edge  of  which  the  azygos 
vein  runs.  It  would  seem  probable  that  the  explanation  is  to  be  found 
in  an  abnormal  course  of  the  azygos  vein.  In  our  series  we  have 
met  this  condition  six  times.  A  supernumerary  left  lung  has  been  met 
with  by  Diirck.1 

Atelectasis  or  Apneumatosis. — Atelectasis  or  apneumatosis  is  a  per- 
sistence of  the  foetal  type  of  the  lung  tissue.  It  is  due  to  the  failure  of 
the  alveoli  to  expand.  This  may  be  caused  by  any  obstruction  to  the 
free  entrance  of  air,  to  pressure  on  the  lung,  and  to  general  feebleness. 
The  presence  of  this  condition  is  of  much  importance  in  medicolegal 
cases.  Congenital  atelectasis  is  also  brought  about  by  certain  intra- 
uterine  affections,  such  as  hydrothorax  or  pleurisy. 


,   CIRCULATORY  DISTURBANCES. 

A  consideration  of  the  finer  structure  and  the  vascular  supply  of 
the  lungs  will  throw  considerable  light  upon  the  pathological  processes 
that  affect  these  organs. 

The  lungs  receive  a  double  blood-supply,  from  the  right  ventricle 
through  the  pulmonary  artery,  and  from  the  aorta  through  the  bronchial 

1  Munch,  med.  Woch.,  42:  1895:456. 


284  THE  LUNGS 

arteries.  And  while  most  of  the  arteries  in  the  lungs  are  end-arteries, 
we  can  readily  understand  how  it  is  that  obstruction  in  one  set  of  vessels 
does  not  necessarily  result  in  cutting  off  the  blood  supply  from  the 
affected  part.  Thus  anemic  infarcts  are  unknown  and  necrosis  or  gan- 
grene, as  a  result  of  vascular  occlusion  alone,  is  almost  impossible, 
unless,  of  course,  both  sets  of  vessels  are  interfered  with  at  once. 

On  the  other  hand,  congestion  and  hemorrhage  are  very  common. 
This  is  due  to  the  fact  that  the  capillaries,  which  are  thin-walled,  con- 
sisting only  of  a  basement  membrane  and  a  single  row  of  cells,  are  but 
badly  supported,  the  alveolar  walls  in  which  they  run  being  made  up 
of  a  few  connective-tissue  and  elastic  fibrils.  The  structure  of  the  alve- 
olar walls  is  necessarily  delicate,  in  order  to  allow  of  the  free  interchange 
of  gases,  and  this  being  so,  the  capillaries  can  readily  become  overdis- 
tended  and  give  way. 

Further,  in  inflammatory  processes,  while  the  alveolar  walls  certainly 
become  swollen,  congested,  and  infiltrated,  the  inflammatory  products 
are  poured  out  into  the  alveolar  spaces,  and  thus  the  cardinal  features 
of  a  pneumonia  are  to  be  seen  in  the  alveolar  spaces  rather  than  in  the 
walls. 

As  will  readily  be  understood  from  what  has  just  been  said,  in  a 
delicate  structure  like  the  lung,  vascular  disturbances  make  their  appear- 
ance very  quickly  and  usually  produce  marked  signs.  Finally,  the 
close  relationship  between  vascular  disturbances  of  the  lungs  and  dis- 
eases of  the  heart  and  bloodvessels  is  sufficiently  obvious. 

Anemia. — Anemia  of  the  lungs  may  be  part  of  a  systemic  oligemia,  or 
may  be  partial  and  due  to  local  causes.  Thus,  pressure  of  a  pleuritic 
exudate  or  effusion,  or  of  a  mediastinal  growth,  may  lead  to  this  condition. 

In  emphysema,  there  is  always  a  certain  amount  of  anemia  owing  to 
the  fact  that  the  vessels  are  occluded  from  pressure,  atrophy,  and  endar- 
teritis.  The  anemia  that  is  part  of  a  systemic  condition  is  most  marked 
about  the  apex  and  the  anterior  border  of  the  lung.  The  organs  are 
pale,  colorless,  and  on  section  very  dry.  By  many  it  has  been  thought 
that  anemia  of  the  lungs,  such  as  is  brought  about  by  congenital  small- 
ness  of  the  pulmonary  artery  or  hypoplasia  of  the  cardiovascular  system 
as  a  whole,  is  a  potent  predisposing  cause  of  pulmonary  tuberculosis. 
According  to  Rindfleisch,  the  fact  that  the  apex  of  the  lung  is  the  first 
to  suffer  in  anemia  explains  why  tuberculosis  so  frequently  starts  in  that 
situation. 

(Edema. — This  is  of  frequent  occurrence,  being  found  at  almost 
every  autopsy — agonal  oedema.  The  condition  may  involve  the  whole 
lung  or  may  be  confined  to  one  or  more  lobes. 

The  lungs  are  somewhat  heavier  and  firmer  than  normal,  pit  on 
pressure,  and  on  section  the  tissue  generally  is  filled  with  a  rather  thin, 
watery  fluid,  which  can  readily  be  demonstrated  by  squeezing  the  lung 
between  the  hands.  When  the  fluid  is  removed  the  lung  regains  its 
normal  crepitant  condition.  The  fluid  is  largely  serous  in  character  and 
may  contain  a  few  red  cells  and  leukocytes,  as  well  as  swollen  epithelial 
cells,  loaded  with  coal  or  blood  pigment,  that  have  been  desquamated 


HYPEREMIA  285 

from  the  alveolar  walls.  The  most  frequent  cause  is  the  relaxation  of 
the  vessels  that  takes  place  just  at  the  time  of  death.  This  is  prob- 
ably assisted  by  the  toxic  condition  so  often  associated  with  the 
last  hours  of  life.  It  is  rather  curious  that  in  this  form  the  upper  lobes 
of  the  lungs  are  frequently  most  affected,  and  often  one  more  than  another. 
Why  this  should  be  has  never  been  adequately  explained. 

A  second  form — congestive  oedema — is  that  seen  in  connection  with 
passive  hyperemia,  in  which  case  the  posterior  portions  of  the  lungs  are 
most  affected.  Here  the  fluid  is  often  reddish  or  reddish-brown,  from 
admixture  with  blood  or  blood  pigment.  In  cases  of  long-standing, 
chronic  oedema  is  not  infrequently  found.  In  this  form  the  fluid  is 
more  viscid,  and  when  the  lung  is  squeezed  the  fluid  does  not  readily 
flow  away. 

A  third  form  is  the  inflammatory  oedema,  which  is  found  in  the  early 
stages  of  pneumonia,  at  the  periphery  of  pneumonic  patches,  and  occa- 
sionally in  cases  of  septicemia.  This  differs  from  the  other  varieties 
mentioned,  in  that  the  fluid  is  richer  in  albumin  and  apt  to  contain 
cellular  elements  in  greater  abundance.  This  form  readily  passes  over 
into  catarrhal  inflammation. 

A  fourth  form  is  the  acute  fulminating  oedema.  This  comes  on  sud- 
denly, sometimes  in  those  who  are  apparently  in  perfect  health.  Usually, 
however,  the  persons  attacked  are  the  subjects  of  arteriosclerosis  or 
chronic  nephritis.  Allbutt  has  observed  it  in  connection  with  aortitis. 
Cases  often  run  their  course  rapidly,  and  may  end  fatally  in  an  hour. 
In  one  case  coming  under  our  knowledge  pints  of  watery  fluid  poured 
out  of  the  mouth  for  some  hours  before  death,  and  at  autopsy  the  lungs 
were  completely  waterlogged.  The  condition  has  been  produced  experi- 
mentally by  the  injection  of  adrenalin. 

Microscopically,  oedema  of  the  lungs  shows  little  worthy  of  note. 
The  vessels  of  the  alveolar  wall  are  somewhat  congested,  and  the  alveoli 
contain  a  few  large,  round,  mononuclear  cells  derived  from  the  lining 
epithelium,  with  a  few  red  and  white  blood  cells.  Should  the  cedema- 
tous  fluid  contain  much  albumin,  although  it  usually  does  not,  the  albu- 
min appears  as  a  fine  granular  deposit  or  in  the  form  of  minute  clear 
globules  produced  by  the  coagulation  of  the  albumin  in  the  process  of 
hardening. 

Hyperemia. — In  the  production  of  the  various  forms  of  congestion 
of  the  lungs,  a  number  of  factors,  mechanical,  chemical,  and  thermic, 
are  concerned,  apart  from  the  question  of  inflammation.  Thus  heat, 
cold,  irritating  gases,  and  other  changes  in  the  respired  air  are  often 
responsible  for  the  condition.  Too  great  functional  activity,  such  as 
sometimes  arises  from  severe  muscular  strain,  leads  to  marked  hyperemia, 
which  in  some  cases  has  been  so  severe  as  to  cause  death  (apoplexia 
pulmonum  vascularis). 

Of  the  forms  due  to  mechanical  causes  might  be  cited  the  hyperemia 
of  the  lungs  which  is  found  so  often  in  cases  of  death  from  respiratory 
failure,  such  as  occurs  from  certain  lesions  producing  pressure  upon  the 
base  of  the  brain,  and  in  the  compensatory  forms,  as,  for  instance,  the 


286  THE  LUNGS 

hyperemia  of  one  lung  which  is  present  when  the  other  lung  is  collapsed 
or  compressed.  One  of  the  main  types  is  that  known  as  hypostatic  con- 
gestion. This  occurs  whenever  there  is  obstruction  to  the  free  outflow 
of  blood  from  the  lungs  or  deficiency  in  the  driving  power  of  the  heart 
or  arteries.  As  will  readily  be  understood,  the  lower  and  posterior 
portions  of  the  lungs  are  the  parts  first  and  chiefly  affected.  This  form 
is  seen  in  patients  that  have  been  confined  to  bed  for  a  length  of  time, 
or  where  there  is  degeneration  of  the  muscle  of  the  heart,  such  as  is  seen 
in  the  severer  forms  of  the  infective  fevers,  for  example,  typhoid  and 
pneumonia.  Mechanical  obstruction  within  the  lung  itself  may  be  at 
the  bottom  of  this,  or  the  heart  may  have  given  way  from  cloudy  or 
fatty  changes  and  dilatation.  It  is  also  obvious  that  this  condition  will 
be  aggravated  whenever,  as  so  frequently  is  the  case  in  severe  fevers, 
the  respiratory  movements  are  weak. 

Deficiency  in  the  driving  power  of  the  right  heart  is  a  potent  cause, 
as  it  allows  the  blood  to  remain  in  the  lesser  circulation  longer  than  it 
should  do  normally.  A  weak  left  heart,  by  lessening  the  power  of  the 
general  circulation,  acts  in  a  similar  way,  but  to  a  less  extent. 

The  most  marked  example  of  the  obstructive  form  is  that  due  to 
insufficiency  or  stenosis  of  the  mitral  valve,  or  to  increased  pressure 
within  the  systemic  circulation.  In  a  marked  case,  the  lung  is  somewhat 
enlarged,  its  consistency  increased,  and  its  elasticity  diminished.  On 
section  the  tissue  may  not  be  cedematous  but  is  full  of  blood  of  venous 
appearance.  The  color  is  dark  red,  or  purple  red. 

Being  a  chronic  condition,  there  is  apt  to  be  proliferation  of  the 
fibrous  tissue  in  the  septa  of  the  lung,  so  that  the  tissue  becomes  firmer 
than  normal,  the  so-called  cyanotic  induration.  In  cases  of  a  longer 
standing  still,  more  or  less  blood  is  effused  by  diapedesis,  and  the  red 
cells  are  eventually  broken  down,  liberating  the  blood  pigment.  In 
such  cases  the  fibrosis  is  still  more  marked.  This  condition  is  called 
brown  induration,  from  the  fact  that  the  cut  surface  of  the  lung  is  of  a 
dark,  rusty  brown  color.  On  squeezing  such  a  lung,  the  fluid  which  is 
exuded  is  also  of  a  brownish  color,  and  if  a  little  of  it  be  examined 
under  a  microscope  it  is  seen  to  contain  large,  clear,  mononuclear  cells 
loaded  with  brownish  pigment.  These  are  the  so-called  "  heart-failure" 
cells. 

Microscopically,  the  appearances  in  congestion  of  the  lungs  are 
characteristic.  The  vessels  are  found  to  be  distended  with  blood.  This 
is  extremely  well  seen  in  the  delicate  capillaries  of  the  alveolar  walls, 
which  can  be  made  out  to  be  filled  with  red  blood^  cells.  They  are  over- 
distended,  so  that  they  assume  a  beaded  or  varicose  appearance  and 
project  into  the  alveolar  spaces.  An  occasional  red  cell  may  be  seen 
free  in  the  alveolar  space. 

In  the  chronic  cases,  such  as  those  due  to  a  long-standing  valvular 
lesion  of  the  heart,  there  is  in  addition  to  the  appearances  just  men- 
tioned a  general  thickening  of  the  fibrous  septa  of  the  lung,  which  is,  in 
places,  infiltrated  with  small,  round  cells,  probably  young  fibroblasts. 
Besides  this,  in  the  alveolar  spaces  there  are  often  considerable  numbers 


HEMORRHAGE  287 

of  cells  of  the  heart-failure  type  just  mentioned.  These  have  the  ap- 
pearance of  large  mononuclear  cells,  containing  the  shadows  of  red 
cells,  or  blood  and  coal  pigment.  They  are  derived  either  from  the 
desquamated  lining  cells  of  the  alveolar  walls  or  are  wandering  cells. 
In  the  advanced  cases  the  pigment  is  not  confined  to  the  desquamated 
cells,  but  is  found  in  the  connective  tissue,  lymphoid  and  epithelial  cells 
as  well. 

Hemorrhage. — Effusions  of  blood  within  the  lung  substance  may 
affect  the  parenchyma  of  the  organ  or  may  involve  the  various  air  spaces. 
The  latter  event  is  by  far  the  more  common,  owing  to  the  anatomical 
peculiarities  of  the  lung  before  referred  to.  As  a  rule,  the  blood  is  de- 
rived from  the  pulmonary  vessels,  but  occasionally  it  comes  from  outside 
the  lung  altogether,  as,  for  instance,  when  an  aneurism  of  the  aorta 
bursts  into  a  bronchus. 

The  effused  blood  may  be  small  in  amount,  taking  the  form  of  petechial 
spots,  varying  in  size  from  that  of  a  pin-head  to  that  of  a  bean,  or  large 
areas  may  be  involved,  even  to  the  extent  of  a  whole  lobe.  The  smaller 
hemorrhages  are  usually  but  imperfectly  defined  from  the  unaffected  lung 
tissue  and  more  or  less  patchy  in  character.  In  the  case  of  the  larger 
areas,  notably  in  infarcts,  the  line  of  demarcation  is  usually  very  distinct. 

With  regard  to  the  ultimate  causes  of  these  effusions,  it  may  be  said 
in  general  that  the  extravasation  is  due  to  alterations  in  the  vessel 
walls  or  to  the  obstruction  of  the  lumen.  Thus,  in  the  early  stages  of 
lobar  and  lobular  pneumonia,  blood  may  be  extra vasated  in  large  amounts, 
owing  to  diapedesis.  In  a  few  cases,  as  in  vicarious  menstruation, 
it  would  appear  that  the  condition  is  due  to  some  defect  in  the  innerva- 
tion  of  the  vessel  walls,  for  the  lung  is  in  such  cases  practically  intact. 
Any  solution  of  continuity  of  a  vessel  wall  is  also  a  cause,  such  as  may 
result  from  fatty  and  hyaline  degeneration,  ulceration,  or  traumatism. 
A  similar  condition  of  things  is  found  in  connection  with  lesions  of  the 
pons,  medulla,  and  occasionally  the  cerebral  cortex. 

The  traumatic  form  is  seen  in  wounds  of  the  lung  produced  by  shoot- 
ing or  stabbing,  or  by  a  broken  rib.  In  rare  instances,  rupture  of  the 
lung  occurs  from  severe  strain  without  any  of  these  causes. 

By  far  the  most  common  cause  of  the  larger  hemorrhages  is  ulceration 
or  degeneration  of  the  vessel  walls  in  the  course  of  necrotic  or  tuber- 
culous inflammation  of  the  lung  substance. 

It  occasionally  happens  that  in  gangrene  of  the  lung  a  vessel  is  opened 
and  free  hemorrhage  takes  place  into  the  cavity,  which  may  eventually 
discharge  into  a  bronchus.  This  is,  however,  a  much  more  frequent 
event  in  chronic  ulcerative  tuberculosis. 

In  the  course  of  the  caseous  and  necrotic  inflammations  which  result 
in  cavity  formation,  the  fibrous  septa  and  bloodvessels,  which  are  the 
most  resistant  of  the  tissues,  are  laid  bare.  Thus,  the  bloodvessels 
are  found  projecting  in  loops  from  the  wall  of  the  cavity  or  running 
in  fibrous  bands  across  the  space,  where  they  are  badly  supported.  The 
vessels  do  not  escape,  however,  but  are  the  seat  of  a  panarteritis  and 
endarteritis,  which  in  some  cases,  but  not  invariably,  leads  to  oblitera- 


288  THE  LUNGS 

tion  of  the  lumen.  The  vessel  wall  becomes  thickened  through  a  chronic 
productive  inflammation,  and  from  the  inner  layers  a  fibrinohyaline 
degeneration  sets  in,  which  gradually  spreads  to  the  periphery  and  may 
eventually  involve  the  whole  circumference.  In  this  way,  the  vessel 
wall  becomes  soft  and  is  no  longer  able  to  resist  the  pressure  of  the  blood. 
Usually  some  part  of  the  wall  gives  way  and  an  aneurism  is  formed 
or  rupture  takes  place.  These  aneurisms  vary  in  size  from  that  of  a 
pin-head  to  a  cherry,  and  may  fill  up  the  entire  space  of  the  cavity. 
Rupture  readily  takes  place,  and  the  resulting  hemorrhage  may  be  very 
severe  or  even  fatal.  Often,  however,  death  is  not  brought  about  by 
a  single  hemorrhage,  but  by  repeated  attacks,  leading  to  oligemia  and 
cachexia.  Post  mortem,  it  is  not  always  easy  to  find  the  aneurism  or 
ruptured  vessel,  for  when  the  bleeding  has  been  repeated  and  prolonged, 
the  vessel  is  often  obliterated  owing  to  pressure  and  inflammation. 

Perhaps  the  most  striking  form  of  hemorrhage  into  the  lung  is  the 
hemorrhagic  infarct.  This  is  found  most  frequently  in  the  right  lung 
and  in  the  lower  lobe,  but  may  be  multiple  and  occur  in  both  lungs. 

In  a  well-marked  case,  the  infarct  is  cone-shaped  and  more  or  less 
sharply  defined  from  the  rest  of  the  lung.  It  is  usually  situated  near 
the  pleural  surface,  the  apex  of  the  cone  being  innermost.  The  pleura 
over  the  affected  part  is  elevated,  of  a  dark,  purplish-red  color,  and  the 
lung  is  felt  to  be  occupied  by  a  firm  mass.  The  pleura  in  the  early 
stages  is  still  quite  smooth,  but  in  a  case  of  some  standing  is  covered 
with  a  slight  fibrinous  deposit,  due  to  reactive  inflammation.  On 
cutting  through  the  mass,  the  affected  area  is  found  to  be  solid,  friable, 
and  of  a  dark,  purplish-red  or  rusty-brown  color.  The  edges  may 
be  quite  sharply  defined  from  the  rest  of  the  lung,  or  the  infarct  may  be 
of  a  more  patchy  character.  In  an  infarct  of  some  standing,  there  may 
be  slight  softening  and  the  apex  and  edges  of  the  cone  are  somewhat 
grayish  in  color,  due  in  part  to  a  deposit  of  fibrin  and  a  leukocytic 
infiltration.  On  scraping,  the  surface  is  at  first  dry  and  granular, 
subsequently  more  juicy.  We  have  met  with  a  case  of  complete  lique- 
faction of  the  area  with  the  formation  of  a  ragged  cavity. 

Microscopically,  the  changes  in  the  affected  tissue  are  very  plain. 
The  alveolar  spaces  are  filled  with  red  blood  cells  so  as  to  be  quite  air- 
less. An  occasional  leukocyte  or  thread  of  fibrin  may  also  be  seen. 
The  alveolar  walls  are  thin  and  compressed,  the  nuclei  staining  badly 
or  not  at  all.  The  various  interlobular  septa  are  free  from  any  extrava- 
sation. About  the  margins  of  the  infarct,  where  a  certain  amount  of 
reactive  inflammation  is  taking  place,  the  leukocytes  are  more  numerous 
in  the  alveolar  spaces  and  the  fibrin  is  more  abundant.  The  lymph 
channels  about  the  periphery  of  the  infarct,  in  addition  to  the  perivascu- 
lar  and  peribronchial  lymphatics,  are  filled  with  reabsorbed  blood  cells. 

As  the  infarct  ages,  a  characteristic  series  of  changes  takes  place. 
The  extra vasated  cells  gradually  break  down  and  the  pigment  is 
set  free  in  brownish  granular  masses.  Gradually  the  circulation  is 
restored  through  the  bronchial  arteries,  the  alveoli  become  emptied, 
and  the  part  becomes  functional.  Rarely  do  we  have  necrosis  and 


FAT  EMBOLISM  289 

cicatrization  as  in  iufarcts  elsewhere.  A  rare  event  is  for  the  centre  of 
the  infarct  to  soften  and  then  become  absorbed,  producing  a  form  of 
cyst;  or  the  infarcted  area  may  become  infiltrated  with  calcareous  salts 
and  a  hard  nodule  be  left.  In  those  cases  where  the  infarct  is  infected 
at  the  start,  or  becomes  so  later,  a  septic  pneumonia  may  develop  with 
the  formation  of  a  lung  abscess  or  a  patch  of  gangrene. 

With  regard  to  the  etiology  of  infarct  of  the  lung,  much  has  been 
written.  A  variety  of  factors  seems  to  enter  into  the  causation.  The 
most  important  and  constant  is  the  condition  of  the  bloodvessels.  As 
a  majority  of  instances  of  lung  infarct  arise  in  connection  of  valvular 
lesions  of  the  heart,  emphysema,  inflammations,  senile  and  marantic 
conditions  of  the  general  system,  we  can  look  for  a  main  contributory 
cause  in  the  fatty  and  hyaline  degeneration  of  the  capillaries  and  smaller 
vessels  which  is  so  often  present  in  such  cases.  A  sudden  disturbance 
of  the  blood  pressure  of  the  part  or  a  loss  of  the  normal  vascular  balance 
which  should  exist  between  the  different  portions  of  a  lung  area  are 
powerful  factors  also,  since  such  may  lead  not  only  to  degenerative  changes 
in  the  vessels  themselves,  but  to  actual  rupture  of  their  walls. 

The  most  frequently  found  condition  is  a  clot  within  the  main  vessels 
leading  to  the  infarcted  area.  The  pulmonary  artery  at  the  apex  of 
the  wedge-shaped  mass  is  usually  stopped  by  a  thrombotic  embolus. 
The  pulmonary  vein  may  be  thrombosed,  but  it  may  not  infrequently  be 
free  from  clot,  except  in  its  terminal  ramifications.  The  vessels  affected 
may  appear  to  be  comparatively  healthy,  or  they  may  show  fatty  and 
other  endarteritic  changes.  Sometimes  the  clot,  from  its  condition  and 
appearance,  can  be  clearly  made  out  to  be  an  embolus  which  has  reached 
the  lung  from  some  other  part.  This  is  seen  not  infrequently  in  cases 
of  endocarditis,  and  when  thrombi  in  the  larger  vascular  trunks  have 
become  dislodged.  In  the  latter  cases  the  emboli  may  be  so  large  as  to 
occlude  the  main  trunk  of  the  pulmonary  vein  and  lead  to  instant  or 
rapid  death.  The  condition  of  thrombosis  of  the  crural  veins  or  of  the 
uterine  veins  after  childbirth  is  of  much  importance  in  this  connection. 

The  various  theories  regarding  the  mode  of  formation  of  these  in- 
farcts  have  already  been  discussed  on  page  39  et  seq. 

In  connection  with  infarction  we  have  spoken  so  far  only  of  emboli 
that  are  formed  of  blood  clots,  but  on  occasion  other  substances,  such 
as  fat,  air,  cells  from  tumors,  fragments  of  vessels,  calcareous  salts, 
and  parasites  enter  the  vessels,  although  they  do  not  by  any  means  always 
result  in  infarction. 

Embolism. — Fat  Embolism. — Fat  embolism  occurs  whenever  fat  in  any 
quantity  gains  entrance  into  the  blood.  Thus,  in  the  case  of  fractures  of 
the  long  bones,  crushing  injuries  to  the  body  wall  or  the  liver,  the  lipemia 
of  diabetes,  fat  droplets  can  be  found  in  the  capillaries  not  only  of  the 
lungs,  but  of  the  other  organs  as  well  (see  p.  54).  A  rapid  method  for 
demonstrating  the  condition  is  to  cut  a  thin  slice  of  tissue  with  a  double- 
bladed  knife,  which  can  then  be  examined  directly  with  the  low  power. 
The  fat  in  the  larger  vessels  looks  like  an  elongated  drop  of  some  semi- 
fluid substance,  clear  and  refractile.  In  the  smaller  capillaries  the  droplets 
19 


290  THE  LUNGS 

have  a  more  varicose  appearance,  or  the  tissue  may  be  treated  with 
Sudan  III  or  osmic  acid,  when  the  fat  takes  either  a  red  or  brownish- 
black  stain. 

Air  Embolism. — Air  embolism  is  apt  to  occur  when  from  any  cause 
a  large  venous  trunk  is  laid  open  to  the  air  (see  p.  55). 

Emboli  composed  of  portions  of  tumors  are  important,  since  it  is  in 
this  way  that  metastases  in  the  lungs  are  usually  produced. 


CONDITIONS   DUE  TO  DISTURBANCE   OF  THE  RESPIRATORY 

FUNCTION. 

Atelectasis. — By  this  is  commonly  meant  the  condition  in  which 
the  alveolar  spaces  are  either  partially  or  completely  undistended  by 
the  air.  The  term  "  atelectasis"  is,  strictly  speaking,  only  applicable 
to  cases  where  the  lung  has  undergone  primary  imperfect  expansion. 
When  complete  airlessness  exists,  "  apneumatosis"  is  the  better  term. 

The  condition  is  congenital  or  acquired.  Before  birth  the  lung  is  a 
solid  organ,  the  alveolar  walls  lying  in  close  contact.  The  lining  epi- 
thelium of  the  alveoli  is  composed  of  small  polyhedral  cells  and  the 
smaller  bronchioles  are  thrown  into  folds  longitudinally.  Thus,  not 
actual  but  only  potential  air  spaces  exist.  With  the  first  inspirations 
the  lung  begins  to  expand,  the  alveolar  walls  are  thrust  apart,  and  the 
lining  cells  are  converted  into  flattened  plates.  The  bronchioles  there- 
upon assume  their  permanent  shape.  The  condition  of  the  lungs  in  the 
newborn  is  of  great  importance  from  a  medicolegal  standpoint,  to  deter- 
mine whether  the  infant  has  breathed  or  not. 

A  number  of  causes  may  contribute  to  the  persistence  of  the  foetal 
condition.  Such  are,  general  asthenia  and  muscular  weakness  commonly 
present  in  premature  children;  weakness,  owing  to  a  syphilitic  or  other 
constitutional  taint;  lesions  of  the  central  nervous  system,  like  cerebral 
hemorrhage  or  similar  lesions  of  the  brain;  defects  of  development, 
such  as  hypoplasia  of  the  lung  and  diaphragmatic  hernia;  finally  external 
causes,  as  compression  of  the  thorax,  obstruction  of  the  bronchi  by 
foreign  bodies,  meconium,  or  secretion,  all  may  play  a  part. 

The  causes  of  the  acquired  form  may  be  included  under  the  general 
heads  of  (1)  defective  respiratory  function  within  the  lung  itself,  and 
(2)  external  mechanical  pressure. 

Of  the  first  type  the  commonest  form  is  that  found  in  cachectic  or 
moribund  individuals.  Owing  to  weak  inspiratory  movements  and  the 
accumulation  of  fluid  in  the  air  passages,  the  lung  tissue  is  not  perfectly 
distended.  Consequently,  small  areas,  chiefly  at  the  margins  of  the 
lungs,  become  partially  or  wholly  collapsed.  This  is  among  the  com- 
monest conditions  found  post  mortem. 

Another  form  is  that  where  the  bronchial  tree  in  some  portion  becomes 
occluded  (obstructive  atelectasis),  as  from  a  foreign  body,  the  accumula- 
tion of  secretion  in  the  lumen,  and  intrabronchial  and  intrapulmonary 
tumors.  The  collapse  in  these  cases  is  brought  about  in  part  by  the 


ATELECTASIS 


291 


obstructing  material  acting  as  a  kind  of  valve,  whereby  air  does  not 
readily  enter  the  affected  portion  while  it  passes  freely  out.  This 
mechanical  action  is,  however,  not  entirely  adequate  to  explain  the 
condition,  for  the  whole  of  the  contained  air  could  not  be  got  rid  of  in 
that  way.  The  residual  air  is  absorbed  into  the  blood  (absorption 
atelectasis)  and  the  collapse  thus  becomes  complete. 

External  pressure  upon  the  whole  lung  or  any  part  of  it  is  a  very 
frequent  cause  of  atelectasis  (compression  atelectasis).  Pressure  may 
be  exerted  by  fluid  or  air  within  the  pleural  cavity,  an  elevated  diaphragm, 
aneurisms  or  tumors  in  the  mediastinum,  kyphosis  and  scoliosis  of  the 
vertebral  column,  enlargement  of  the  heart  or  pericardial  cavity, 
thickening  and  contraction  of  the  pleura.  The  effect  is  due  not  only 
to  direct  pressure,  but  also  to  the  interference  with  the  proper  respiratory 
movements. 


: 

>'    ^iivT-j. 


Compression  collapse  of  the  lung.  Note  the  fact  that  the  alveolar  spaces  are  obliterated,  the 
walls  being  in  contact.  Zeiss  obj.  DD,  ocular  No.  1.  (From  the  Pathological  Laboratory  of 
McGill  University.) 

Atelectasis  may  affect  one  or  both  lungs,  one  lobe,  or  any  portion 
thereof.  When  the  lung  as  a  whole  is  atelectatic,  as,  for  instance,  in 
hydrothorax,  it  lies  close  to  the  spinal  column,  somewhat  high  up  in  the 
thorax.  Its  volume  is  much  diminished  and  the  surface  is  thrown  into 
fine  wrinkles.  The  consistence  is  increased  and  the  organ  has  a  leathery 
feel,  the  normal  crepitation  of  the  healthy  lung  being  absent.  In  color 
it  is  usually  purplish  or  brownish-red.  If,  as  sometimes  happens, 
the  tissue  is  ischemic  from  pressure,  it  assumes  more  of  a  grayish  or 
slaty  appearance.  On  section,  the  lung  does  not  crepitate,  and  resembles 
flesh,  The  affected  part  sinks  in  water.  If  squeezed  below  the  surface 


292  THE  LUNGS 

of  the  water  a  few  air  bubbles  may  sometimes  be  expressed.  In  most 
collapsed  lungs  there  is  a  tendency  to  vascular  stasis,  so  that  the  cut 
surface  is  congested.  When  only  a  portion  of  a  lobe  is  collapsed,  the 
affected  part  is  of  a  dark  purplish-red  color,  and  somewhat  sunken  below 
the  general  level  of  the  lung.  The  pleura  is  smooth  and  the  tissue  has 
all  the  characteristics  already  described. 

Microscopically,  the  alveolar  walls  are  found  to  be  in  more  or  less 
close  contact,  so  that  the  alveolar  spaces  are  obliterated.  Occasionally, 
in  the  infundibula,  groups  of  epithelial  cells  can  be  made  out.  The 
cells  lining  the  alveoli  have  resumed,  in  fact,  the  embryonic  type.  The 
bronchioles  are  corrugated  and  the  bloodvessels  are  everywhere  congested. 
Sometimes  small  hemorrhages  and  masses  of  blood-pigment  can  be 
observed. 

When  of  long  standing,  atelectasis  is  frequently  complicated  with  other 
conditions.  Thus,  in  infants,  bronchiectasis  has  been  observed.  Again, 
from  the  fact  that  the  atelectatic  portion  is  usually  congested  while 
the  cause  of  the  condition  is  often  an  inflammatory  one,  infection  readily 
takes  place.  Bronchopneumonia,  therefore,  is  not  an  infrequent  sequel. 
In  other  cases,  the  lung  undergoes  a  chronic,  indurative  change,  owing 
to  proliferation  of  the  fibrous  tissue,  fatty  degeneration  of  the  alveolar 
epithelium,  and  becomes  permanently  collapsed  and  carnified. 

Emphysema. — Emphysema  of  the  lungs  is  one  of  the  commonest 
pathological  conditions.  The  term  "emphysema"  was  originally  applied 
to  a  condition  in  which  air  is  found  in  the  tissues  (interstitial  emphysema) , 
but  is  very  appropriately  applied  to  that  affection  of  the  lungs  in  which 
they  are  overdistended  and  contain  more  than  the  normal  amount  of  air. 

The  condition  may  be  generalized,  affecting  nearly  the  whole  of  both 
lungs,  or  may  be  restricted  to  certain  parts.  In  an  advanced  and  typical 
case  of  generalized  emphysema  the  patient  has  a  characteristic  appear- 
ance. The  neck  is  short  and  thick;  the  chest  is  enlarged  in  all  its 
diameters,  but  particularly  anteroposteriorly,  so  that  it  assumes  a  barrel 
shape.  The  abdomen  is  relatively  sunken  and  the  accessory  muscles 
of  respiration  are  well  developed. 

When  the  thorax  is  opened,  the  lungs  are  more  voluminous  than  normal, 
encroaching  upon  the  cardiac  area,  and  do  not  collapse.  The  costal 
cartilages  are  lengthened  and  sometimes  calcified.  The  lungs  are 
considerably  enlarged,  although  their  weight  may  be  actually  dimin- 
ished. The  tissue  is  inelastic  and  much  less  crepitant  than  normal, 
retaining  the  impress  of  the  fingers  and  having  the  feel  of  a  bag  of 
feathers.  The  pleural  surface  is  very  pale,  and  the  pigmentation  of  the 
lung  is  not  so  conspicuous  as  it  usually  is.  With  a  hand  lens,  or  even 
with  the  naked  eye,  small  vesicles,  the  size  of  a  pin-head  or  less,  which 
represent  the  enlarged  alveolar  spaces,  can  be  seen  on  the  surface.  In 
advanced  cases  these  vesicles  may  attain  the  size  of  peas,  or  even  larger, 
giving  the  lung  a  bullous  appearance  (emphysema  bullosa).  In  the  larger 
vesicles  can  often  be  traced  the  fragments  of  what  were  originally  the 
alveolar  walls.  On  section,  the  lung  is  pale  and  dry  and  the  larger  vesicles 
collapse.  These  can,  however,  be  again  distended  with  water.  Associ- 


/,XN/;.\77.t/v  EMPHYSEMA  293 

ated  with  the  condition  is  usually  ;i  certain  amount  of  chronic  bronchitis, 
with  some  thickening  of  the  bronchi  and  pulmonary  septa.  Bronchiec- 
tusis,  however,  is  not  often  met  with.  In  less  extreme  cases,  the  apices, 
the  anterior  borders,  and  the  inner  surface  near  the  root  are  the  parts 
affected.  These  areas  are  somewhat  distended  or  inflated  as  compared 
with  the  rest  of  the  lung  and  present  the  characters  just  mentioned. 

Several  classifications  of  emphysema  have  been  proposed.  The  usual 
one  of  hypertrophic,  atrophic,  and  compensatory  forms,  while  a  useful 
clinical  division,  is  open  to  some  criticism  from  the  point  of  view  of  the 
pathologist.  Perhaps  the  best  general  division  is  into  vesicular  and 
inlerxtiliul  emphysema.  In  the  first  variety  it  is  the  alveolar  spaces  that 
are  distended  and  contain  a  surplus  of  air.  In  the  second,  the  air  is 
present  in  the  interstitial  tissue  of  the  lungs. 

Vesicular  emphysema  may  further  be  subdivided  into  (1)  essential 
or  substantial  emphysema,  and  (2)  complementary. 

Essential  Emphysema. — Various  grades  of  essential  emphysema  exist, 
which  pass  imperceptibly  one  into  the  other.  Orth  recognizes  three 
forms:  (1)  Simple  emphysema,  in  which  the  lung  is  simply  inflated  with 
air  to  its  full  capacity,  or,  in  other  words,  is  in  a  permanent  inspiratory 
position.  This  form  is  Traube's  Volumen  Pulmonum  Auctum,  and 
while,  on  anatomical  grounds,  it  is  correctly  differentiated  from  the 
other  forms  of  emphysema,  it  cannot  be  separated  clinically  from  them 
by  any  definite  signs.  (2)  Ectatic  emphysema.  This  is  a  farther  stage 
in  which  the  limit  of  physiological  dilatation  of  the  alveoli  is  overstepped 
and  the  spaces  become  enlarged  though  without  actual  atrophy. 

The  structural  changes  present  consist  in  dilatation  of  the  alveolar 
spaces  in  connection  with  certain  infundibula.  The  alveolar  walls 
become  gradually  stretched  and  thinned,  and  the  spaces  tend  to  assume 
a  globular  form.  Thus,  the  multiloculated  structure  of  the  lung  becomes 
much  simplified.  (3)  Atrophic  emphysema.  In  this  variety,  the  alveolar 
walls  atrophy  and  the  vessels  gradually  become  obliterated.  Rupture 
of  the  air  spaces  is  here  a  striking  feature.  Several  alveolar  spaces  are 
thrown  into  one,  so  that  actual  bullse  are  the  result. 

In  atrophic  emphysema,  the  microscopic  changes  are  well  marked. 
The  alveolar  walls  are  thinned  and  in  many  cases  broken.  Thus,  we 
see  large  spaces  which  are  clearly  due  to  the  coalescence  of  several 
adjacent  alveoli,  for  the  remains  of  the  ruptured  walls  can  be  seen  pro- 
jecting into  the  cavity.  The  bloodvessels  are  small  and  in  many  places 
obliterated.  The  atrophy  affects  first  and  chiefly  the  bloodvessels  and 
elastic  tissue,  the  connective  tissue  being  more  resistant.  By  special 
preparation,  the  lining  epithelium  can  be  recognized  on  the  alveolar 
walls.  The  cells  are,  of  course,  forced  apart  by  the  process  of  distension, 
but  isolated  ones  can  be  made  out  here  and  there  on  the  walls,  many 
of  them  fattily  degenerated.  In  the  infundibula  small  groups  of  these 
cells  can  be  found.  The  bronchi  usually  show  signs  of  chronic  inflam- 
mation and  the  septa  are  infiltrated.  This  is  not  a  feature  of  the  emphy- 
sema, however,  but  is  due  to  the  inflammation  and  catarrh  which  is  so 
frequent  an  accompaniment  of  the  disease. 


294  TffE  LUNGS 

Complementary  Emphysema. — Complementary  emphysema  is  gener- 
ally localized  to  one  or  more  lobules,  but  may,  under  certain  circum- 
stances, affect  the  whole  lung.  Local  emphysema  is  quite  a  common 
condition,  and  is  frequently  only  a  temporary  state,  due  not  to  structural 
changes  in  the  lung,  but  brought  about,  for  the  most  part,  by  irregularity 
in  the  intra-alveolar  pressure.  Thus,  in  pneumonia,  fibroid  induration 
and  in  tuberculosis,  where  a  portion  of  the  lung  becomes  impervious  to 
air,  the  remaining  air  sacs  are  subjected  to  increased  pressure  and  in 
consequence  become  distended.  As  soon  as  the  cause  is  removed  the 
affected  air  sacs  return  to  their  normal  state,  but  in  prolonged  or 
repeated  attacks  the  temporary  may  give  place  to  a  permanent  condition. 
The  distension  of  the  alveoli  is  due  to  a  compensatory  process  and  is 
found  in  the  neighborhood  of  the  atelectatic  or  consolidated  areas  of 
the  lung. 

FIG.  69 


Emphysema  of  lung.      Leitz  obj.  No.  7,  without  ocular.      The  atrophied  alveolar  wall  and  the 
rupture  of  several  of  them  are  well  shown.      (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

Interstitial  Emphysema. — Interstitial  emphysema  is  a  relatively  unim- 
portant condition.  In  this  form  air  is  present  in  small  beads  in  the 
interstitial  tissue.  The  air  bubbles  resemble  a  string  of  beads  and  may 
clearly  define  the  various  lobules.  The  bubbles  rarely  attain  any  size 
and  can  be  pushed  along  the  septa  with  the  finger.  This  form  may  be 
present  in  ordinary  emphysema  due  to  rupture  of  an  alveolus,  in  injuries 
of  the  lung,  and  in  those  who  have  died  of  suffocation.  The  artificial 
inflation  of  the  lungs  of  the  asphyxiated  newborn  infant  has  been  known 
to  produce  it.  The  condition  is  usually  met  with  beneath  the  pleura, 
but  may  spread  to  the  hilus  of  the  lungs  and  even  to  the  mediastinal 
tissues. 


PNEUMONIA 

A  word  should  be  said  about  the  conditions  due  to  and  associated 
with  pulmonary  emphysema.  In  advanced  cases  there  is  nearly  always 
chronic  bronchitis  and  sometimes  bronchiectasis.  Owing  to  the  in- 
creased intrapulmonary  pressure  the  right  side  of  the  heart  becomes 
hypertrophied,  and  eventually  the  whole  heart  may  become  hyper- 
trophied  and  dilated  with  evidences  of  valvular  incompetency,  arterio- 
sclerosis being  also  a  not  infrequent  accompaniment.  The  peculiar 
changes  in  the  chest  have  already  been  referred  to. 

In  senile  atrophy  of  the  lung,  which  is  not  properly  emphysema  at  all, 
though  classed  with  it  by  many  writers,  the  thorax  and  indeed  the  whole 
body  is  small  and  shrunken,  the  ribs  close  together,  and  very  obliquely 
situated,  so  that  they  approximate  very  closely  to  the  crest  of  the  ilium. 
The  lungs  in  such  cases  are  small  and,  as  Jenner  expressed  it,  feel  like 
an  inflated  bag  of  wet  paper. 


INFLAMMATIONS. 

Pneumonia. — Pneumonia  is  to  be  referred  to  the  direct  irritant 
action  of  microorganisms  upon  the  lung  substance.  While,  however, 
certain  clinical  types  are  recognized  on  the  ground  of  the  anatomical 
distribution  of  the  lesions  and  a  well-defined  clinical  course,  it  should 
be  remembered  that  there  are  many  intermediate  and  atypical  forms, 
so  much  so  that  to  the  bacteriologist  pneumonia  is  not  a  single  disease 
entity,  but  rather  a  multiplicity  of  pathological  manifestations  dependent 
on  a  variety  of  causes. 

Numerous  inquiries  into  the  etiology  of  acute  pneumonia  have  been 
made  in  recent  years,  and  almost  all  the  known  pathogenic  microorgan- 
isms have  been  proved  to  be  capable  of  producing  pulmonary  inflam- 
mation.1 Among  these  may  be  mentioned  the  Fraenkel-Weichselbaum 
Diplococcus  of  pneumonia,  Friedlander's  pneumobacillus,  Streptococcus 
pyogenes,  Staphylococcus  albus  and  aureus,  B.  tuberculosis,  B.  typhi 
abdominalis,  B.  coli  communis,  B.  influenzse,  B.  pestis,  B.  anthracis, 
B.  diphtherise,  B.  enteritidis  of  Gaertner.  Any  one  of  these  alone  is 
able  to  cause  the  affection,  but  mixed  infections  are  common.  To 
produce  the  disease  it  is  necessary  for  the  bacteria  to  invade  the  lung, 
and  this  they  do,  either  through  the  bronchi  (inhalation  or  aerogenic 
pneumonia)  or  through  the  blood  and  lymph-stream  (hematogenic, 
lymphogenic  pneumonia).  Many  of  the  germs  above  mentioned  have 
been  found  in  the  buccal  secretion  of  healthy  people,  so  that  it  is  not 
surprising  that  inhalation  or  bronchogenic  pneumonia  is  a  common 
affection.  Recent  investigations  go  to  prove  that  the  lungs  and  peri- 
bronchial  glands  play  an  important  role  in  the  protection  of  the  organism 
from  infective  agencies  coming  from  without  (see  vol.  i,  p.  290). 

BartheP  found  in  the  healthy  lung  both  pathogenic  and  non-patho- 

1  For  a  study  of  the  bacteriology  of  pneumonia,  see  Curry,  Jour,  of  Exper.  Med., 
4:  1899: 169. 

2  Centralbl.  f.  Bakt.,  24: 1898: 11. 


296  THE  LUNGS 

genie  bacteria  in  the  trachea  and  larger  bronchi,  though  the  bronchioles 
and  alveoli  were  free.  The  bacteria,  of  which  the  diplococcus  was  most 
frequently  present,  were  more  abundant  in  the  lung  than  in  the  mouth, 
a  condition  of  things  due  possibly  to  local  growth  or  to  the  inhibiting 
and  eliminating  power  of  the  saliva.  The  later  researches  of  Beco,1 
on  the  whole,  confirm  this  view. 

It  would  appear,  too,  that  bacteria  may  pass  by  means  of  the  lymphatics 
through  the  lung  without  eliciting  any  manifestation  of  their  presence. 
Whether  pneumonia  is  produced  or  not  must  depend,  therefore,  upon 
some  second  cause,  either  increased  virulence  of  the  bacteria  or  dimin- 
ished resisting  power  on  the  part  of  the  organism.  In  the  case  of  the 
Diplococcus  pneumonias,  increased  virulence  is  scarcely  likely  to  be  an 
important  factor,  for,  as  we  know,  this  germ,  when  freely  exposed  to  the 
air,  rapidly  loses  its  power,  and  is  readily  killed  out  by  the  presence  of 
certain  other  microorganisms.  Any  diplococci,  therefore,  in  the  mouth 
or  respiratory  tract  are  likely  to  be  so  weakened  that  in  many  instances 
they  are  non-pathogenic.  Susceptibility  seems  to  vary  also  in  different 
individuals  and  races.  Thus,  for  man  the  Diplococcus  pneumonia?  is 
comparatively  mild,  for  the  majority  of  cases,  at  least  in  healthy  adults, 
get  well.  In  the  case  of  some  of  the  lower  animals,  such  as  mice  and 
rabbits,  the  infection  is  very  severe,  so  much  so  that  when  infected  they 
die  from  general  bacteriemia  rather  than  pneumonia.  It  may  be  re- 
marked, however,  in  passing,  that  the  oftener  careful  bacteriological 
studies  are  made,  the  more  frequently  do  we  find  that  pneumonia,  even 
in  man,  is  to  be  regarded  as  a  generalized  infection  with  a  local  mani- 
festation, as  is  the  case  also  with  typhoid  fever  and  some  other  infections. 
Any  circumstance  that  leads  to  a  lowered  vitality  of  the  organism  may 
be  a  predisposing  or  contributory  cause,  such  as  previous  attacks,  wast- 
ing diseases,  alcoholism,  diabetes,  B  right's  disease,  pulmonary  lesions, 
traumatism  to  the  lung,  bodily  injuries,  chronic  nervous  disorders,  and 
exhausting  occupations.  Climate,  season,  and  age  are  of  some  impor- 
tance, as  affecting  the  resisting  power  of  the  system.  "  Catching  cold" 
does  not  seem  to  have  the  importance  that  once  was  thought.  The 
influence  of  traumatism  is  shown  very  prettily  in  a  case  cited  by  Luca- 
tello,2  where,  in  a  patient  in  previously  perfect  health,  pneumonia  devel- 
oped a  few  days  subsequently  to  a  severe  contusion  of  the  shoulder. 

On  account  of  the  anatomical  structure  of  the  lungs,  inflammatory 
processes  occurring  therein  differ  materially  from  those  met  with  in  the 
secreting  glands.  The  chief  characteristic  of  pulmonary  inflammation  is 
that  the  inflammatory  products  are  poured  out  into  the  alveolar  spaces, 
while  the  cells  lining  these  spaces  undergo  degeneration  and  desquama- 
tion.  The  process  may  be  more  or  less  confined  to  the  alveolar  walls, 
but  in  many  cases  the  interlobular  septa  are  involved,  so  that  an  inter- 
stitial process  is  at  work  as  well.  The  changes  are  exudative  and  des- 
quamative  rather  than  parenchyma tous. 

1  Arch,  de  m6d.  exp£r.  et  d'anatom.  path.,  May,  1899. 

2  Centralbl.  f.  Bakt.,  8:  1890:  239. 


PNEUMONIA  207 

The  anatomical  picture  in  pneumonia  varies  greatly.  Sometimes  i In- 
whole  or  the  greater  part  of  a  lobe  is  affected,  hence  the  term  lobar 
pneumonia.  At  other  times  the  disease  is  confined  to  the  lobules — lobular 
pneumonia — or  to  minute  but  numerous  portions  of  the  lobules — 
miliary  pneumonia. 

The  infection  is  in  many  cases  derived  from  the  air  passages,  and  to 
an  important  group  of  these,  in  which  the  process  seems  to  begin  in  the 
smaller  bronchioles  and  extends  to  the  alveoli,  the  term  bronchopneu-. 
monia  has  been  given.  The  miliary  form  is  due  to  infection  through  the 
blood  stream.  In  certain  other  cases,  inflammation  begins  in  the  neigh- 
boring tissues  and  spreads  to  the  lung  by  means  of  the  lymphatics.  An 
important  type  of  this  form  is  the  so-called  pleurogenetic  pneumonia, 
where  the  disease  begins  in  the  pleura  and  extends  as  a  lymphangitis 
and  perilymphangitis  of  the  lung  substance. 

In  most  varieties  of  pneumonia  the  bronchi  are  more  or  less  affected, 
either  primarily  or  secondarily,  and  the  peribronchial  lymph-glands  are 
enlarged,  succulent,  and  inflamed. 

Pneumonia  may  be  acute  or  chronic,  depending  upon  the  nature  of 
the  offending  microorganism  and  the  resisting  power  of  the  tissue. 

It  is  perhaps  difficult  to  account  satisfactorily  for  the  variability  in 
the  distribution  of  the  lesions.  Certain  germs  like  the  Diplococcus 
pneumonise  and  the  Pneumobacillus  of  Friedlander  tend  to  produce  the 
lobar  variety,  while  others  like  the  pus  cocci,  the  Bacillus  of  influenza, 
and  the  B.  coli,  nearly  always  produce  a  lobular  inflammation.  The 
explanation  probably  is  that  the  Diplococcus  pneumonia,  being  relatively 
non-malignant  in  man,  can  only  act  in  the  event  of  a  locus  resistentice 
minoris  being  formed,  and  this  in  ordinary  healthy  people  is  apt  to  be 
present  in  only  one  portion  of  the  lung.  Lobular  pneumonia,  however, 
while  it  does  occasionally  develop  as  a  primary  affection,  nearly  always 
occurs  in  those  previously  weakened  by  disease.  It  is  characteristically 
a  "terminal"  infection,  and  as  in  all  chronic  cases  the  lungs  are  uniformly 
weakened,  not  only  from  degenerative  processes,  but  also  from  defective 
respiration,  any  infective  agents  present  in  the  respiratory  passages  are 
likely  to  exert  a  disseminated  activity.  The  hematogenic  and  lympho- 
genic  forms  are,  of  course,  more  easily  understood. 

Another  important  feature  in  the  differentiation  of  the  various  pneu- 
monias is  the  character  of  the  exudate.  From  this,  we  are  often  enabled 
to  decide  as  to  the  nature  of  the  infecting  microorganism.  According 
to  the  acuteness  of  the  case,  the  inflammatory  products  are  exudative 
or  productive,  and  either  element  may  predominate  over  the  other. 
In  one  common  form,  the  so-called  "fibrinous"  or  "croupous"  pneu- 
monia, due  to  the  Diplococcus  pneumonia?,  the  alveoli  are  filled  with  a 
rather  dry,  granular  exudate  of  a  grayish  color.  When  resolution  is 
commencing,  the  alveolar  contents  can  be  expressed  in  the  form  of  little 
plugs.  In  the  form  due  to  the  Friedlander  bacillus,  the  lung  is  congested- 
looking,  juicy,  and  the  cut  surface  has  the  appearance  of  being  coated 
with  gelatin.  The  exudate  can  be  scraped  off  with  the  knife,  and  is  so 
viscid  that  it  depends  in  long,  glairy  strings  from  the  knife. 


298  THE  LUNGS 

Acute  Lobar  Pneumonia. — In  acute  lobar  pneumonia  (Fraenkel- 
Weichselbaum  diplococcus)  the  lung  is  usually  said  to  pass  through  four 
stages:  (1)  Engorgement,  (2)  red  hepatization,  (3)  gray  hepatization, 
and  (4)  resolution.  This  is  a  convenient  division  for  purposes  of  descrip- 
tion, but  it  is  very  doubtful  how  far  it  is  warranted  by  the  actual  facts. 
The  mode  of  development  of  the  lesions  is  certainly  quite  varied  and  a 
uniform  order  is  not  always  adhered  to.  It  is  not  uncommon  to  find 
several  of  the  above-mentioned  stages  present  simultaneously  in  the  lung. 
There  can  be  no  doubt,  however,  that  the  inflammation  begins  with  con- 
gestion followed  by  consolidation.  The  first  two  stages  are  rarely  ever 
seen  except  in  persons  dying  by  accident,  or  in  limited  areas  at  the  edge 
of  a  creeping  pneumonia,  for,  as  might  be  supposed,  the  cases  that  come 
to  autopsy  are  in  the  advanced  stages.  In  the  period  of  engorgement 
the  condition  is  that  of  a  simple  active  or  inflammatory  hyperemia. 
The  lung  is  redder  than  normal  and  possibly  slightly  edematous.  Micro- 
scopically, the  capillaries  in  the  alveolar  walls  are  congested  and  varicose, 
the  epithelial  cells  are  swollen  and  occasionally  desquamated,  and 
there  may  be  an  occasional  red  corpuscle  in  the  alveolar  spaces.  In  the 
second  stage  the  affected  portion  of  the  lung  is  swollen,  heavier,  and 
firmer  than  normal,  pitting  on  pressure,  and  somewhat  friable.  It  is 
intensely  red,  and  on  section  an  abundant  turbid,  blood-stained  fluid  can 
be  squeezed  out.  Although  the  exudate  appears  reddish  and  contains 
a  majority  of  red  corpuscles,  even  at  this  early  stage  numerous  leukocytes 
are  present.  Microscopically,  the  capillaries  are  greatly  congested, 
the  lining  epithelium  of  the  alveolar  spaces  is  swollen,  and  the  cells 
are  found  in  all  stages  of  proliferation  and  desquamation.  The  alveolar 
spaces  are  more  or  less  filled  with  red  blood  cells,  and  desquamated 
epithelial  cells — the  so-called  "catarrhal"  cells — enmeshed  in  fibrin, 
but  numerous  leukocytes  can  be  seen.  A  round-celled  infiltration  is 
also  to  be  noted  about  the  vessels  of  the  interlobular  septa.  Owing  to 
the  solid  appearance  of  the  lung  and  its  reddish  color,  it  was  compared 
by  the  older  pathologists  to  the  liver,  whence  the  term  "red  hepatization." 
Imperceptibly  the  condition  passes  on  into  the  third  stage,  that  of 
gray  hepatization.  Here  the  lung  is  still  more  swollen,  so  that  it  shows 
the  impression  of  the  ribs,  is  heavy  and  firm  to  the  touch.  The  pleura 
has  lost  its  glassy  appearance,  is  granular  and  cloudy,  and  covered  with 
a  varying  amount  of  fibrinous  exudation.  In  fact,  every  acute  lobar 
pneumonia  is  also  a  pleuropneumonia.  The  lung  is  quite  airless, 
friable,  and  sinks  in  water.  On  section,  the  surface  is  granular,  and, 
according  to  the  age  of  the  process,  of  a  color  varying  from  dark  red 
through  the  different  shades  of  reddish-gray  to  gray  or  yellow.  This, 
with  the  deposit  of  coal  pigment  so  often  present,  gives  the  lung  a  curious 
mottled  appearance  that  has  been  compared  to  granite.  The  character- 
istic ashen-gray  color  is  due  in  part  to  the  leukocytic  exudation  and 
in  part  to  the  anemia  of  the  tissue  produced  by  the  pressure  of  the 
alveolar  contents  upon  the  vessels.  On  scraping  the  surface,  a  small 
amount  of  granular  material  can  be  removed.  The  bronchioles  of  the 
affected  area  are  usually  blocked  with  fibrin.  Microscopically,  the 


ACUTE  LOBAR  PNEUMONIA 
FIG.  70 


299 


Acute  lobar  pneumonia  (gray  hepatization).      The  lower  lobe  is  involved. 
Pathological  Laboratory  of  the  Royal  Victoria  Hospital.) 

FIG.  71 


(From  the 


Acute  lobar  pneumonia.      Leitz  obj.  No.  7,  without  ocular.      The  alveolar  spaces  are  filled  with 
leukocytes  and  fibrin.      (From  the  collection  of  Dr.  A.  G.  Nicholls.) 


300  THE  LUNGS 

alveolar  walls  are  compressed  and  the  capillary  channels  are  obliterated. 
The  exudate  is  made  up  almost  entirely  of  leukocytes  and  fibrinous 
threads,  with  an  occasional  erythrocyte  and  catarrhal  cell.  By  Weigert's 
method  the  fibrin  threads  can  be  beautifully  demonstrated  and  may 
sometimes  be  seen  passing  through  the  stomata  from  one  alveolar  space 
to  the  other. 

When  the  fibrin  is  beginning  to  break  up  and  become  granular,  and  the 
leukocytes  show  advanced  fatty  degeneration,  the  stage  of  resolution  is 
being  initiated.  The  lung  begins  to  shrink,  the  pleura  is  relaxed  and 
thrown  into  folds,  and  the  organ  has  a  boggy  feel.  On  section,  the  tissue 
is  grayish  verging  on  yellow,  and  is  moist,  so  that  a  fluid  not  unlike 
pus  can  be  expressed.  On  scraping,  little  plugs  of  fibrin  and  leukocytes 
readily  come  away.  Microscopically,  the  appearances  are  not  unlike 
the  last  stage,  except  that  the  alveolar  capillaries  are  again  becoming 
permeable,  the  fibrin  threads  are  broken  up  into  a  granular  debris,  and 
the  leukocytes  especially  about  the  margin  of  the  clot  show  signs  of 
fatty  degeneration  and  solution  (autolysis).  Later,  regeneration  of  the 
alveolar  epithelium  takes  place.  The  exudation  is  removed  chiefly  by 
the  lymphatics  but  also  to  some  extent  through  expectoration.  The  lym- 
phatics are  frequently  found  distended  with  leukocytic  and  fibrinous 
exudation.  Indeed,  a  true  lymphangitis  and  perilymphangits  may  occur, 
so  that  the  framework  of  the  lung  is  profoundly  involved  in  the  inflam- 
matory process.  Should  the  lymphatics  be  damaged  by  previous  disease, 
as,  for  instance,  from  emphysema,  absorption  is  rendered  so  much  the 
more  difficult.1 

As  has  been  remarked,  it  is  by  no  means  necessary  for  pneumonia  to 
pass  regularly  through  the  stages  described.  Clinical  experience  teaches 
us  that  some  cases  attain  their  acme  very  rapidly  and  subside  in  two  or 
three  days  instead  of  lasting  ten  or  more,  as  is  the  rule.  Some  variation 
in  the  amount  of  exudation  occurs  also.  In  children,  the  aged,  and  the 
asthenic,  the  amount  of  fibrin  produced  may  be  small  and  the  alveoli 
not  greatly  distended,  so  that  the  usual  dry,  granular  appearance  of  the 
lung  is  not  observed. 

As  to  the  site  of  the  pneumonic  process,  according  to  Orth,  52  per 
cent,  occur  on  the  right  side,  33  per  cent,  on  the  left,  and  15  per  cent, 
on  both.  When  both  lungs  are  involved  it  is  common  to  find  the  process 
less  advanced  in  one  than  in  the  other.  One  lung  may  be  in  the  stage 
of  gray  hepatization,  while  the  other  shows  merely  engorgement  or  red 
hepatization.  The  portions  of  the  lung  uninvolved  in  the  consolidation 
are  not  free  from  pathological  change.  They  are  usually  much  con- 
gested and  there  may  be  local  emphysematous  dilatation  of  the  alveoli 
of  a  compensatory  character. 

With  regard  to  the  distribution  of  the  lesions  in  the  lung,  the  lower 
lobe  of  the  right  lung  is  the  one  most  frequently  affected,  next  to  this 
the  lower  lobe  of  the  left  lung.  The  tendency  is  for  a  whole  lobe  to 

1  For  a  study  of  the  histological  appearances,  see  Pratt,  Jour.  Boston  Soc.  of  the 
Med.  Sci.,  4:1900:183. 


ACUTE  LOBULAR  PNEUMONIA  301 

be  involved.  Atypical  forms  are,  however,  not  infrequently  met  with, 
as,  for  instance,  the  "central"  pneumonia,  where  the  process  begins 
about  the  hilus  and  is  most  in  evidence  in  the  centre  of  the  lung.  In 
other  cases  the  disease  may  be  localized  to  a  small  area  in  the  apex  of 
the  lung — "apical"  pneumonia.  The  presence  of  such  a  focus,  by  the 
way,  should  always  raise  the  suspicion  of  tuberculosis.  A  curious  form 
is  the  so-called  "creeping"  pneumonia,  in  which  the  consolidation 
presents  various  stages  of  development,  at  one  part  resolution  being  in 
progress,  at  another  fresh  pneumonic  infiltration.  This  conforms  to  a 
well-known  clinical  type. 

It  is  the  rule  in  young  and  otherwise  healthy  persons  for  resolution 
to  take  place,  but,  apart  from  a  lethal  termination,  certain  other  results 
may  follow.  Not  infrequently,  tuberculosis  is  superadded  and  the 
case  progresses  instead  of  resolving.  Rarely  the  exudate  dries  up  within 
the  alveoli  into  a  granular  caseous  mass,  so  that  the  condition  resembles 
massive  tuberculosis.  Again,  secondary  infection  may  take  place,  and 
if  pyogenic  or  putrefactive  microorganisms  be  present,  abscess]  or  gan- 
grene may  result.  Further,  there  is  a  close  relationship  between 
abscess  of  the  lung  and  gangrene,  for  the  one  may  initiate  the  other. 
While  it  must  be  admitted  that  the  pneumococcus  is  capable  of  producing 
a  suppurative  and  necrosing  process,  it  is  probable  that  this  is  a  rare 
event.  No  doubt,  owing  to  the  close  resemblance  between  a  pneumonic 
exudate,  when  undergoing  liquefaction  and  absorption,  and  pus,  two 
different  conditions  have  been  confused.  Gangrene  is  said  to  be  more 
frequent  than  abscess.  Here  the  lung  is  converted  into  a  foul,  pulpy 
mass  of  dark  greenish  color.  When  softening  has  taken  place,  irregular 
cavities  with  shaggy,  necrotic  walls  are  seen.  A  line  of  demarcation  is 
not  usually  formed.  Gangrene  is  specially  apt  to  supervene  in  those 
cases  where  there  is  putrid  bronchitis  and  bronchiectasis,  where  the 
circulation  is  poor,  and  in  cases  where  a  hemorrhagic  exudate  is  a  promi- 
nent feature.  The  most  common  sequel,  however,  is  fibroid  induration 
of  the  lung.  Here,  with  the  signs  of  delayed  or  absent  resolution,  the 
exudate  becomes  organized  by  a  process  similar  to  that  occurring  in 
a  thrombus.  The  affected  part  is  enlarged,  very  firm  and  heavy,  cutting 
with  some  difficulty.  The  color  is  reddish  or  reddish-gray,  mixed  with 
the  black  of  the  coal  pigment,  and  the  septa  are  prominent  as  bands 
of  grayish-white  appearance.  Thus,  a  peculiar  marbled  appearance  is 
the  result.  The  pleura  is  also  greatly  thickened.  If  the  patient  live 
long  enough  the  exudate  is  absorbed  and  the  lung  shrinks  into  a  hard, 
irregular,  fibrous  mass. 

Acute  Lobular  Pneumonia  (Catarrhal  Pneumonia,  Bronchopneumonia). — 
This  form  differs  somewhat  from  lobar  pneumonia  in  that  the  exudate 
tends  to  be  catarrhal  rather  than  fibrinous.  The  process  is  associated 
with  bronchitis,  and,  indeed,  almost  always  starts  with  inflammation  of 
the  smaller  bronchioles,  which  spreads  to  the  adjacent  alveoli  (bronchitis 
and  peribronchitis).  This  is  the  "capillary  bronchitis"  of  the  older 
writers.  The  exudate  is  at  first  serous  and  contains  a  few  erythrocytes, 
but  more  numerous  white  cells.  A  striking  feature  is  the  great  abundance 


302  THE  LUNGS 

of  the  so-called  "catarrhal"  cells,  large  mononuclear  cells  with  clear 
protoplasm.  These  are  believed  by  many  to  be  swollen  and  desquamated 
epithelial  cells  from  the  alveolar  walls,  and,  while  they  are  present  in 
all  forms  of  pneumonia,  they  are  specially  numerous  in  the  lobular 
variety. 

Only  exceptionally  does  this  disease  occur  as  a  primary  affection. 
As  a  rule,  it  is  a  sequel  of  bronchitis  or  a  complication  of  the  infective 
fevers,  such  as  measles,  scarlatina,  whooping-cough,  diphtheria,  influ- 
enza, typhoid,  and  variola.  It  is  generally  met  with  in  the  young  or 
aged,  or  in  those  debilitated  from  any  cause.  Particularly  is  it  liable  to 
happen  in  the  bedridden  and  those  suffering  from  congestive  conditions 
of  the  lungs  (hypostatic  pneumonia).  A  class  of  cases  worthy  of  special 
note  is  that  due  to  the  inhalation  of  infective  material  from  the  mouth  and 
upper  air  passages  (aspiration  pneumonia).  This  is  met  with  after 
operations  upon  the  nose  and  mouth  and  in  certain  nervous  diseases 
with  involvement  of  the  vagus  nerve  (vagus  pneumonia).  The  micro- 
organisms at  work  here  are  the  same  as  in  the  lobar  form,  but  there  is 
a  greater  tendency  for  the  pyogenic  cocci  to  be  concerned.  The  disease 
usually  affects  both  lungs,  but  may  involve  only  one,  or  even  a  single  lobe 
or  portion  thereof.  The  affected  organ  is  heavier  than  normal,  some- 
what congested,  and  in  its  substance  can  be  felt  areas  of  increased  con- 
sistence. These  are  friable,  of  a  reddish-gray,  gray,  or  grayish-yellow 
color,  contrasting  somewhat  with  the  rest  of  the  lung.  In  other  cases  the 
whole  or  the  greater  part  of  a  lobe  is  consolidated,  but  irregularly  so, 
pointing  to  the  origin  of  the  condition  in  the  coalescence  of  isolated 
foci.  On  pressure,  a  turbid  blood-stained  fluid  can  be  expressed,  in 
which  may  be  seen  small  particles  of  a  more  gray,  grayish-yellow,  or 
purulent  appearance. 

These  often  represent  the  contents  of  the  bronchioles.  From  the 
sporadic  distribution  of  the  consolidated  areas,  the  term  "  splenization" 
has  been  given  to  the  condition.  As  in  the  lobar  form,  both  red  and 
gray  stages  are  recognized.  On  section,  the  lung  as  a  whole  is  markedly 
hyperemic.  The  smaller  bronchi  and  bronchioles  show  inflammation 
and  are  filled  with  exudate.  In  the  alveolar  spaces  the  exudate  consists 
mainly  of  serum,  a  few  red  cells,  abundant  leukocytes,  and  "catarrhal" 
cells.  These  catarrhal  cells  frequently  contain  pigment  and  bacteria. 
Fibrin  is  not  a  striking  feature,  and  in  this  the  lobular  differs  materially 
from  the  lobar  form.  At  most,  a  few  of  the  alveoli  at  the  periphery  of 
a  consolidated  patch  may  contain  a  few  threads  of  fibrin.  Consequently, 
granulation  of  the  lung  is  absent.  It  is  worthy  of  note  that  pneumonic 
infiltration  may  supervene  upon  the  previous  collapse  of  a  pulmonary 
lobule.  The  explanation  is  simple,  since  bronchitis  and  obstruction  of 
the  bronchial  lumen  lead  alike  to  collapse  and  bronchopneumonia. 

In  the  form  due  to  the  presence  of  foreign  bodies  in  the  bronchi  or 
to  the  inhalation  of  infective  material,  the  exudation  may  become 
purulent.  Such  a  condition  usually  leads  to  a  diffuse  purulent  infiltra- 
tion of  the  lung  and  eventually  abscess. 

Like  lobar  pneumonia,  the  lobular  form  presents  several  variations  in 


SEPTIC  OR  PURULENT  PNEUMONIA  303 

its  course  and  development.  It  is  a  serious  affection,  since  it  nearly 
always  attacks  those  of  low  vitality,  or  complicates  other  grave  disease. 
A  fatal  termination  is  therefore  quite  frequent.  When  resolution  does 
occur,  it  takes  place  quickly,  for  a  relatively  small  area  of  lung  sub- 
stance is  involved,  and  the  lymphatics  are  less  interfered  with.  In  certain 
cases,  when  the  inflammatory  process  has  originated  in  an  area  of  old 
fibroid  or  caseous  pneumonia,  the  secretion  tends  to  become  fatty  and 
finally  inspissated,  so  that  the  affected  area  remains  consolidated  and 
appears  somewhat  gelatinous  with  yellowish  specks.  This  condition  was 
called  by  Virchow  "chronic  catarrhal  pneumonia."  Occasionally 
abscess  and  gangrene  of  the  lung  may  result.  This  is  the  case  in  aspira- 
tion pneumonias,  in  the  weak,  and  those  with  general  circulatory  dis- 
turbance. In  another  class  of  cases  local  areas  of  induration  result 
with  bronchiectasis.  An  important  result  is  a  secondary  infection  with 
the  tubercle  bacillus,  so  that  a  miliary  or  caseous  bronchopneumonia 
results. 

Septic  or  Purulent  Pneumonia. — The  characteristics  of  this  form  are 
its  great  intensity,  a  purulent  exudation  into  the  alveolar  spaces  and  into 
the  interstitial  tissues,  and  a  tendency  to  destruction  of  the  lung.  Ex- 
pectoration in  some  cases  is  profuse,  and  the  sputum  may  contain  elastic 
tissue  and  other  fragments  of  lung  substance.  The  process  is  rarely 
primary,  but  usually  occurs  as  a  sequel  or  complication  of  some  other 
condition.  It  is  due  to  the  action  of  pyogenic  microbes  that  reach 
the  lung  through  the  air  passages,  the  bloodvessels,  and  lymphatics. 
The  type  of  the  bronchogenic  form  is  the  so-called  "aspiration"  pneu- 
monia. According  to  the  virulence  of  the  infecting  agent  a  simple 
catarrhal  pneumonia  may  be  the  first  result,  leading  eventually  to  sup- 
puration or  rapid  destruction  with  gangrene.  One  or  both  lungs  may 
be  affected  and  the  lower  lobes  are  the  seat  of  election.  According  to 
the  character  and  amount  of  the  aspirated  material,  scattered  foci  of 
infiltration,  separated  one  from  the  other  by  comparatively  healthy 
lung  tissue,  may  be  seen,  or  a  diffuse  and  confluent  condition.  The 
affected  lung  is  usually  congested  and  oedematous,  and  the  abscesses  can 
frequently  be  recognized  on  the  surface  as  nodules  of  a  reddish  or  reddish- 
yellow  color,  over  which  the  pleura  shows  some  cloudiness  and  injection. 
On  section,  the  nodules  referred  to  are  seen  to  be  due  to  a  more  or  less 
complete  consolidation.  The  centre  is  apt  to  be  broken  down  and  com- 
posed of  thick,  yellowish,  or  blood-stained  pus.  About  the  affected  areas 
there  is  generally  a  zone  of  intense  hyperemia.  The  tissue  is  very  friable, 
and  if  the  process  be  of  some  standing,  cavitation  may  be  observed. 
Sometimes  gangrene  supervenes  and  then  the  affected  part  is  of  a  dirty, 
greenish  color  with  a  foul  odor.  The  bronchioles  are  often  plugged 
with  exudate. 

Microscopically,  the  parenchyma  of  the  lung  is  intensely  congested. 
The  smaller  abscesses  are  composed  of  an  intense  intra-alveolar  and  inter- 
stitial leukocytic  infiltration,  forming  a  local  cellular  focus,  the  centre 
of  which  stains  badly  and  contains  leukocytes  in  all  stages  of  degenera- 
tion and  debris.  In  the  neighborhood,  the  capillaries  of  the  alveolar 


304  THE  LUNGS 

walls  are  much  congested,  and  there  is  a  certain  amount  of  oedema  and 
desquamation  of  the  lining  cells,  together  with  some  diapedesis  of  the 
red  and  white  cells.  Frequently,  little  clumps  of  bacteria  can  be  seen 
in  the  centre  of  the  mass.  Stained  with  hematoxylin,  these  are  a  purple- 
black  color. 

Metastatic  or  Septic  Embolic  Pneumonia. — Metastatic  or  septic  embolic 
pneumonia  is  more  common  than  the  last  form,  and  is  the  type  of  purulent 
pneumonia.  It  is  hematogenic  in  origin  and  due  to  the  lodging  of 
multiple  infecting  agents  in  the  vessels  of  the  lung.  It  is  commonly 
met  with  as  a  manifestation  of  general  septicemia,  and  results  from  such 
conditions  as  osteomyelitis,  thrombophlebitis,  septic  arthritis,  septic 
endometritis,  malignant  endocarditis,  erysipelas,  and  the  like.  In 
cases  of  infarction  of  the  lung,  when  due  to  an  embolus  containing 
pyogenic  microorganisms,  suppuration  will  result.  In  other  cases 
clusters  of  bacteria  are  found  obstructing  the  capillaries,  and  thus 
bring  about  the  condition.  It  is  not  likely  that  germs  are  ever  in  the 
blood  in  such  quantities  as  to  form  emboli,  but  it  is  more  probable  that 
one  or  two  become  entangled  in  the  lining  cells  of  the  vessels  and, 
being  strong  enough  to  overcome  the  defensive  power  of  the  cells,  pro- 
liferate there. 

Both  lungs  are  usually  uniformly  affected,  but,  exceptionally,  only  a 
few  lobules,  chiefly  those  of  the  lower  lobes.  The  abscesses  appear  as 
multiple  nodular  swellings  beneath  the  pleura,  varying  in  size  from  that 
of  a  pin-head  to  a  cherry  or  even  larger.  The  areas  are  usually  more  or 
less  spherical  or,  if  the  condition  have  originated  in  infarction,  irregularly 
wedge-shaped.  The  overlying  pleura  is  congested  and  cloudy  and 
generally  covered  with  fibrinopurulent  exudation.  On  section,  the 
lung  is  filled  with  inflammatory  foci  in  all  stages,  from  simple  consolida- 
tion or  purulent  infiltration  to  actual  abscess  and  excavation.  The  color 
varies  from  reddish-yellow  to  yellow.  In  the  earlier  stages  there  is 
not  much  destruction  of  tissue,  but  softening  soon  occurs  and  the  lung 
has  the  appearance  of  a  loose,  spongy  matrix  filled  with  a  thick,  reddish- 
yellow  purulent  fluid.  Sequestration  of  the  tissue  is  not  uncommon, 
and  in  advanced  stages,  on  washing  out  the  pus,  the  abscesses  are  re- 
vealed as  cavities  with  dirty,  necrotic  walls,  surrounded  by  a  zone  of 
intense  inflammatory  hyperemia.  In  cases  which  have  lasted  some  time 
the  cavities  may  be  bounded  by  a  rather  dense  layer  of  infiltration  walling 
off  the  abscesses  more  or  less  completely,  the  so-called  "  pyogenic" 
membrane.  In  certain  cases  of  intense  infection  gangrene  supervenes. 
Microscopically,  the  picture  is  much  like  that  in  other  forms  of  suppura- 
tive  pneumonia,  with  the  exception  that  the  process  is  more  localized, 
The  minuter  abscesses  show  merely  collections  of  small  round  cells, 
but  the  larger  ones  stain  badly  in  the  centre,  owing  to  necrosis.  (Edema, 
congestion,  and  hemorrhage  in  the  neighborhood  are  marked  features. 

Besides  the  bronchogenic  and  hematogenic  forms  just  described,  local 
suppurative  and  gangrenous  inflammation  may  occur  in  the  lung  as  the 
result  of  traumatism. 

Perhaps  more  frequently  than  in  any  other  organ  secondary  suppura- 


CHRONIC  PNEUMONIA  305 

tion  is  met  with,  complicating,  for  instance,  fibrinous  pneumonia,  chronic 
ulcerative  tuberculosis,  actinomycosis,  and  echinococcus  disease.  Buhl 
has  described  a  purulent  peribronchitis  that  may  lead  to  septic  pneumonia. 

Septic  Pneumonia  by  Extension. — Another  important  form  of  septic 
pneumonia  is  that  arising  by  extension.  Abscesses  of  the  liver,  sub- 
phrenic  abscess,  and  suppuration  of  the  lymphatic  glands  may  extend 
to  the  lung.  Frequently,  the  infection  is  by  way  of  the  pleural  cavity  and 
lymphatics  (pleurogenic).  This  gives  rise  to  a  suppurative  interstitial 
lymphangitis,  and  is  said  to  be  most  common  in  children,  especially  in 
connection  with  empyema.  In  this  case  the  subpleural  lymphatics  are 
dilated,  varicose,  and  of  a  yellowish  color.  On  section,  the  lobules  are 
found  to  be  separated  from  each  other  by  broad  succulent  yellowish 
bands  representing  the  connective-tissue  septa.  The  process  may  go  on 
to  the  extent  that  portions  of  the  lung  tissue  are  sequestrated  and  cast 
off  (pneumonia  dissecans  of  Ziegler).  The  lung  tissue,  as  a  whole,  except 
in  the  neighborhood  of  the  affected  lymphatics,  departs  but  little  from 
the  normal.  As  a  rule,  if  recovery  take  place,  the  affected  tissue  remains 
somewhat  thickened. 

Chronic  Pneumonia. — This  is  characterized  by  the  overgrowth  of 
connective  tissue  in  the  lung,  so  that  it  becomes  hard,  traversed  by 
fibrous  bands,  and  more  or  less  shrunken.  The  condition  leads  to 
destruction  of  the  alveolar  spaces,  sometimes  with  bronchiectasis,  and 
always  to  marked  impairment  of  function.  As  already  mentioned,  it 
may  be  one  of  the  methods  of  termination  of  ordinary  lobar  pneumonia, 
and  may  also  follow  catarrhal,  tuberculous,  and  syphilitic  disease  of 
the  lung,  and  frequently  passive  congestion  or  atelectasis.  This  may  be 
called  (1)  the  secondary  fibroid  or  indurative  pneumonia  (2)  or  it  may  fol- 
low the  inhalation  of  various  kinds  of  dust,  and  is  then  termed  pneu- 
monokoniosis ;  (3)  lastly,  it  may  arise  by  the  extension  of  chronic  pleurisy 
to  the  lung  substance — pleurogenetic  fibroid  pneumonia. 

The  induration  following  upon  ordinary  acute  pneumonia,  when  of 
the  lobar  type,  takes  the  form  of  a  generalized  substitution  of  the  spongy 
tissue  by  compact  fibrous  bands,  while  the  pleura  and  the  interlobular 
septa  are  thickened  as  well.  When  due  to  lobular  pneumonia,  the  fibrous 
tissue  production  follows  the  course  of  the  bronchial  tree  (peribronchial 
fibroid  pneumonia).  The  lung  is  greatly  increased  in  weight,  has  lost 
its  spongy  feel,  and  is  quite  hard.  It  cuts  firmly  and  is  of  a  grayish- 
white  color,  mottled  with  black  from  the  inhaled  coal  dust.  Sometimes 
small  areas  of  necrosis  not  unlike  caseation  may  be  seen.  In  advanced 
cases  the  pleura  is  thickened  and  the  lung  is  greatly  distorted.  The 
two  layers  of  the  pleura  are  usually  matted  together  and  the  tissues  of 
the  mediastinum  may  be  indurated.  Not  infrequently,  contraction  of 
the  fibrous  tissue  leads  to  deformity  of  the  chest,  drooping  of  the  shoulder, 
and  scoliosis.  Microscopically,  the  condition  is  seen  to  be  due  to  an 
imperfect  absorption  of  the  exudate  within  the  alveolar  spaces  and 
the  overgrowth  of  connective  tissue,  so  that  not  only  are  the  septa  of  the 
lung  thickened,  but  the  newly-formed  fibrous  tissue  grows  out  into  the 
alveoli  and  thus  tends  to  obliterate  them.  The  affected  alveoli  are  small, 
20 


306  THE  LUNGS 

collapsed,  or  compressed,  and  often  all  that  can  be  made  out  is  an 
irregular  cavity  containing  a  few  catarrhal  cells  or  a  leukocytic  exudation 
in  various  phases  of  degeneration  and  absorption.  Small  bands  of 
fibrous  tissue  project  into  the  cavity  of  the  alveolus  and  may  even  form 
polypoid  excrescences.  The  bronchi  are  either  compressed  so  that 
they  are  thrown  into  longitudinal  folds,  or  are  rendered  dilated  and 
irregular  from  traction.  Here  and  there  in  the  fibrous  tissue  are  large 
masses  of  inflammatory  leukocytes  and  young  fibroblasts,  pointing  to 
a  continuous  proliferative  process.  Greatly  contracted  alveoli  may  be 
found  almost  devoid  of  lumen  and  lined  by  an  epithelium  which  is 
almost  cubical  in  type. 

FIG.  72 


Chronic  indurative  pneumonia,  showing  great  thickening  of  the  alveolar  walls  and  distortion  of 
the  alveoli.      Zeiss  obj.  DD,  without  ocular.      (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

In  cases  due  to  passive  congestion  the  fibrosis  is  never  so  extreme, 
and  there  is  considerable  deposit  of  blood-pigment  with  dilatation  of  the 
veins,  while  the  alveoli  contain  numerous  catarrhal  cells  filled  with 
pigment.  The  lung  has  a  brownish  color  (brown  induration).  An 
important  variety  is  that  due  to  the  inhalation  of  dust.  But  a  small 
part  of  the  dust  that  we  commonly  inhale  reaches  the  lungs,  and  what 
finally  gets  there  probably  does  not  reach  the  termination  of  the  bronchial 
tree,  but  is  carried  into  the  lung  by  means  of  the  phagocytes.  With 
regard  to  the  events  that  follow,  much  depends  upon  the  nature  of  the 
dust  inhaled.  All  kinds  lead  to  a  certain  amount  of  bronchial  irritation 
and  inflammation,  and  when  carried  along  the  peribronchial  lymphatics 
into  the  substance  of  the  lung,  are  deposited  in  the  alveolar  walls  and 
the  deeper  layers  of  the  pleura,  where  they  lead  to  catarrhal  inflammation 
and  leukocytic  infiltration.  The  more  irritating  kinds  of  dust,  such  as 
stone  or  iron,  may  lead  to  much  more  extensive  lesions.  The  particles, 
being  sharp,  penetrate  the  walls  of  the  bronchioles  and  the  alveoli  and 
lead  to  marked  inflammation  with  proliferation  of  the  connective  tissue. 
Thus,  it  is  common  to  see  in  the  neighborhood  of  the  bronchi  and  in 


TUBERCULOSIS  307 

the  alveolar  walls  more  or  less  rounded  nodules  of  considerable  hard- 
ness that  are  composed  of  connective  tissue  enclosing  a  granular  detritus 
with  clumps  of  pigment.  In  advanced  cases  the  peribronchial  glands 
are  greatly  enlarged  and  full  of  gritty  material.  Occasionally,  the  pig- 
ment may  pass  the  lung  and  be  deposited  in  the  glands  about  the  lesser 
curvature  of  the  stomach  and  the  hilus  of  the  liver,  and  may  even  reach 
the  liver  and  general  circulation.  Among  the  forms  of  dust  that  are 
inhaled  may  be  mentioned  coal  dust,  stone  dust,  kaolin,  iron,  wool,  flour, 
tobacco,  iron  oxide,  and  ultramarine  blue.  When  coal  dust  is  present, 
the  condition  is  called  anthracosis.  This  condition  is  found  in  the  lungs 
of  all  those  past  the  age  of  infancy,  and  is  more  marked  in  those  living 
in  the  cities.  Coal  dust  is  relatively  innocuous,  and  all  we  see  is  scattered 
patches  of  black  pigment  with  comparatively  little  fibrosis.  Sometimes 
small,  rounded  nodules  with  a  black  centre  are  met  with,  the  so-called 
"anthracotic  tubercles."  It  is  the  rule  for  the  peribronchial  glands  to  be 
enlarged  and  contain  coal  pigment.  In  coal  miners  the  lung  is  uniformly 
infiltrated  with  coa1,  is  heavy,  and  has  a  gritty  feel  on  cutting.  In 
such  cases  the  expectoration  may  be  black.  In  cases  of  pneumonokoniosis 
due  to  stone  dust  or  particles  of  steel,  fibrosis  may  be  very  marked. 
In  the  stonemason's  lung  (chalicosis)  the  lung  is  heavy  and  filled  with 
a  grayish,  gritty  material.  Siderosis  is  the  name  applied  to  those  cases 
due  to  the  inhalation  of  iron  or  steel,  such  as  is  seen  among  needle  grinders, 
file  makers,  and  founders.  The  results  of  pneumonokoniosis  are  not 
unlike  those  of  the  secondary  fibroid  pneumonias.  There  is  the  same 
induration  and  deformity  of  the  lung,  together  with  deformity  of  the 
thorax  and  dislocation  of  the  mediastinal  tissues.  Calcification  has  been 
observed,  and  true  bone  formation.  In  rare  cases,  suppuration  and 
cavitation  has  been  met  with.  It  should  be  noted  that  the  presence 
of  dust  within  the  lung  predisposes  to  tuberculosis. 

Pleurogenetic  Fibroid  Pneumonia. — Pleurogenetic  fibroid  pneumonia  is 
due  to  an  extension  of  pleuritic  inflammation,  whereby  both  the  pleura 
and  the  septa  leading  from  it  are  infiltrated  with  inflammatory  products 
and  show  fibrous  hyperplasia.  This  condition  is  also  frequently  com- 
plicated with  collapse  of  the  lung. 

Tuberculosis. — This  disease  may  present  both  acute  and  chronic 
manifestations,  and,  as  its  name  implies,  is  characterized  by  the  forma- 
tion of  tubercles  or  specific  granulomata  in  the  lungs.  The  disease  is 
caused  by  the  B.  tuberculosis,  first  described  by  Koch  in  1881.  This 
microorganism  is  constantly  present,  and  conforms  to  all  the  requirements 
of  Koch's  law  as  to  specificity.  The  search  for  this  bacillus  in  the 
sputum  is  now  one  of  the  routine  practices  of  clinical  research.  Some 
difficulty  has  been  imported  into  the  subject  owing  to  the  discovery  of 
several  forms  of  acid-resisting  bacilli  that  morphologically  and  tincto- 
rially  are  similar  to  the  tubercle  bacillus,  some  of  which  are  met  with 
under  similar  circumstances.  Such  are  the  smegma  bacillus  found  in 
cases  of  gangrene  of  the  lung  by  Pappenheim  and  Fraenkel,1  and  by  one 

1  Berl.  klin.  Woch.,  53:1898:880. 


308  THE  LUNGS 

of  us  (A.  G.  N.),  the  butter  bacillus  of  Moller,  the  timothy-grass  bacillus, 
the  bacillus  of  Lydia  Rabinovitch,  and  certain  streptothrix  forms 
described  by  Flexner.1  Some  of  these  can  produce  granulomata  in  the 
tissues,  so  that  the  resemblance  to  the  true  B.  tuberculosis  is  close.  Care- 
ful culture  experiments  alone  will  differentiate.  We  have  to  recognize 

now  that,  as  there  is  a  colon  group 

FlG-  73  of  bacilli  including  a  great   number 

of  allied  forms,  so  there  is  a  tuber- 
.  culosis  or  acid-fast  group,  containing 

//©//'V    :': *-0       jl  several  forms  differing  in  virulence 

and  in  minor  cultural  peculiarities. 
\,  With  regard  to  the  modes  of  in- 


I:  ;\;)'\ ,          fection,  it  may  be  said  at  once  that 

Ji-v    X/itJ  /"''  ' 


tuberculosis  is  practically  never  in- 
herited.     Some   few    authenticated 
•:;£•  ••  ••:••  ^  cases  are  on  record  of  the  transmission 

'"«i,  'i^^atA-'  of  the  specific  bacillus  from  mother 

to  offspring  through  the  placental 
blood,  but  such  cases  are  so  rare 
that  direct  inheritance  may  be  dis- 
missed as  an  unimportant  factor. 
rodsTaii  else"  is7t*ainedTiueT~  ( Abbott o  *~  At  most,  we  can  say  that  there  is 

an  inheritance  of  the  soil,  in  that  in 

certain  individuals  there  is  a  weak  resisting  power  of  the  tissues  toward 
the  tubercle  bacillus,  so  that  when  infection  takes  place  there  is  growth 
of  the  bacillus  within  the  body  with  all  its  consequences.  The  primes 
vice  of  infection  are  various.  It  used  to  be,  and  in  many  quarters  still  is, 
accepted  by  clinicians  and  pathologists  that  tuberculosis  of  the  lungs 
is  bronchogenic  as  a  rule.  It  should  be  mentioned,  however,  that  by 
certain  observers,  notably  Ribbert,  Aufrecht,  and  Baumgarten, .  a  more 
or  less  successful  attempt  has  been  made  to  disprove  this.  Ribbert,2 
while  not  denying  absolutely  that  infection  may  take  place  by  inhalation, 
believes  that  pulmonary  tuberculosis  is  usually  hematogenic,  in  the 
sense  that  the  peribronchial  glands  are  infected  through  aerial  trans- 
mission by  way  of  the  buccal  mucosa,  and  that  when  they  break  down 
the  products  of  the  destructive  inflammation  are  discharged  into  the 
blood  and  so  reach  the  lung.  Baumgarten3  goes  still  farther,  and  holds 
that  the  glands  also  are  invaded  hematogenously.  The  experiments  of 
Aufrecht4  would  seem  to  prove  that  it  is  impossible  for  bacilli  to  reach 
the  terminal  bronchioles  and  alveoli  through  inhalation,  and  post- 
mortem evidence  also  supports  this  contention.  It  would  seem  probable 
that  we  will  have  to  give  up  the  view  that  the  bronchi  are  the  first  structures 
to  be  attacked,  and  adopt  a  modified  inhalation  theory  somewhat  similar 
to  Ribbert's,  admitting  an  infection  through  the  blood  or  lymph-stream 

1  Johns  Hopkins  Hosp.  Bull.,  8:1897:128.      2  Deut.  med.  Woch.,  1902:  No.  17. 

3  Wien.  med.  Woch.,  November  2,  1901. 

4  Aufrecht,  Pathologic  und  Therapie  der  Lungenschwindsucht,  Wien,  1908. 


TUBERCULOSIS  309 

from  the  mouth  and  nose  and  the  upper  respiratory  passages.  In  this 
connection  Ravenel1  has  shown  recent!}7  that  tuberculous  material, 
when  ingested,  frequently  reaches  the  tonsils  and  peribronchial  glands. 
In  some  cases  the  lungs  have  become  secondarily  affected  from  tuberculous 
disease  of  the  intestinal  tract.  This  is  rare,  at  least  in  this  country, 
although  apparently  more  common  in  Great  Britain.  In  1200  autopsies 
at  the  Royal  Victoria  Hospital,  Montreal,  active  tuberculous  lesions  of 
one  kind  or  another  were  found  295  times;  in  only  three  of  these  was 
the  disease  obviously  primary  in  the  intestine,2  although  this  source  of 
origin  seemed  probable  in  six  more. 

In  rare  instances  the  lungs  have  been  infected  in  cases  of  primary 
tuberculosis  of  the  skin.  The  skin  is,  however,  a  relatively  unfavorable 
medium  for  the  growth  of  the  tubercle  bacillus,  probably  owing  to  local 
temperature  conditions  and  the  nature  of  the  epithelium,  so  that  the 
disease  does  not  often  become  general.  The  bovine  strain  of  the  organ- 
ism, however,  when  invading  the  human  subject  in  this  way,  is  apt  to 
rise  to  widespread  and  virulent  infection. 

After  a  careful  consideration  of  the  methods  of  infection  of  the  lung 
we  are  reduced  to  three:  (1)  the  aerogenic,  (2)  the  hematogenic,  and 
(3)  the  lymphogenic.  Frequently  two  or  even  all  three  methods  may 
be  combined.3 

The  first  method,  if  tuberculosis  of  the  bronchi  be  an  essential  lesion, 
would  appear  to  be  rare,  except  in  those  cases  where  a  lobe  of  the  lung 
is  secondarily  involved  by  the  inhalation  of  infective  excretions  from 
some  other  part  of  the  respiratory  tract.  Such  cases  do  not  represent 
the  frequency  of  infection  through  the  air,  as  it  is  possible  for  bacilli 
to  pass  through  an  intact  bronchial  mucosa  and  lodge  in  the  deeper  struc- 
tures. As  before  hinted,  we  must  distinguish  between  aerogenic  and 
bronchogenic  tuberculosis.  The  disease  usually  arises  from  the  inhala- 
tion of  dried  sputum,  but  also  infective  material  from  tuberculous  cavities 
or  caseous  peribronchial  glands  may  be  aspirated  into  the  air  passages. 

When  the  bacilli  are  inspired  they  become  entangled  in  the  mucus 
at  certain  parts  of  the  bronchial  tree,  where  they  set  up  irritation. 
Judging  from  clinical  experience,  bronchitis  is  a  frequent  result,  but 
postmortem  studies  suggest  that  this  is  simple  and  not  caseous,  for 
primary  caseous  bronchitis  is  rare.  As  a  rule,  the  bacilli  are  picked 
up  by  the  phagocytes  and  carried  through  the  lymphatics  to  the  recesses 
of  the  lung.  Thus,  a  lymphogenic  distribution  of  the  infection  is  quite 
common. 

In  another  set  of  cases  infection  is  through  the  blood,  as,  for  instance, 
when  a  caseous  gland  discharges  its  contents  into  the  pulmonary  artery, 
or  when  the  receptaculum  chyli  or  the  thoracic  duct  are  invaded. 

1  Ravenel,  Amer.  Jour.  Med.  Sci.,  134:1907:469. 

2  Xicholls,  Montreal  Med.  Jour.,  31:1902:327. 

3  For  a  very  good  consideration  of  the  modes  of  infection  in  tuberculosis,  see 
D.  E.  Salmon's  report  on  the  Relation  of  Bovine  Tuberculosis  to  the  Public  Health, 
U.  S.  Dept.  Agric.  Bureau  of  Animal  Industry,  Bull.  No.  33:  1901:  Washington; 
also  A.  D.  Blackader,  Montreal  Med.  Jour.,  30:1901:905. 


310  THE  LUNGS 

Wherever  the  bacilli  become  lodged  is  a  focus  for  the  development  of 
a  tuberculous  lesion,  and  the  number  of  these  will,  of  course,  depend 
very  much  on  the  amount  of  infective  material  reaching  the  lung,  and 
the  character  of  the  phagocytes  and  lymph  currents.  The  anatomical 
picture  frequently  produced  is  that  of  a  localized  bronchopneumonia.  In 
adults  the  site  of  predilection  is  just  below  the  apices,  probably  because 
the  excursion  of  the  lung  is  slighter  at  these  points  and  the  movements 
of  fluids  and  gases  are  consequently  slower.  At  first,  the  patch  of 
infiltration  is  small,  somewhat  gelatinous  in  appearance,  and  imper- 
fectly defined  from  the  healthy  tissue.  At  this  stage  the  vessels  of  the 
alveolar  walls  are  congested  and  a  cellular  exudate  is  thrown  out  into 
the  alveoli.  The  vessels  gradually  become  blocked,  owing  to  prolifera- 
tion of  the  endothelium,  and  the  picture  speedily  changes  as  the  centre 
of  the  area  breaks  down  and  becomes  caseous.  A  sharply  defined  nodule 
thus  results.  This  may  heal,  being  finally  represented  by  a  fibrous 
scar  with  some  puckering  of  the  apex,  or  a  fibrous  nodule  containing 
caseous  or  calcareous  matter.  Should,  however,  the  process  continue, 
as  frequently  happens,  the  bacilli  are  carried  along  the  lymphatics  of 
the  neighboring  septa,  and  secondary  foci  are  the  result.  The  original 
nodule  gradually  increases  in  size  through  continued  infiltration  and 
proliferation,  until  coalescence  takes  place  between  it  and  the  surrounding 
nodules.  The  disease  may  spread  rapidly,  especially  in  children,  until 
the  most  distant  parts  of  the  lung  become  involved.  Where  the  lesions 
reach  the  surface  the  pleura  is  inflamed.  The  peribronchial  glands  are 
liable  to  be  infected  early  in  the  disease.  Quite  frequently  auto-infection 
takes  place  in  cases  where  a  caseous  abscess  discharges  into  a  bronchus 
or  into  a  vessel.  Aspiration  of  infective  sputum  will  bring  about  the 
same  result. 

The  lung-substance  between  the  tubercles  may  show  little  or  no  change, 
but  in  their  immediate  vicinity  there  are  always  congestion,  inflammatory 
infiltration,  and  cell-proliferation.  The  tubercles  are  commonly  sur- 
rounded by  a  zone  of  simple  pneumonia,  which,  in  turn,  rapidly  becomes 
caseous.  In  the  progressive  form  the  original  focus  may  attain  con- 
siderable size,  and,  owing  to  the  interference  with  the  circulation  and  the 
toxic  action  of  the  bacilli,  undergoes  caseation  and  colliquative  necrosis. 
A  ragged  cavity  is  the  result.  Even  in  the  more  chronic  cases,  where 
there  is  much  fibrous  tissue,  this,  in  turn,  may  become  caseous.  The 
cavities  are  usually  circumscribed  and  filled  with  soft,  puriform  material 
containing  granular  and  calcareous  particles  with  shreds  of  tissue.  In 
many  cases  the  cavity  becomes  lined  with  pyogenic  membrane  or  bounded 
by  a  fibrous  wall.  Bands  of  tissue  are  not  infrequently  found  traversing 
these  cavities,  and  represent  the  original  interlobular  septa  of  the  lung. 
In  the  strands,  bloodvessels,  sometimes  showing  aneurismal  dilatations, 
may  be  found,  the  rupture  of  which  leads  at  times  to  fatal  hemorrhage. 
Small  cavities  may  gradually  contract  and  their  contents  become  inspis- 
sated, so  that  a  cheesy  and  calcareous  nodule  surrounded  by  dense  fibrous 
tissue  results.  The  larger  cavities  may  contract,  but  seldom  become 
obliterated.  When  free  communication  exists  between  a  cavity  and  a 


TUBERCULOSIS  3H 

bronchus  it  is  a  common  thing  to  find  a  condition  of  tuberculous  broncho- 
pneumonia  in  the  other  lobes  or  in  the  opposite  lung.  This  is  due  to 
the  aspiration  of  the  infective  products,  a  process  that  is  rendered  easy 
by  cough  or  forced  inspiration,  and  by  bodily  activity.  Here,  in  the 
earlier  stages  of  the  process,  small  isolated  tubercles  form  along  one  or 
more  branches  of  the  bronchial  tree,  which  eventually  coalesce  and  pro- 
duce large  and  perfectly  defined  caseous  masses  surrounded  by  a  zone 
of  simple  pneumonia.  The  course  of  the  disease  depends  on  the  nature 
of  the  infection.  Many  cases  are  examples  of  mixed  infection  with  the 
bacillus  tuberculosis  and  pyogenic  microorganisms.  This  aspect  of 
the  subject  has  received  considerable  attention,  notably  from  Ortner1 
and  Sprengler.2 

FIG.  74 


Caseation  (tuberculous)  in  the  lung.  Area  of  caseation  to  the  right;  the  bloodvessels 
injected  to  show  the  avascularity  of  the  necrotic  part.  Leitz  obj.  No.  3.  (From  Dr.  A.  G. 
Nicholls'  collection.) 

In  a  moderately  severe  form  of  secondary  tuberculous  bronchopneu- 
monia,  a  local  cellular  exudation  and  proliferation  take  place,  followed 
shortly  by  the  formation  of  a  caseous  nodule.  When  such  an  area  is 
examined  microscopically,  one  sees  at  the  periphery  of  the  caseous 
mass  an  exudation  of  fluid  into  the  alveolar  spaces,  diapedesis  of  leuko- 
cytes, swelling  and  desquamation  of  the  lining  epithelium,  and  occa- 
sionally threads  of  fibrin.  The  alveolar  walls  show  round-celled  infiltra- 
tion, and  the  lymphatics,  not  only  the  perivascular  and  peribronchial, 
but  also  the  interalveolar  and  interlobular,  are  more  or  less  blocked  with 

1  Die  Lungentuberculose  als  Mischinfection,  Wien,  1893. 

2  Lungentuberculose  u.  Mischinfection,  Zeitschr.  f.  Hyg.,  18:  1894:  343. 


312 


THE  LUNGS 


inflammatory  products.  As  the  process  extends,  all  the  manifestations 
seen  in  the  original  focus  are  repeated.  The  degree  of  involvement  of 
the  bloodvessels  in  the  neighborhood  of  the  tubercles  is  a  matter  of 
some  importance.  Generally,  owing  to  proliferation  of  the  lining  endo- 
thelium,  they  become  more  or  less  occluded.  Thus,  the  tubercle  is 
avascular.  It  sometimes  happens,  however,  that  erosion  of  the  vessel 

FIG.  75 


Left  lung,  superior  lobe,  and  upper  part  of  lower  lobe,  the  former  containing  a  number  of 
communicating  caverns,  brought  about  by  tuberculous  infiltration,  caseation,  and  evacuation  of 
the  contents  through  the  bronchi.  A,  aneurismal  dilatation  of  an  artery  spanning  one  margin  of  a 
large  cavity;  B,  communication  with  another  cavity;  C,  C,  thickened  and  adherent  pleura 
between  the  two  involved  lobes.  The  pleura  over  both  lobes  is  thickened,  and  at  the  autopsy 
the  cavity  had  been  obliterated  by  universal  adhesion;  D,  the  pointer  from  the  letter  D  leads  to 
a  small  group  of  tubercles  in  which  caseation  is  just  beginning;  E,  a  fused  group  of  tubercles, 
farther  advanced  than  at  D.  (Hare.) 

walls  takes  place  and  hemorrhage  results.  Again,  a  caseous  focus  may 
erode  into  a  large  vessel  and  lead  to  a  miliary  dissemination  of  the 
bacilli  throughout  the  body. 

As  we  meet  with  this  form  of  tuberculosis  post  mortem,  the  lung  is 
more  or  less  adherent  to  the  thoracic  wall,  is  increased  in  weight,  the 


TUBERCULOSIS. 


313 


upper  lobe  largely  caseous  with  multiple  cavities,  while  tuberculous 
nodules  are  scattered  here  and  there  throughout  the  rest  of  the  lung.  The 
bronchi  are  usually  inflamed.  The  cavities  that  are  so  frequently  found 
in  this  form  of  tuberculosis  are  produced  by  the  softening  and  subsequent 
evacuation  of  the  contents  of  a  caseous  focus,  or  at  times  by  the  enlarge- 
ment of  a  bronchiectatic  cavity  through  necrosis  of  its  walls.  Their 
numbers  and  size  vary  considerably.  Sometimes  a  single  cavity,  the 
size  of  a  walnut,  is  found  near  the  apex,  or,  again,  a  whole  lobe  may 
be  converted  into  a  thin  shell.  It  is  not  uncommon  to  find  the  upper 
part  of  the  upper  lobe  riddled  with  cavities  of  varying  size,  communi- 
cating more  or  less  completely  one  with  the  other.  Depending  upon 

FIG.  76 


",•.£*•-•.  i 

$ 


Fibroid  induration  of  the  lung  in  tuberculosis.  The  alveoli  are  destroyed  and  replaced  by 
fibrous  tissue.  Round-celled  infiltration  is  marked  at  the  lower  part.  Zeiss  obj.  DD,  without 
ocular.  (From  the  collection  of  the  Royal  Victoria  Hospital.) 

the  chronicity  of  the  process,  the  walls  are  either  rough  and  shaggy, 
lined  by  a  pyogenic  membrane,  or  smooth  and  fibrous.  The  walls  are 
often  irregular  from  the  presence  of  the  fibrous  septa  of  the  lung,  which 
are  more  resistant  than  the  rest  of  the  lung,  so  that  an  imperfectly  locu- 
lated  cavity  is  the  result.  The  cavities  contain,  besides  air,  a  mixture 
of  serum,  pus  cells,  caseous  matter,  and  detritus,  occasionally  blood. 
One  or  more  of  the  cavities  may  communicate  with  the  bronchi,  which 
show  ulceration  and  other  inflammatory  change.  In  the  older  regions 
of  the  disease  considerable  fibroid  induration  may  be  met  with. 

Besides  the  common  form  of  tuberculous  involvement  just  described, 
which  might  be  correctly  termed  the  caseofibroid  variety,  or  chronic 
ulcerative  tuberculosis,  there  are  several  conditions  arising  in  the  same 


314  THE  LUNGS 

way  that  are  worthy  of  attention.  Not  infrequently,  a  fibroid  or  cal- 
careous focus  in  the  lung,  or  a  partially  healed  cavity,  for  a  long  time 
giving  rise  to  no  symptoms,  will  suddenly  start  into  activity  and  rapidly 
invade  the  rest  of  the  lung.  In  one  form,  minute  miliary  tubercles 
are  produced  along  the  course  of  the  bronchi,  and  a  picture  not  unlike 
the  hematogenic  miliary  tuberculosis  is  the  result.  This  has  been 
termed  disseminated  miliary  tuberculous  bronchopneumonia.  In  other 
cases  the  foci  become  rapidly  larger  and  coalesce,  showing  little  or  no 
tendency  to  fibrosis,  so  that  caseation  and  softening  proceed  apace. 
This  is  the  caseous  nodular  or  lobular  bronchopneumonia.  Again,  a  whole 

FIG.  77 


Tuberculous  pneumonia  (acute  pneumonic  phthisis).      (From  the  Pathological  Laboratory 

of  McGill  University.) 

lobe,  or,  indeed,  a  whole  lung,  may  become  rapidly  and  uniformly  in- 
volved, owing  to  the  coalescence  of  the  various  foci,  and  we  get  the 
caseous  lobar  pneumonia  or  acute  pneumonic  phthisis.  This  is  the  form 
that  has  been  called  by  clinicians  " florid"  phthisis  or  "galloping  con- 
sumption." Here  the  affected  lobe  or  lung  is  uniformly  consolidated, 
very  heavy,  with  a  thickened  and  adherent  pleura.  On  section,  it  is 
dry,  granular,  and  caseous,  and  with  it  may  be  small  areas  of  cavitation 
near  the  apex.  The  condition  is  not  unlike  lobar  pneumonia,  except 
that  the  infiltration  is  denser  and  the  exudate  white  and  caseous,  rather 
than  gray  and  fibrinous. 


HEMATOGENIC  TUBERCULOSIS  315 

Another  type  is  the  chronic  fibroid  tuberculosis,  a  disease  that  often 
lasts  for  years.  Here  tissue  proliferation  is  in  excess  and  leads  to 
induration,  contraction,  and  deformity  of  the  lung.  The  organ  is  trav- 
ersed by  numerous  bands  of  fibrous  tissue,  with  some  caseation  and  old 
contracting  cavities.  The  parenchyma  of  the  lung  is  greatly  damaged, 
being  substituted  in  great  part  by  compact  fibrous  tissue.  In  the 
parts  less  affected  the  alveoli  are  emphysematous.  This  form  may 
affect  the  upper  lobe,  which  may  form  a  very  small  appendage  to  the 
rest  of  the  lung,  and  may  contain  partially  contracted  cavities  and  cal- 
careous nodules.  The  bronchi  are  often  dilated.  The  pleura  is  also 
greatly  thickened  and  is  adherent.  After  a  time,  owing  to  contraction, 
deformity  of  the  chest  wall  sets  in  with,  sometimes,  dislocation  of  the 
heart.  This  form  frequently  arises  from  old  apical  disease,  but  is 
occasionally  pleurogenic. 

FIG.  78 


Miliary  tuberculosis  of  the  lung.      Zeiss  obj.  DD,  without  ocular.      (From  Dr.  A.  G. 
Nicholls'  collection.) 

Hematogenic  Tuberculosis. — Hematogenic  tuberculosis  may  be  part 
and  parcel  of  a  generalized  systemic  dissemination  of  the  infection, 
or  may  be  confined  to  the  lungs  alone.  The  specific  bacilli  are  conveyed 
to  the  lungs  by  the  blood  stream.  The  condition  often  arises  from  the 
discharge  of  a  suppurating  lymph-gland  into  a  vein  or  from  tuberculosis 
in  the  neighborhood  of  the  receptaculum  chyli  and  thoracic  duct.  As 
a  rule,  the  tubercles  are  numerous  but  minute  (miliary  tuberculosis). 

A  lung  affected  in  this  way  is  dark  red  from  hyperemia  and  some- 
what heavier  than  normal.  In  the  earlier  stages,  the  tubercles  can  be 
felt  rather  than  seen  as  minute  shotty  granules.  Later  they  are  noticeable 


316 


THE  LUNGS 


FIG.  79 


as  small  pin-point  dots  of  a  grayish  color,  becoming  in  time  caseous. 
In  the  generalized  systemic  infection  the  tubercles  are  numerous  and 
uniformly  scattered  throughout  the  lungs  and  on  the  pleurae.  In  other 
cases  they  are  confined  to  one  lung  or  to  one  lobe.  They  may  often  be 
seen  at  the  periphery  of  a  large  caseous  focus.  The  lung  is  less  crepitant 
than  normal  and  the  bronchi  are  reddened  and  inflamed. 

Owing  to  the  small  size  of  the  lesions,  they  form  a  convenient  form 
for  the  study  of  the  development  and  minuter  structure  of  the  typical 
tubercle.  The  bacilli  usually  lodge  in  the  capillaries  of  the  alveolar 
walls.  Here  they  become  entangled  in  the  endothelial  cells  lining  the 
vessels,  which  possess  phagocytic  powers,  and  proliferate  there.  The 
irritation  produced  leads  to  inflammatory  hyperemia  and  the  out- 
pouring of  leukocytes,  chiefly  lympho- 
cytes, not  only  into  the  interstices  of 
the  alveolar  septa,  but  also  into  the 
alveolar  spaces.  Accompanying  this 
there  is  cedema,  exudation  of  fluid, 
and  desquamation  and  proliferation 
of  the  cells  lining  the  alveoli.  The 
cells  lining  the  capillaries  also  pro- 
liferate, so  that  sooner  or  later  ob- 
struction of  the  lumina  takes  place. 
As  the  disease  progresses,  another 
type  of  cell  makes  its  appearance, 
somewhat  larger  than  the  leukocyte, 
with  a  single  pale  nucleus — the  so- 
called  'epithelioid"  cell.  The  exact 
origin  of  these  is  not  settled,  but 
they  are  probably  derived  from  the 
proliferation  of  the  fixed  mesoder- 
mic  connective-tissue  elements.  Thus, 
a  typical  tubercle  consists  in  a 

localized  collection  of  lymphocytes  with,  toward  the  centre,  a  num- 
ber of  epithelioid  cells,  accompanied  by  the  usual  signs  of  inflam- 
mation, viz.,  hyperemia,  exudation  of  fluid  sometimes  containing 
fibrin,  and  catarrh  of  the  cells  lining  the  alveoli.  As  the  condition 
progresses,  the  central  portion  of  the  tubercle  breaks  down  or  caseates. 
In  the  more  chronic  forms,  large,  multinucleated  cells — giant  cells — 
make  their  appearance  at  the  periphery.  Any  that  may  have  been 
present  in  the  central  portion  are  usually  destroyed  in  the  caseating 
process.  The  tubercle  increases  by  the  gradual  involvement  of  the 
peripheral  cells  in  the  caseating  process.  It  used  to  be  thought  that 
giant  cells  were  characteristic  of  tuberculosis,  but  we  know  now  that 
they  are  present  in  many  forms  of  chronic  inflammation  and  wherever 
foreign  matter  is  being  absorbed.  In  fact,  there  is  nothing  character- 
istic in  the  structure  of  the  tubercle  as  a  whole,  except  in  the  prepon- 
derance of  lymphocytes,  the  caseation,  and  the  presence  of  the  specific 
bacilli.  Both  plasma  cells  and  "  Mast-zellen"  are  met  with  in  tubercles, 
but  are  equally  non-specific. 


Schematic  representation  of  a  tubercle : 
a,  giant  cell  with  necrotic  centre  and  mul- 
tiple nuclei  peripherally  arranged;  6,  epi- 
thelioid cells;  c,  lymphocytes. 


PSEUDOTUBERCULOSIS  317 

In  the  more  chronic  forms,  or  where  the  resisting  power  of  the  individual 
is  strong,  there  is  a  new  formation  of  connective  tissue  which  tends  to 
wall  in  the  focus.  This  may  be  so  well  marked  that  firm  shotty  masses, 
the  size  of  hemp  seeds,  with  relatively  little  central  caseation  may  be 
produced.  This  is  the  chronic  miliary  tuberculosis,  or  the  chronic  granular 
tuberculosis  of  the  Vienna  school. 

Pseudotuberculosis  (see  also  p.  319). — This  term  has  been  employed 
to  designate  a  condition  closely  resembling  tuberculosis,  but  due  to 
microorganisms  other  than  the  bacillus  of  Koch.  The  lesions  found, 
like  those  of  tuberculosis  proper,  may  be  caseous  granulomata  or 
abscesses.  The  etiology  of  the  condition  is,  curiously,  most  diverse. 
Perhaps  most  of  the  cases  have  been  due  to  bacterial  microorganisms 
allied  to  the  streptothrices  or  to  certain  hypomycetes,  but  some  have 
been  due  to  animal  parasites,  or  even  to  foreign  bodies. 

One  form,  the  pseudotuberculosis  of  rodents  (tuberculose  zoogleique), 
is  found  in  guinea-pigs,  rabbits,  hares,  and  mice,  but  occasionally,  also, 
in  chickens.  An  organism  has  been  isolated,  which  seems  to  belong  to 
the  same  class  as  the  bacillus  of  hemorrhagic  septicemia.  Two  cases 
are  reported  where  this  form  of  the  disease  has  been  transmitted  to  man. 
The  evidence  is,  however,  somewhat  inconclusive.1  Some  few  cases, 
also,  have  been  described  in  which  pseudotuberculosis  in  man,  though 
not  of  the  lungs,  has  been  caused  by  germs  not  identical  with  the  Bacillus 
pseudotuberculosis  rodentium,  but  only  differing  from  it  in  minor  points.2 
Preisz3  and  Kutscher4  have  met  with  cases  due  to  organisms  resembling 
the  bacillus  of  diphtheria.  Eppinger0  has  reported  a  case  of  pseudo- 
tuberculosis  due  to  a  cladothrix,  and  Flexner,6  one  apparently  due  to  a 
form  of  streptothrix. 

An  interesting  form  of  the  affection,  found  in  pigeons  and  transmissible 
to  man,  has  been  described  by  Chantemesse.7  The  organism  at  work 
here  is  the  Aspergillus  fumigatus.  We  have  found  this  also  common  in 
quite  young  chickens.  A  number  of  cases  affecting  the  lung  in  man 
have  been  reported. 

An  organism  producing  a  rare  and  curious  disease  of  the  skin,  found 
in  South  America  and  California,  allied  to  blastomycetic  dermatitis, 
has  been  known  to  invade  the  internal  organs,  including  the  lungs, 
producing  lesions  resembling  those  of  tuberculosis.8 

Among  the  animal  parasites  that  may  cause  pseudotuberculosis, 
we  may  mention  the  eggs  of  the  strongylus,  which  have  been  found  by 
Marsden9  in  the  lungs  of  hogs,  sheep,  and  goats. 

Small,  dead,  foreign  bodies  can  also  produce  nodules  if  they  enter  the 

1  Massa  and  Mensi,  Rev.  Baumg.  Jahresb.  :  1895. 

2  Wrede,  Ziegler's  Beit.,  32: 1902:  526. 

3  Lubarsch  u.  Ostertag  Ergebnisse:  1 :  1896:  733. 

4  Centralbl.  f.  Bact,,  17:  1895:  835. 

5  Ziegler's  Beit.,  9:  1890:  287. 

6  Jour,  of  Exper.  Med.,  3:  1898:  435. 

7  Rev.  Centralbl.  f.  Path.,  1:  1890:  581. 

8  Ophiils  and  Moffitt,  Phila.  Med.  Jour.,  5:  1900:  1471. 

9  Munch,  med.  Woch.,  35:  1898:  1100. 


318  THE  LUNGS 

lung,  as  has  been  shown  by  Cruveilhier1  and  Waldenburg.2  Tubercles 
of  this  order,  due  to  coal  dust,  are  quite  common. 

Syphilis. — Syphilis  of  the  lung,  apart  from  the  congenital  form, 
is  rare,  and  does  not  always  manifest  itself  in  a  characteristic  way. 
It  is  at  times  impossible  to  differentiate  it  from  non-specific  forms  of 
inflammation.  No  doubt,  many  of  the  cases  of  bronchitis  and  pneu- 
monia attributed  to  syphilis  are  more  correctly  to  be  regarded  as  com- 
plications or  examples  of  mixed  infection.  Syphilis  is  met  with  in  the 
form  of  £ummas  and  diffuse  interstitial  fibrosis. 

Gummas  are  rare  in  acquired  syphilis  and  are  not  common  even  in  the 
lungs  of  newborn  syphilitic  children.  The  gummas,  which  are  usually 
quite  numerous,  when  present,  are  in  the  earlier  stages  grayish-red  or 
grayish-white,  somewhat  translucent,  and  form  nodules  of  all  sizes  up 
to  that  of  a  hen's  egg,  surrounded  by  an  area  of  congestion.  Later, 
they  undergo  a  process  allied  to  caseation  and  become  opaque  white 
and  more  or  less  walled  off  by  connective  tissue.  The  contents  of  the 
granuloma  may  liquefy  and  be  discharged  into  a  bronchus,  or  again 
may  become  inspissated  and  calcareous.  Healed  gummas  are  to  be 
distinguished  from  old  tubercles  and  abscesses  only  with  the  greatest 
difficulty.  Gummas  are  more  common  near  the  hilus  of  the  lung  than 
elsewhere.  Microscopically,  they  only  can  be  distinguished  from 
tubercles  by  the  absence  of  the  tubercle  bacilli. 

In  the  second  type  the  lung  becomes  the  site  of  a  more  or  less  dif- 
fused and  extensive  cellular  infiltration,  together  with  hyperplasia  of  the 
connective  tissue  and  proliferation  and  desquamation  of  the  alveolar 
epithelium.  To  any  one  who  has  seen  the  extraordinary  number  of 
spirochsetes  present  in  these  cases,  this  marked  inflammation  is  not 
surprising.  The  alveolar  walls  become  greatly  thickened,  and  large 
areas  are  converted  into  a  somewhat  fibrous  mass,  in  which  compressed 
air  spaces  and  groups  of  cells,  representing  the  proliferated  and  des- 
quamated epithelium,  can  be  recognized.  The  bloodvessels  are  thick- 
ened so  that  the  lung  is  pale  and  anemic,  an  appearance  that  has  given 
to  the  condition  the  name  of  "white  pneumonia."  Diffuse  pneumonia 
of  this  type  may  be  combined  with  gummas.  Virchow,  followed  by 
Pankritius,3  has  described  a  form  of  induration  starting  from  the  hilus 
of  the  lung  as  of  syphilitic  origin.  Others  mention  a  form  starting  from 
the  pleura  and  interlobular  septa.  Renter4  has  demonstrated  the 
presence  of  the  Spirochseta  pallida  in  the  lung  of  a  child  dying  of 
hereditary  syphilis  with  "  white"  pneumonia. 

Actinomycosis. — This  disease  may  affect  the  lung  primarily,  but  it 
is  more  common  for  it  to  originate  in  actinomycosis  of  the  mouth, 
pharynx,  or  oesophagus,  by  inhalation  or  by  extension  from  the  anterior 
mediastinum.  Occasionally  the  disease  is  metastatic,  as  in  a  case  we 
saw  in  which  the  primary  lesion  was  in  the  liver. 

1  Trait^  d'Anat.  path,  gen.,  4:  1862. 

2  Tuberculosis,  Pulmonary  Phthisis,  and  Scrofulosis,  Berlin,  1869. 

3  Ueber  Lungensyphilis,  Berlin,  1881. 

4  Zeit.  f.  Hyg.  u.  Infect.,  54;  1906:  49. 


PARASITES  319 

Multiple  nodules  of  a  miliary  type  are  met  with,  or  there  may  be 
large  areas  of  infiltration  formed  by  the  fusion  of  several  granulomas. 
Not  infrequently,  the  affection  conforms  to  the  type  of  a  bronchopneu- 
monia.  Clinically,  the  disease  may  resemble  bronchitis,  there  being 
a  discharge  of  fetid  muco-pus,  containing  at  times  the  characteristic 
"sulphur  grains."  Under  the  microscope  these  are  found  to  be  the 
specific  ray-fungus.  In  more  chronic  cases,  signs  of  consolidation  and 
cavitation  of  the  lung  may  be  met  with,  so  that  the  disease  is  not  unlike 
chronic  tuberculosis. 

In  the  metastatic  form,  the  lungs  are  riddled  with  small  abscesses 
containing  creamy  pus  and  surrounded  by  an  inflammatory  areola. 
When  young,  the  granulomas  are  grayish  or  grayish-red  in  color  and 
surrounded  by  a  pneumonic  zone,  or  what  amounts  to  a  vascular  granu- 
lation tissue. 

In  long-standing  cases  the  nodules  are  more  or  less  completely 
walled  off  by  fibrous  tissue,  the  interlobular  septa  are  thickened,  and 
there  are  all  the  signs  of  a  diffuse  fibrous  hyperplasia  with  alveolar 
catarrh  and  inflammatory  exudation.  The  lung  may  eventually  be 
converted  into  a  contracted  nodular  mass  full  of  cavities  and  riddled 
with  sinuses.  The  disease  occasionally  spreads  through  the  thoracic 
wall  and  invades  the  pectoral  muscles,  or,  again,  may  spread  through  the 
diaphragm  to  the  abdominal  viscera.  The  mediastinum  and  pericardium 
are  liable  to  be  involved. 

Glanders. — This  is  rare  in  man,  and  is  almost  invariably  contracted 
from  animals  suffering  from  the  disease.  The  affection  takes  the  form 
of  multiple  cellular  nodules  of  a  grayish  or  yellowish-white  color,  varying 
in  size  from  a  millet-seed  to  a  pea.  In  other  cases  there  is  lobar  or 
lobular  pneumonia,  or  a  diffuse  purulent  infiltration,  with  abscess  forma- 
tion. The  affection  can  only  be  recognized  by  the  presence  of  the  mallein 
reaction  during  life,  or  the  detection  of  the  B.  mallei  in  the  excretion. 

Parasites. — Apart  from  the  bacterial  forms  already  mentioned, 
such  as  the  B.  tuberculosis,  Diplococcus  pneumonise,  pyogenic  cocci, 
B.  mallei,  B.  anthracis,  and  the  Actinomyces,  vegetable  parasites  are 
rare  and  few  of  them  are  of  importance. 

Various  forms  of  moulds,  such  as  aspergilli  and  the  thrush-fungus, 
have  been  described.  They  are  liable  to  be  found  wherever  destruc- 
tion of  the  lung  substance  is  going  on  or  where  there  is  stagnation  and 
decomposition  of  secretion.  Some  are  accidental,  while  others  must  be 
regarded  as  pathogenic.  A  Pneumonomycosis  aspergillina,  as  it  has  been 
termed  by  Saxer,1  has  been  described  by  Virchow,  Dieulafoy,  Chante- 
messe  and  Witlal,  and  later  by  Renon.2  Cases  also  have  been  reported 
in  England  by  Boyce,  Arkel,  and  Hinds,  and  by  Pearson  and  Ravenel3 
in  America.4 

1  Pneumonomycosis  Aspergillina,  Jena,  1900. 

2  Etude  sur  1'aspergillose  chez  les  animaux  et  chez  1'homme,  1897. 

3  Proc.  Path.  Soc.  Phila.,  new  series:  111:  1900:  10. 

^<-e  also  Rolleston,  Article  on  Pulmonary  Aspergillosis,  Allbutt  and  Rolleston's 
System  of  Medicine,  5:  1909:  440. 


320  THE  LUNGS 

The  disease  is  met  with  in  birds,  horses,  and  cattle,  but  occasionally 
attacks  man.  The  lesions  produced  resemble  those  of  tuberculosis  (see 
above). 

Of  the  animal  parasites,  the  most  important  is  the  Echinococcus. 
Echinococcus  disease  may  be  primary  or  secondary.  The  lung  may  be 
invaded  from  the  liver.  Rarely,  infection  takes  place  through  the 
hepatic  vein,  the  inferior  vena  cava,  and  the  right  heart.  The  cyst  may  be 
single  or  multiple,  and  may  reach  the  size  of  a  man's  head.  The  cavities 
are  filled  with  clear  fluid  containing  the  characteristic  hooklets,  or 
again  may  suppurate.  When  healing  takes  place,  the  fluid  is  to  some 
extent  absorbed,  and  calcareous  deposit  may  take  place.  Occasionally 
the  cysts  may  rupture  into  a  bronchus,  the  pleural  cavity,  or  the  abdomen. 
Dislocation  of  the  neighboring  organs  is  likely  to  occur. 

The  Cysticercus  celluloses  is  rare.  The  Strongylus  longivaginatus , 
Monas  lens,  Cercomonas,  coccidia,  and  psorosperms  have  been  met  with. 

A  rare  but  important  affection  is  that  called  by  Stiles1  "  Paragoni- 
miasis."  This  is  due  to  a  trematode  worm,  the  "lung  fluke"  or  Para- 
gonimus  Westermannii.  It  is  most  common  in  Asia  and  Africa, 
although  some  few  cases  have  been  met  with  in  America.  The  disease 
affects  the  tiger,  cat,  dog,  and  swine,  and  sixty-six  cases  in  man  have  been 
collected  by  Stiles.  The  infection  is  probably  through  drinking-water. 

The  most  striking  symptom  is  hemoptysis.  The  parasites,  which  look 
not  unlike  small  almonds,  fix  themselves  by  their  suckers  to  the  mucosa 
of  the  bronchi  and  burrow  their  way  through  the  lung,  so  that  a  series 
of  intercommunicating  cavities  are  produced.  These  may  communi- 
cate with  a  bronchus.  The  bronchi  are  much  inflamed  and  the  lung 
tissue  near  the  cavities  is  much  congested.  The  cavities  contain  broken- 
down  lung  tissue,  ova,  hematoidin,  and  the  parasites.  The  disease  tends 
to  run  a  chronic  course.  Recovery  is  the  rule.2 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy  of  the  Lungs. — This  is  a  comparatively  unimportant 
condition. 

The  form  known  as  senile  atrophy,  sometimes  called  "atrophic  emphy- 
sema," has  already  been  referred  to.  It  is  to  be  regarded  as  a  physio- 
logical involution  rather  than  a  pathological  process.  Emphysema 
may,  however,  be  associated  with  it,  and  is  generally  due  to  a  concomitant 
chronic  bronchitis  which  is  found  in  so  many  old  persons. 

Besides  this,  a  certain  amount  of  local  atrophy  is  found  in  a  variety 
of  conditions,  such  as  emphysema,  atelectasis,  and  indurative  pneumonia. 

Rokitansky3  also  recognized  a  form  of  atrophy  due  to  inactivity  of  the 
lung  in  cases  where  the  pleura  presents  marked  thickening. 

1  Proc.  Path.  Soc.  Phila.,  February,  1901. 

2  For  details  of  pathological  anatomy,  see  Katsurada,  Ziegler's  Beitr.,  28: 1900:  506. 
3Lehrb.,  3:  1861:47. 


PROGRESSIVE  METAMORPHOSES  321 

Degenerations. — Fatty  Degeneration. — This  affects  chiefly  the  alveo- 
lar endothelium  and  the  walls  of  the  bloodvessels.  It  is  found  in  poison- 
ing by  arsenic  and  phosphorus,  and  is  met  with  as  a  secondary  mani- 
festation in  a  great  variety  of  inflammations  and  new-growths.  The 
change  is  best  seen  in  the  epithelial  cells  lining  the  air  spaces,  which  are 
swollen  and  desquamated. 

Hyaline  Degeneration. — This  also  affects  the  alveolar  epithelium 
and  the  walls  of  the  bloodvessels.  It  is  found  chiefly  in  tuberculous 
and  syphilitic  affections  of  the  lung.  The  so-called  corpora  amylacea 
have  been  referred  to  elsewhere  (vol.  i,  p.  848). 

Amyloid  Disease. — This  is  singularly  rare  in  the  lungs.  Only  in  the 
most  extensive  condition  of  disseminated  amyloid  disease  do  we  find 
the  vessels  of  the  lungs  affected.  Occasionally,  in  cases  of  syphilis, 
with  induration  of  the  lungs  and  marked  amyloid  degeneration  of  the 
organs,  the  condition  is  met  with. 

Calcareous  Degeneration. — This  is  seen  usually  in  the  form  of  concre- 
tions, old  tuberculous  foci,  and  in  tumors.  A  rare  condition  is  a  deposit 
of  lime  salts  in  the  alveolar  walls,  bloodvessels,  or  septa  of  the  lung. 
This  has  been  attributed  to  a  lime  metastasis,  where  the  blood  is  loaded 
with  salts  derived  from  the  skeleton  (see  vol.  i,  p.  852). 

Pneumonomalacia. — Apart  from  that  form  of  gangrene  of  the  lungs 
due  to  inflammation  or  to  the  germs  of  putrefaction,  there  is  a  form 
analogous  to  myomalacia  of  the  heart  and  encephalomalacia.  Small 
areas  are  seen  which  are  softened,  shaggy,  and  necrotic  looking.  The 
patches  have  a  reddish-brown  color  and  are  devoid  of  any  putrid  odor. 
The  condition  seems  to  be  a  simple  necrosis.  It  is,  of  course,  rare,  as 
will  readily  be  understood  when  we  consider  how  easy  it  is  for  any  dis- 
eased portion  of  the  lung  to  become  infected  by  germs.  Some  cases  are 
due  to  pulmonary  embolism;  others  are  met  with  in  diabetes. 

PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — We  must  be  careful  not  to  conclude  that  because 
a  lung  is  enlarged  it  is  hypertrophic,  for,  unlike  other  organs  which 
belong  to  the  group  of  epithelial  glands,  the  lungs  do  not  tend  to  undergo 
hypertrophy.  Owing  to  its  anatomical  structure,  when  from  any  cause 
a  portion  of  the  lung  is  rendered  functionless,  the  rest  becomes  enlarged, 
it  is  true,  yet  not  from  hypertrophy  but  from  emphysematous  dilatation 
of  the  air  spaces.  Still  the  lung  does  seem  to  have  a  certain  amount  of 
regenerative  power,  for  cases  are  on  record  where,  in  the  event  of  com- 
plete atrophy  of  one  lung,  the  other  enlarged  so  much  as  to  fill  not  only 
one  pleural  cavity  but  to  encroach  upon  the  other,  and  this  in  the  absence 
of  sufficient  emphysema  to  account  for  the  enlargement. 

Besides  this  form,  which  is  rare,  hypertrophy  of  the  muscle  fibers 
about  the  smaller  bronchioles  has  been  observed  in  cases  of  brown 
induration  of  the  lungs.  This  is  probably  to  be  attributed  to  the  catarrh 
of  the  bronchi,  which  is  usually  present  in  such  cases  and  should  be 
classed  with  the  hvpertrophies  due  to  increased  work. 
21 


322  THE  LUNGS 

Tumors. — Primary  tumors  of  the  lung  are  comparatively  rare. 
Among  the  benign  growths,  fibromas,  lipomas,  chondromas,  and  osteomas 
have  been  observed.  Not  all  the  cases  reported  as  "osteomas/'  however, 
are  to  be  regarded  as  true  tumors.  Many  are  examples  of  ossification 
developing  in  hyperplastic  connective  tissue  arising  from  chronic  inflam- 
mation (metaplasia).  Ribbert  has  described  ossification  in  caseous 
areas,  and  we  have  met  with  the  same  condition  ourselves.  Care  should 
also  be  taken  not  to  confuse  the  calcification  that  takes  place  in  old  tuber- 
culous and  other  inflammatory  foci  with  tumor  formation.  Chondroma 
is  also  rare  and  arises  from  the  bronchial  cartilages,  and,  possibly, 
from  embryonic  "rests." 

Dermoids. — Dermoids  are  occasionally  met  with.  Albers  has  described 
a  case  in  which  there  was  a  cystic  tumor  communicating  with  a  bronchus. 
For  years  hairs  were  discharged  in  the  sputum. 

Adenoma. — Adenomas  derived  from  the  peribronchial  mucous  glands 
have  been  described  by  Chiari,1  but  are  very  rare. 

Sarcoma. — Primary  sarcoma  is  also  rare.  Ranglaret2  has  recorded  a 
case  of  spindle-celled  sarcoma  of  the  left  lung,  and  Reymond3  one  of  the 
round-celled  variety.  Lymphosarcoma  is  not  so  uncommon.  Less  fre- 
quent are  the  cases  that  arise  in  the  mediastinal  tissues  and  the  lymph- 
glands  at  the  root  of  the  lung.  It  is  interesting  in  this  connection  to 
note  that  chronic  irritation  from  certain  kinds  of  dust  seems  to  be  an 
exciting  cause,  for  Ancke4  has  pointed  out  how  frequent  sarcoma  of 
the  lung  is  among  the  miners  of  the  Schneeberg  district.  Very  interest- 
ing and  important  are  those  new-growths — the  endotheliomas — that  are 
intermediate  in  structure  between  the  sarcomas  and  the  carcinomas. 
They  develop  superficially  beneath  the  pleura,  or  at  the  hilus  of  the  lung 
in  the  lymph-channels.  The  aberrant  growth,  however,  soon  passes 
beyond  the  lymphatic  vessels  and  invades  the  adjacent  tissues,  forming 
larger  or  smaller  nodules,  arranged,  in  some  cases,  about  the  bronchial 
tree  like  a  string  of  beads. 

A  very  rare  form  of  primary  growth  is  the  simple  melanotic  tumor 
(tumeur  melanique  simple)  described  by  Cornil  and  Ranvier,5  which 
may  take  on  malignant  action. 

Carcinoma. — Primary  carcinoma  of  the  lung  generally  affects  by  prefer- 
ence the  right  side,  and  is  either  nodular  or  diffuse.  There  are  three 
main  types,  in  which  the  new-growth  starts  from  the  bronchi,  the  alveolar 
epithelium,  or  the  peribronchial  mucous  glands.  The  first  form  is 
composed  of  columnar  cells,  while  the  second  is  made  up  of  flattened 
plates  in  which  cell-nests  may  sometimes  be  seen.  It  is  characteristic 
of  carcinomas  in  this  situation  that  they  readily  soften  or  become  hemor- 
rhagic,  so  that  they  are  not  unlike  caseous  tuberculous  masses.  In  such 

1  Verschiedene  Tumoren,  Prager  med.  Woch.,  1883. 

2  Bull,  de  la  soc.  anat.  de  Paris,  Ser.  V:  Tom.  VII:  Fasc.  22:  p.  591. 

3  Ibid.,  p.  256. 

4  Dissert.  Munch.,  1884. 

5  Tumeurs  melaniques  simples,  Manuel  d'histol.  path.,  2:  1882:  140. 


CARCINOMA  323 

cases,  when  the  contents  are  discharged  through  a  bronchus,  cavities 
are  the  result.     Metastases  appear  to  be  relatively  infrequent. 

Secondary  growths  are  much  more  common,  and  are  generally  due  to 
malignant  emboli  that  get  into  the  venous  circulation,  an  event  that 
easily  occurs.  The  first  to  be  mentioned  is  the  chondroma,  which, 
when  found  in  the  lungs,  is  nearly  always  secondary.  Osteoid  chondromas 
myxomas,  lipomyxomas,  have  also  been  met  with.  Besides  these  are 
the  sarcomas,  including  pigmented  forms,  and  all  varieties  of  carcinomas. 
In  the  case  of  the  latter  the  primary  seat  is  nearly  always  in  the  stomach 
or  mamma.  An  interesting  and  not  uncommon  form,  in  our  experience, 
of  secondary  carcinoma  is  that  in  which  small  cancerous  nodules  are 
formed  along  the  course  of  the  pleural  lymphatics,  so  that  a  distinct 
network  is  produced. 


CHAPTER    XIV. 

THE  PLEURA. 

THE  pleurae  are  sacs  composed  of  a  thin,  rather  loose,  connective- 
tissue  membrane,  containing  numerous  bloodvessels  and  elastic  fibrillae. 
They  are  covered  by  a  single  layer  of  flattened  mononuclear  cells — the 
endothelium.  The  pleurae  are  not  very  liable  to  be  affected  by  primary 
disease,  but  owing  to  their  close  association  with  the  lungs  are  fre- 
quently involved  by  contiguity  or  extension.  Not  only  so,  but  inasmuch 
as  the  pleural  sacs  are  lymph-spaces  having  communication  more  or 
less  closely  with  the  pericardial  and  peritoneal  cavities,  inflammatory 
processes  originating  in  either  of  these  regions  readily  extend  to  the 
pleurae.  Disease,  therefore,  of  the  lungs,  peribronchial  and  mediastinal 
glands,  oesophagus,  aorta,  thoracic  duct,  stomach,  liver,  and  thoracic 
wall  may  rapidly  involve  the  pleurae.  Conversely,  lesions  of  the  pleurae 
may  extend  to  the  contiguous  parts.  In  the  dissemination  of  disease 
the  movements  of  the  membrane  incident  to  respiration  play  an  important 
part. 

ANOMALIES  OF  DEVELOPMENT. 

Perhaps  the  most  common  anomaly  is  an  infolding  of  the  membrane 
at  the  upper  part  of  the  cavity  associated  with  the  apical  fissure  of  the 
lung  before  referred  to.  Along  the  free  border  an  abnormal  azygos  vein 
frequently  runs.  Partial  defects  of  the  pleurae  are  found  associated  with 
congenital  diaphragmatic  hernia.  In  monstrous  births  the  pleura  may 
be  absent  altogether  or  redundant.  In  the  rare  congenital  atrophy  of 
the  lung,  the  pleural  cavity  may  be  filled  with  fat  mixed  with  a  mucoid 
connective-tissue  substance. 


CIRCULATORY  DISTURBANCES. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  occurs  as  an 
accompaniment  of  congestion  of  the  lung  proper,  and  also  from  a 
sudden  relaxation  of  tension,  such  as  takes  place  during  the  operation 
of  thoracentesis.  In  the  latter  event  the  vessels  not  infrequently  give 
way  and  hemorrhage  results. 

Passive  Hyperemia. — Passive  hyperemia  is  found  in  all  forms  of  ob- 
struction to  the  greater  or  lesser  circulation. 

Hemorrhages. — Multiple  hemorrhages,  ecchymoses,  and  petechite 
are  common.  They  are  found  especially  in  cases  where  there  has  been 


PNEVMOTHORAX  325 

marked  interference  with  respiration,  as  in  death  by  suffocation.  Sim- 
ilar manifestations  are  met  with  in  kidney  and  heart  affections,  in  the 
infections  and  intoxications,  nervous  diseases,  and  in  the  hemorrhagic 
diatheses. 

(Edema. — (Edema  of  the  pleura  is  indistinguishably  associated  with 
oedema  of  the  lungs. 


ABNORMAL    STATES    OF    THE    PLEURAL    CAVITIES. 

Hematothorax. — When  blood  is  effused  into  the  pleural  cavity,  the 
condition  is  called  hematothorax.  It  is  somewhat  rare  for  the  sac  to  con- 
tain pure  blood,  for  the  fluid  is  generally  mixed  with  transudation  or  the 
products  of  inflammation.  The  affection  is  brought  about  by  rupture 
of  the  vessels,  and  is  not  infrequently  met  with  in  rupture  of  tuberculous 
and  gangrenous  cavities,  in  certain  forms  of  inflammation,  and  in  carci- 
noma. Other  causes  that  should  be  mentioned  are  the  bursting  of  an 
aneurism  into  the  cavity,  fracture  or  caries  of  the  ribs,  and  stab  wounds 
of  the  chest. 

Hydrothorax. — Hydrothorax  is  a  collection  of  transuded  fluid  in  the 
pleural  cavity.  This  occurs  by  preference  on  the  right  side,  although  it 
may  be  bilateral.  The  reason  for  this  is  said  to  be  that  most  people 
lie  upon  the  right  side,  or  else  that  there  is  pressure  of  a  distended  right 
heart  on  the  veins  of  that  side.  When  pleural  adhesions  are  present, 
the  effusion  may  be  sacculated. 

The  fluid  is  usually  pale,  straw-colored,  alkaline,  the  specific  gravity 
varying  between  1009  and  1012,  or  a  little  higher,  and  contains  no  flakes. 
The  amount  of  albumin  varies  from  2.78  per  cent.  (Hoppe-Seyler)  to 
4.97  per  cent.  (Scherer).  Microscopically,  there  are  a  few  leukocytes 
and  desquamated  cells  from  the  pleural  endothelium.  The  pleural 
surface  is  still  smooth,  but  may  be  slightly  sodden,  and  in  long-standing 
cases  the  membrane  becomes  turbid,  pearly,  and  somewhat  thickened, 
owing  to  overgrowth  of  the  connective  tissue. 

Small  quantities  of  fluid  may  be  poured  out  into  the  cavity  during 
the  death  agony,  but  the  larger  collections  are  found  in  nephritis,  un- 
compensated  heart  lesions,  nodular  cirrhosis  of  the  liver,  hydremia,  and 
poisoning  with  carbon  monoxide. 

Chylous  and  Pseudochylous  Hydrothorax. — Chylous  and  pseudo- 
chylous  hydrothorax  are  due  to  rupture  or  obstruction  respectively 
of  the  thoracic  duct  above  its  point  of  entrance  into  the  pleural  cavity. 
The  fluid  is  opaque,  milk-white,  and  contains  granules  and  lymph 
cells.  In  chylous  fluid  fat  globules  are  also  present. 

Small  collections  of  fluid  in  the  thorax  are  of  no  significance,  but  the 
larger  ones  lead  to  compression  and  collapse  of  the  lung  and  disloca- 
tion of  the  neighboring  structures,  such  as  the  heart  and  diaphragm. 

Pneumothorax. — When  the  pleural  cavity  contains  air,  the  condition 
is  termed  pneumothorax.  Owing  to  the  nature  of  the  exciting  causes,  the 
affection  is  accompanied,  in  the  vast  majority  of  cases,  by  inflammation, 


326  THE  PLEURA 

so  that  the  cavity  contains  serum  or  pus  as  well  as  air  (hydropneumo- 
thorax,  pyopneumothorax).  Pneumothorax  is  rare  as  a  primary  dis- 
ease, and  is  usually  due  to  a  lesion  of  the  lung  or  pleura.  The  most 
frequent  cause  is  the  rupture  of  a  tuberculous  focus  in  the  lung  during 
cough  or  other  strain.  Occasionally,  it  follows  the  rupture  of  a  gan- 
grenous or  suppurating  area.  It  is  said  that  it  may  also  be  caused  by 
the  giving  way  of  an  emphysematous  bulla.  It  may  be  regarded  as 
certain  that  it  never  occurs  from  the  rupture  of  a  healthy  lung.  Air  may 
also  enter  the  pleura  as  the  result  of  stab  wounds  of  the  chest,  fractured 
ribs,  thoracentesis,  or  from  the  stomach,  oesophagus,  and  bowel.  In  the 
last  case  the  usual  condition  found  is  a  malignant  growth  in  the  viscus 
which  attaches  it  to  the  diaphragm.  An  empyema  may  also  erode  its 
way  into  the  lung  and  discharge  into  a  bronchus. 

Pneumothorax  due  to  perforation  has  been  divided  by  Weil  into  three 
varieties:  (1)  Open  pneumothorax,  in  which  air  passes  freely  in  and  out. 
(2)  Ventilated  pneumothorax,  in  which  there  is  an  oblique  or  valve-like 
opening,  so  that  air  enters  readily  but  cannot  escape.  (3)  Closed 
pneumothorax,  where  the  opening  has  become  occluded.  In  many  cases 
the  fistula  becomes  closed,  and  at  autopsy  it  is  frequently  impossible  to 
find  the  point  of  rupture. 

Laennec  was  perhaps  the  first  to  describe  pneumothorax  without 
perforation.  Although  for  some  time  doubted,  it  is  now  abundantly 
demonstrated  that  there  is  such  a  thing  as  non-perforative  or  essential 
pneumothorax,  caused  by  the  growth  of  gas-producing  microorganisms 
in  the  pleura.  Cases  due  to  the  B.  coli  have  been  recorded  by  R.  May 
and  Adolf  Gebhart,1  and  in  other  cases  the  B.  Welchii  has  been  found.2 

The  result  of  pneumothorax  will  depend  very  much  on  the  cause  and 
on  the  persistence  or  otherwise  of  the  communication  with  the  outer 
air.  So  long  as  the  fistula  remains,  the  lung  is  completely  collapsed 
unless  this  result  be  prevented  by  the  presence  of  adhesions.  In  cases 
where  a  valvular  opening  is  present,  the  pleural  cavity  becomes  gradually 
inflated  with  air,  so  that  the  lung  is  compressed,  the  heart  pushed  over 
to  the  opposite  side,  the  diaphragm  depressed,  and  the  whole  side  of 
the  thorax  distended.  In  cases  of  pneumothorax  without  infection,  the 
wound  may  close  and  the  air  is  then  gradually  absorbed,  so  that  the 
lung  resumes  its  normal  condition.  A  false  pneumothorax  is  met  with 
post  mortem  owing  to  self-digestion  of  the  walls  of  the  stomach  and  the 
diaphragm  and  a  consequent  discharge  of  gas  into  the  pleural  cavity. 
Cases  with  serofibrinous  inflammation  or  empyema,  provided  they  are 
cured  at  all,  generally  leave  traces  in  the  shape  of  pleural  thickening  and 
adhesions. 

INFLAMMATIONS. 

Pleurisy. — Inflammation  of  the  pleura  (pleurisy  or  pleuritis)  is  only 
occasionally  a  primary  infection,  and  usually  originates  in  disease  of 

1  Deuts.  Archiv  f.  klin.  Med.,  61  :  1898  :  323. 

2  Nicholls,  Brit.  Med.  Jour.,  2  : 1897  : 1844. 


EXUDATIVE  PLEURISY 


327 


the  lung  or  the  neighboring  cavities.  The  type  of  disease  varies  con- 
siderably according  to  the  nature  of  the  infecting  agents.  These  are 
almost  invariably  bacterial,  since  the  lung,  owing  to  its  association  with 
the  outer  air,  always  contains  bacteria.  Pleurisy  may  be  partial  or 
complete,  the  exudate  free  or  sacculated.  Frequently,  pleurisy,  par- 
ticularly when  left-sided,  is  combined  with  pericarditis.  It  is  not  un- 
common also  for  several  serous  membranes  to  be  progressively  involved, 
a  condition  for  which  certain  Italian  observers  have  proposed  the  term 
"polyorrhomenitis."  According  to  Taylor,1  the  course  of  involvement 
of  the  various  serosse  is  as  follows:  (1)  Peritoneum  involved  first  with 
extension  to  the  pleura,  usually  the  right;  (2)  the  pleura,  then  the  peri- 
toneum; (3)  the  pleura  of  one  side  and  then  of  the  other;  and  (4)  one 
pleura,  then  the  peritoneum,  and  finally  the  other  pleura.  According 
to  the  course  of  the  inflammation,  we  may  recognize  exudative  and  pro- 
ductive pleurisy.  These  forms  may  exist  independently,  but  it  is  not 
uncommon  for  exudative  pleurisy  to  develop  into  the  productive  variety. 

Exudative  Pleurisy. — Exudative  pleurisy  is  met  with  occasionally  in 
persons  of  low  vitality  or  as  a  manifestation  of  the  rheumatic  infection. 
It  is  very  common  in  pneumonia,  tuberculosis,  infarction,  and  in  new- 
growths. 

FIG.  80 


Acute  serofibrinous  pleurisy.  Zeiss  obj.  DD,  ocular  No.  1.  Long  strands  of  fibrin,  between 
which  inflammatory  leukocytes  are  enmeshed,  are  well  seen  on  the  surface  of  the  lung.  (From 
the  Pathological  Laboratory  of  McGill  University.) 

The  exudation  may  be  fibrinous  (plastic),  serofibrinous,  fibrino- 
purulent,  purulent,  or  hemorrhagic. 

1  British  Medical  Journal,  2  :  1900  :  1693. 


328  THE  PLEURM 

Fibrinous  Pleurisy. — In  the  fibrinous  form,  or  what  is  sometimes  called 
"dry"  pleurisy,  the  pleura  is  opaque,  slightly  turbid,  and  upon  it  is  a 
delicate  layer  of  fibrin  which  can  readily  be  scraped  oft'.  Microscopically, 
the  pleura  is  oedematous ;  the  vessels  of  the  pleura,  both  blood  and  lym- 
phatic, and  the  subjacent  layer  of  the  lung  are  congested,  with  some 
perivascular  leukocytosis ;  not  infrequently  meshes  of  fibrin  can  be  made 
out  within  the  layers  of  the  connective  tissue  of  the  pleura.  Upon  the 
surface  the  exudate  is  seen  to  consist  of  delicate  interlacing  fibrillas  of 
fibrin  with  some  leukocytes.  Occasionally,  the  fibrin  is  fused  together 
into  hyaline  masses.  By  appropriate  methods  of  staining,  bacteria  may 
be  demonstrated  in  the  exudate.  In  the  early  stages  it  is  not  always  easy 
to  detect  the  presence  of  pleurisy,  since  there  may  be  merely  a  trifling 
cloudiness  and  turbidity  of  the  membrane,  but  in  more  advanced  cases 
the  fibrin  forms  a  definite  layer,  even  amounting  to  a  membrane. 

Serofibrinous  Pleurisy. — Very  few  pleurisies  remain  dry  for  long  and 
there  is  usually  a  more  or  less  abundant  outpouring  of  fluid  into  the 
cavity  (serofibrinous  pleurisy).  The  serum  exuded  is  of  a  yellowish  color, 
clear,  or,  if  mixed  with  cells,  somewhat  turbid.  The  fibrin  is  usually 
abundant  in  the  fissures  of  the  lung  and  in  the  dependent  or  posterior 
portions  of  the  pleural  cavity.  It  is  often  curdy,  whitish-yellow,  and 
forms  shaggy  masses  adhering  to  the  lung  and  thoracic  walls.  In  many 
cases,  loose,  friable,  rather  gelatinous-looking  clots  are  produced.  The 
amount  of  exudation  varies  from  a  few  cubic  centimeters  to  several  liters. 
The  specific  gravity  is  almost  always  above  1025.  The  fluid  coagulates 
readily  on  the  application  of  heat,  and  often  spontaneously  when 
removed  from  the  body.  The  exudation  differs  from  the  fluid  of  hydro- 
thorax  in  being  of  higher  specific  gravity,  containing  more  albumin,  and 
also  more  uric  acid,  cholesterin,  and  sugar.  Microscopically,  it  contains 
leukocytes  and  blood  cells,  bacteria,  somewhat  swollen  endothelial 
cells,  and  shreds  of  fibrin. 

When  the  effusion  is  large,  the  lung  is  more  or  less  completely 
compressed  and  atelectatic,  lying  in  the  upper  part  of  the  pleural 
cavity  close  to  the  spine,  unless  previously  existing  adhesions  limit 
its  movements.  When  free,  the  fluid  moves  on  posturing  the  patient. 
The  airless  portion  of  the  lung  is  tough,  lacks  crepitation,  and  is  of 
a  dull  gray,  grayish-brown,  or  blue  black  color.  The  heart  may  be 
dislocated  to  the  side  and  the  large  thoracic  vessels  compressed.  The 
diaphragm  is  depressed  and  the  intercostal  spaces  may  bulge.  In 
large  right-sided  effusions  the  liver  is  pushed  down.  When  healing 
takes  place,  the  fluid  portions  are  absorbed  by  the  lymphatics,  the  fibrin 
breaks  down,  becomes  granular,  and  in  its  turn  is  carried  off.  Thus, 
few  signs  of  trouble  may  remain,  except  possibly  a  slight  thickening 
of  the  pleura.  Not  infrequently,  the  connective  tissue,  however,  pro- 
liferates, grows  out  into  the  fibrinous  layer,  and  leads  to  adhesion  of  those 
parts  which  are  in  contact.  The  union  is  at  first  intimate,  but,  as 
the  lung  regains  its  function,  the  adhesions  are  pulled  upon  so  that 
velamentous  strands  are  produced.  These  are  very  commonly  found 
between  the  lobes  and  about  the  upper  lobe  posteriorly.  The  adhesions 
may  be  partial  or  lead  to  complete  obliteration  of  the  pleural  space. 


CHRONIC  OH  PRODUCTIVE  PLEURISY  320 

Purulent  Pleurisy. — Serofibrinous  pleurisy  occasionally  develops  into 
a  purulent  or  fibrinopurtdent  one  (empyetna).  Here  the  exudate  is 
more  cloudy,  yellowish,  and  contains  abundant  leukocytes.  There  is 
often  more  or  less  fibrin,  but  it  tends  to  diminish  both  absolutely  and 
relatively,  as  both  less  is  produced  and  what  is  present  is  gradually 
digested  by  the  action  of  the  pus  corpuscles.  Empyema  is  rarely  a 
primary  affection,  except  in  children.  It  sometimes  is  met  with  in  pneu- 
monia, especially  that  form  due  to  influenza,  but  occurs  perhaps  most 
commonly  from  the  rupture  of  a  tuberculous  cavity  in  the  pleura  or 
follows  abscess  and  gangrene  of  the  lung.  A  subdiaphragmatic  abscess 
may  discharge  into  the  pleura  as  well  as  cancerous  or  other  ulcers 
of  the  oesophagus,  stomach,  or  bowel.  When  putrefactive  germs  are 
present,  the  pus  is  often  dark  colored,  decomposed,  and  very  foul  smell- 
ing. Some  of  the  bacteria,  notably  the  Diplococcus  pneumonise  and  the 
B.  typhi,  which  usually  produce  a  simple  inflammation,  are  competent 
to  produce  pus,  either  from  an  increase  in  their  virulence  or  a  diminution 
in  the  resisting  power  of  the  patient.  As  a  rule,  however,  when  a  simple 
pleurisy  becomes  purulent,  it  is  due  to  secondary  infection  with  pus- 
producing  organisms. 

Unless  relieved  by  surgical  interference,  the  consequences  of  empyema 
are  apt  to  be  serious.  The  patient  may  die  of  exhaustion,  amyloid  disease, 
or  septicemia,  or  the  pus  may  burrow  through  the  lung  and  discharge  into 
a  bronchus,  with  the  formation  of  pyopneumothorax.  The  pus  may  also 
dissect  its  way  beneath  the  parietal  pleura  and  point  externally,  thus 
finally  discharging  (empyema  nccessitatis).  The  usual  site  for  this  is 
near  the  lower  end  of  the  sternum.  The  pus  may  also  discharge  into 
the  oesophagus,  stomach,  peritoneum,  pericardium,  or  mediastinum.  Em- 
pyema, as  a  rule,  unless  recognized  and  treated  early,  usually  leads  to 
great  thickening  of  the  pleura  and,  in  protracted  cases,  to  deformity  of 
the  chest.  In  some  cases  the  pleura  becomes  so  infiltrated  with  lime  salts 
that  it  is  converted  into  a  dense  calcareous  cuirass. 

Hemorrhagic  Pleurisy. — Hemorrhagic  pleurisy  is  not  rare  as  a  secondary 
complication.  It  is  found  in  debilitated  persons  or  those  suffering 
from  scurvy  and  the  hemorrhagic  diathesis.  It  is  common  in  tuber- 
culosis and  carcinoma  of  the  pleura.  In  one  variety,  which  is  strictly 
comparable  to  pachymeningitis  hsemorrhagica,  there  is  a  formation  of 
a  vascular  granulation  tissue  upon  the  surface  of  the  pleura. 

Chronic  or  Productive  Pleurisy. — Chronic  or  productive  pleurisy  is 
common.  It  may  exist  as  a  late  manifestation  of  an  acute  simple  or 
purulent  inflammation,  or  may  begin  insidiously  as  a  primary  affection 
without  exudation.  The  condition  leads  to  great  thickening  of  the  pleura 
with  more  or  less  extensive  adhesions.  The  lung  is  coated  with  a  firm 
whitish  or  whitish-gray  membrane  which  may  be  one  or  more  centimeters 
thick.  Microscopically,  it  is  composed  of  somewhat  interlacing  fibrils  of 
connective  tissue  with  areas  of  coagulation  necrosis,  and  in  the  deeper 
layers  newly-formed  capillaries  and  perivascular  leukocytic  infiltration.  In 
some  cases,  the  membrane  is  very  thick,  pearly  white,  and  of  a  firm  carti- 
laginous consistence  (Zuckerguss).  This  form  is  rare  as  a  primary  dis- 


330  THE  PLEURAE 

ease  of  the  pleura,  but  usually  is  due  to  a  chronic  inflammation  extending 
from  the  peritoneum  or  from  the  pericardium.  Rosenbach1  has  pointed 
out  that  chronic  pleurisy  may  extend  to  the  liver  capsule  causing  con- 
traction of  the  organ  and  to  the  pericardial  sac,  which,  in  time  becomes 
obliterated.  The  cartilaginous  appearance  is  due  to  extensive  hyaline 
degeneration  of  the  connective  tissue.  In  such  cases  the  lung  usually 
shows  some  atrophy,  but  when  adhesions  have  not  taken  place  it  may 
be  considerably  contracted  and  deformed.  In  other  cases,  particularly 
those  connected  with  pneumonia,  the  lung  itself  participates  in  the  pro- 
liferation and  becomes  indurated.  In  long-standing  cases,  calcareous 
masses,  and  even  plates  of  cartilage  and  bone,  are  formed  in  the  pleura. 
The  disease  has  been  known  to  follow  simple  pleurisy  and  tuberculosis. 

Tuberculosis. — Exceptionally,  tuberculosis  of  the  pleura  is  met  with 
as  a  primary  manifestation  without  discoverable  disease  elsewhere. 
Such  cases  are  difficult  of  explanation,  but  the  bacilli  probably  reach 
the  sac  through  the  blood-stream  or  the  lymphatic  system.  A  common 
form  is  that  in  which  the  pleura  is  affected  as  a  part  of  a  general  miliary 
dissemination  of  the  disease.  Here  the  membrane  is  studded  with  pin- 
point tubercles,  with  possibly  slight  surrounding  congestion,  but  without 
exudation  or  adhesion,  so  that  the  inflammatory  manifestations  are  of 
the  slightest.  Most  frequently,  tuberculosis  of  the  pleura  is  an  extension 
from  the  lung  or  peribronchial  glands,  and  it  has  been  found  to  be  occa- 
sioned by  tuberculosis  of  the  peritoneum,  the  ribs,  and  the  spinal  column. 
In  tuberculous  bronchopneumonia  it  is  not  uncommon  to  find  clusters  of 
tubercles  upon  the  pleura,  somewhat  elevated  above  the  general  surface, 
and  covered  with  a  delicate  layer  of  fibrin.  In  other  cases,  a  more 
abundant  exudation  takes  place,  frequently  of  a  serofibrinous  character, 
and  with  some  admixture  of  blood.  The  surface  of  the  lung  is  covered 
with  a  layer  of  blood-stained  fibrin  that  may  be  readily  removed.  On 
scraping  it  off,  one  can  make  out  tubercles  on  the  under  surface.  When 
the  disease  is  of  long  standing,  several  layers  of  caseating  tubercles  are 
formed  in  the  exudation  which  is  converted  into  a  thick,  cheesy,  and 
brittle  membrane.  Another  common  form,  sometimes  combined  with 
the  last,  is  that  where  a  more  or  less  extensive  adhesion  of  the  pleural 
surfaces  takes  place.  In  the  adhesions,  which  are  due  to  the  organiza- 
tion of  the  exudate,  isolated  or  confluent  caseous  tubercles  are  produced, 
leading  to  marked  thickening. 

A  purulent  tuberculous  pleurisy  also  exists  and  is  due  commonly  to  the 
rupture  of  a  cavity  into  the  pleural  space.  Here,  no  doubt,  a  mixed 
infection  is  at  work. 

A  word  should  be  said  here  about  a  peculiar  form  that  is  common 
in  cattle  and  has  been  met  with,  though  rarely,  in  man — the  so-called 
"grape  disease"  or  "Perlsucht."  In  cattle,  the  disease  is  frequently 
found  not  only  in  the  pleura,  but  in  the  lungs,  lymph-glands,  pericardium, 
peritoneum,  and  liver.  As  it  affects  the  pleura,  the  disease  presents  a 
very  striking  and  characteristic  appearance.  The  tubercles  vary  some- 

1  Die  Erkrankungen  des  Brustfells,  Nothnagel,  14:  1894:  1: 18. 


TUMORS  331 

what  in  size  and  take  the  form  of  warts  and  polypoid  excrescences,  often 
bound  one  to  the  other  by  fibrous  bands,  so  that  they  have  been  been  com- 
pared to  pearls  on  a  string.  At  first  they  are  of  a  gray  or  grayish-red 
color  but  sooner  or  later  degenerate  at  the  centres,  becoming  opaque, 
yellow,  and  brittle.  They  may  finally  calcify.  Hodenpyl  regards  the 
minute,  lenticular,  pearly  dots  sometimes  found  on  the  pleura  as  of 
tuberculous  nature. 

Syphilis. — Syphilitic  induration  of  the  lung,  already  referred  to, 
commonly  leads  to  thickening  of  the  pleura.  Lancereaux1  has  described 
a  pleuritis  gummosa.  It  is  excessively  rare. 

Leprosy. — This  is  found  in  the  form  of  granulomas  of  varying  size 
upon  the  pleura. 

Foreign  Bodies. — These  are  rare  except  blood  and  pus  as  already 
mentioned.  Foreign  bodies  may  be  introduced  from  without,  or  may 
gain  entrance  from  the  stomach  and  oesophagus.  Rarely,  detached 
portions  of  tumors  may  be  found,  or  sequestra  from  the  lung. 

Parasites. — Echinococcus  cysts  are  found  both  primarily  and  second- 
arily. Psorosperms  have  also  been  met  with.  The  Amoeba  coli  is  found 
in  cases  where  an  amoebic  abscess  of  the  liver  has  ruptured  into  the 
pleura. 

PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Hypertrophy,  if  it  can  be  regarded  as  occurring 
at  all,  is  possibly  that  form  of  enlargement  of  the  pleura  that  occurs 
when  the  volume  of  the  lung  is  increased. 

Tumors. — Tumors  are  primary  and  secondary.  The  primary 
benign  growths  are  fibroma,  lipoma,  osteoma,  and  angioma.  Lipomas 
originate  in  the  subserous  fatty  tissue  of  the  intercostal  spaces,  but 
Rokitansky  has  described  a  branching  lipoma  (lipoma  arbor  escens), 
starting  at  the  free  edge  of  the  base  of  the  lung. 

The  most  important  malignant  growth  is  the  endothelioma,  which  is 
found  in  the  pleura  more  frequently  than  elsewhere.  This  may  occur, 
as  in  a  case  coming  under  our  own  observation,  in  the  form  of  minute 
flattened  nodules  of  miliary  type,  but  more  frequently  large  scattered  or 
coalescing  nodules  of  whitish  color,  occasionally  connected  by  fibrous 
bands,  are  produced.  The  growth  leads  to  considerable  infiltration 
and  thickening  of  the  pleura,  and  is  usually  accompanied  by  a  serous  or  - 
hemorrhagic  pleurisy.  Sometimes  large,  soft,  solitary  tumors  are  pro- 
duced, but  generally  the  tumor  has  more  the  characteristics  of  a  hard 
cancer.  Microscopically,  it  consists  of  a  dense,  fibrous  stroma,  in  which 
are  nests  of  cells  of  an  endothelial  type.  When,  however,  the  tumor 
is  soft  and  rapidly  growing,  the  resemblance  to  sarcoma  is  somewhat 
close.  The  growth  originates  in  an  overgrowth  of  the  lining  cells  of 
the  pleura  and  may  gradually  extend  to  the  lung  and  lymphatic  glands. 

Primary  sarcoma  is  usually  of  the  spindle-celled  variety  and  begins  in 

1  Trait<§  de  la  Syphilis,  1873:  326. 


332  THE  PLEURA 

the  subpleural  connective  tissue.  It  is  said  to  be  common  in  children. 
It  may  extend  to  the  lung  and  lead  to  pressure  upon  the  brachial  plexus 
and  axillary  vessels,  as  in  a  case  examined  by  one  of  us.1 

Secondary  tumors  invade  the  lung  by  metastasis  or  by  direct  extension. 
The  most  common  are  those  due  to  carcinoma  of  the  thyroid,  mamma, 
stomach,  and  oesophagus. 

1  Stewart  and  Adami,  Montreal  Med.  Jour.,  22:  1893-94:  909. 


CHAPTER    XV. 

THE  MEDIASTINUM. 

THE  mediastinum  is  that  portion  of  the  thorax  which  lies  between  the 
two  pleurae,  bounded  in  front  by  the  sternum  and  behind  by  the  verte- 
bral column.  The  anatomists  generally  divide  it  into  two  parts — the 
superior  mediastinum,  lying  above  the  pericardium;  and  the  inferior 
mediastinum,  which  is  further  subdivided  into  three,  the  anterior,  median, 
and  posterior  mediastina.  The  anterior  mediastinum  is  bounded  in 
front  by  the  sternum,  laterally  by  the  pleura,  and  behind  by  the  peri- 
cardium. The  posterior  is  bounded  in  front  by  the  pericardium  and 
roots  of  the  lungs,  laterally  by  the  pleurae,  and  posteriorly  by  the  spinal 
column  from  the  lower  border  of  the  fourth  dorsal  vertebra  downward. 
The  middle  mediastinum  is  the  remaining  space. 

Pathologically,  the  mediastinum  interests  us  on  account  of  the  great 
number  of  important  organs  which  it  contains  and  its  intimate  relation- 
ships with  other  parts.  All  the  viscera  of  the  thorax  with  the  exception 
of  the  lungs  and  pleurae  are  to  be  found  within  it. 

The  superior  mediastinum  contains  the  origins  of  the  sternohyoid 
and  sternothyroid  muscles  and  the  lower  ends  of  the  longus  colli;  the 
transverse  part  of  the  aortic  arch;  the  innominate,  left  carotid,  and  sub- 
clavian  arteries;  the  vena  cava  superior,  the  innominate  veins,  and  the 
left  superior  intercostal  vein;  the  pneumogastric,  cardiac,  phrenic,  and 
left  recurrent  laryngeal  nerves;  the  trachea,  oesophagus,  and  thoracic 
duct;  the  thymus  gland  and  lymphatics. 

The  anterior  mediastinum  contains  the  origins  of  the  triangularis 
sterni  muscles,  the  internal  mammary  vessels,  some  areolar  tissue,  and 
some  lymphatic  channels  and  nodes. 

The  middle  mediastinum  contains  the  heart  and  pericardium,  the 
ascending  aorta,  the  superior  vena  cava,  the  bifurcation  of  the  trachea, 
the  pulmonary  arteries  and  veins,  and  the  phrenic  nerves. 

The  posterior  mediastinum  contains  the  descending  limb  of  the  aortic 
arch,  the  descending  thoracic  aorta,  the  greater  and  lesser  azygos  veins, 
the  pneumogastric  and  splanchnic  nerves,  the  oesophagus,  thoracic  duct, 
and  some  lymph-nodes. 

In  the  consideration  of  disorders  of  this  part  of  the  body,  we  need  deal 
with  only  a  few,  but  they  are  of  not  a  little  importance.  The  affections 
of  the  trachea,  bronchi,  oesophagus,  heart,  pericardium,  vessels,  and 
nerves  are  more  conveniently  described  elsewhere,  though  it  must  not 
be  forgotten  that  the  mediastinum  is  often  secondarily  involved  in  dis- 
ease of  these  structures.  In  this  place,  therefore,  we  shall  confine  our 
remarks  chiefly  to  the  areolar  connective  tissue,  the  lymph-nodes,  the 
thymus  gland,  and  the  various  ailments  affecting  them. 


334  THE  MEDIASTINUM 


CONGENITAL  ANOMALIES. 

The  mediastina  will,  of  course,  be  modified  in  their  shape,  extent,  and 
boundaries  by  anomalies  of  development  of  the  heart,  lung,  vertebral 
column,  and  sternum. 

ACQUIRED  ANOMALIES  OF  SIZE,  SHAPE,  AND  POSITION. 

Transudations,  inflammatory  effusions,  extravasations  of  blood;  infil- 
trations, inflammatory  or  neoplastic,  whether  of  the  mediastinal  space 
itself  of  the  neighboring  viscera  and  serous  cavities;  and  aneurisms  will 
alter  the  size,  shape,  and  position  of  the  mediastina.  In  cases  of  exten- 
sive pleural  effusion  the  heart  and  mediastinum  may  be  dislocated  con- 
siderably to  one  side;  or  the  mediastinum  may  be  dragged  out  of  its 
normal  position  by  induration  and  retraction  of  the  lung. 

CIRCULATORY  DISTURBANCES. 

Hyperemia. — Active  Congestion. — Active  congestion  occurs  in  the  first 
stages  of  acute  inflammation. 

Passive  Congestion. — Passive  congestion  is  found  in  general  venous 
stasis,  and  in  local  conditions  which  lead  to  obstruction  in  the  veins 
leaving  the  mediastinum,  such  as  enlarged  glands,  and  tumors.  Hemor- 
rhage into  the  mediastinum  may  arise  from  traumatism,  the  erosion  of 
vessels,  or  the  rupture  of  an  aortic  aneurism. 

INFLAMMATIONS. 

Mediastinitis. — Inflammation  of  the  mediastinum — mediastinitis — 
is  not  uncommon,  and  is  to  be  attributed  to  trauma,  the  extension  of 
disease  from  neighboring  parts,  and  to  hematogenic  infection. 

Traumatic  Mediastinitis. — The  traumatic  form  is  due  to  external 
injuries,  such  as  stab  wounds  or  gunshot  wounds.  Occasionally,  too, 
foreign  bodies  within  the  oesophagus  may  be  the  cause. 

Mediastinitis  by  Extension. — Mediastinitis  arising  per  extensionem  is 
much  the  most  common  form.  It  may  be  secondary  to  pleurisy,  pericar- 
ditis, or  peritonitis;  it  may  extend  from  the  neck  along  the  vessels,  from 
the  retropharyngeal  glands,  the  larynx,  trachea,  or  oesophagus;  from  the 
thymus,  lungs,  or  bronchial  glands;  or  from  the  vertebrae.  According 
to  the  type,  we  may  recognize  simple,  suppurative,  and  specific  medias- 
tinitis; these  may,  again,  be  acute  or  chronic. 

Hematogenic  or  Metastatic  Mediastinitis. — Hematogenic  or  metastatic 
mediastinitis  has  been  met  with  in  connection  with  typhoid  fever,  ery- 
sipelas, acute  rheumatism,  pneumonia,  and  variola. 

Simple  mediastinitis  usually  is  a  complication  of  acute  pleurisy  or 
pericarditis.  It  is  almost  certain,  too,  that  there  is  an  inflammatory 
hyperplasia  of  the  mediastinal  glands  in  cases  of  bronchitis,  pneumonia, 


SYPHILIS  335 

and  many  of  the  infectious  fevers.  No  doubt  most  of  these  cases  heal 
without  leaving  any  untoward  results,  but  occasionally  the  process  ends 
in  suppuration,  or  the  glands  become  chronically  enlarged  and  indu- 
rated. Subsequently,  should  contraction  of  the  inflamed  structures 
occur,  we  may  get  traction  or  pressure  upon  important  structures,  such 
as  the  vessels,  trachea,  bronchi,  or  oesophagus.  One  form  of  diverticulum 
of  the  oesophagus  is  due  to  the  traction  of  a  contracting  gland  which 
has  become  adherent  to  this  organ.  Mediastinal  abscesses  may  extend 
and  rupture  externally,  but  have  been  known  to  discharge  into  the 
trachea,  oesophagus,  pleural  cavity,  pericardial  sac,  left  ventricle,  and 
aorta.  A  common  sequel  of  mediastinal  inflammation  is  the  formation 
of  bands  of  adhesion  between  the  external  surface  of  the  pericardium 
and  the  pleura,  or  between  the  pericardium  and  the  chest  wall  (mediastino- 
pericarditis).  In  the  latter  event  systolic  retraction  of  the  thoracic  wall 
in  the  neighborhood  of  the  apex  of  the  heart  may  be  produced,  a  fact 
that  is  of  some  diagnostic  import  in  connection  with  pericarditis.  In 
some  cases  adhesions  may  be  so  widespread  that  the  mediastinal  space  is 
practically  obliterated. 

Apart  from  the  adhesion  and  induration  of  the  tissues  just  referred  to, 
which  are  to  be  regarded  rather  as  relics  of  an  inflammation  past  and 
gone  than  as  evidences  of  a  presently  active  process,  there  is  a  form  of 
chronic  mediastinal  inflammation  which  is  of  a  steadily  progressive 
character.  In  this  case  the  process  begins  either  as  a  perihepatitis,  which 
extends  to  the  mediastinum  by  the  lymphatics,  involving  in  its  course  the 
right  pleura,  or  as  a  pericarditis.  The  mediastinal  space  is  obliterated 
and  the  various  serous  sacs  are  eventually  more  or  less  completely  involved 
(multiple  progressive  hyaloserositis,1  chronic  multiserositis,  polyorrho- 
menitis,  Concato's  disease).  The  adhesions  produced  are  very  numerous 
and  dense  and  the  newly-formed  fibrous  tissue  may  undergo  hyaline 
degeneration,  so  that  a  peculiar  substance,  of  pearly  white  color  and 
cartilaginous  appearance,  is  produced.  This  material  may  form  thick 
sheets  on  the  surface  of  the  different  viscera,  liver,  lungs,  or  heart 
(Zuckerguss). 

Tuberculosis. — Tuberculosis  of  the  mediastinum  arises  by  extension 
from  the  vertebral  column  or  from  the  lymph-nodes.  It  is  frequently 
suppurative  in  type. 

Syphilis. — Syphilis  of  the  mediastinum  appears  to  be  as  rare  as 
tuberculosis  is  common.  The  few  recorded  cases  appear  to  have  been 
secondary  to  gummata  of  the  sternum  or  ribs.  In  one  there  was  enlarge- 
ment of  the  mediastinal  glands  as  well. 

Xo  consideration  of  inflammation  of  the  mediastinal  structures  would 
be  complete  without  a  more  detailed  reference  to  the  important  role 
played  by  the  mediastinal  lymph-nodes.  Repeated  observations  have 
proved  beyond  question  that  the  tracheobronchial  lymph-nodes,  both  in 
man  and  the  lower  animals,  not  infrequently  contain  living  bacteria, 
even  in  the  absence  of  local  disturbance  or  disease  elsewhere.  The 
presence  of  pneumococci,  staphylococci,  or  tubercle  bacilli  has  been 

1  Nicholls,  Studies  from  the  Royal  Victoria  Hospital,  1:  1902:  No.  3. 


336  THE  MEDIASTINUM 

determined  in  these  cases.  Pizzini,  for  instance,  found  the  bacilli  of 
tuberculosis  in  the  peribronchial  nodes  of  non-tuberculous  adults,  dying 
from  accident,  suicide,  or  acute  infectious  disease,  in  42  per  cent,  of 
cases.  This  being  the  fact,  the  potency  for  evil  of  the  mediastinal 
lymph  apparatus  must  be  admitted.  Moreover,  in  probably  every  case 
of  bronchitis  and  pneumonia  the  tracheobronchial  and  peribronchial 
lymph-nodes  are  involved,  as  are  the  anterior  mediastinal  nodes  in  acute 
peritonitis.  A  simple  inflammatory  hyperplasia  may  result,  either  acute 
or  chronic,  which  may  lead  to  enlargement  and  often,  finally,  induration 
of  the  structures;  or,  again,  a  suppurative  process  may  be  initiated. 
Among  the  commonest  forms  of  disease  of  the  mediastinal  lymph-nodes 
is  tuberculosis,  which  may  be  of  the  acute  miliary  or  the  caseating  type. 
It  was  for  a.  long  time  thought  that  this  affection  was  secondary  to  pul- 
monary tuberculosis.  Considerable  evidence  has  now  accumulated  to 
show  that  this  view  is  incorrect,  however.  Weigert  has  demonstrated 
that  the  dissemination  of  tubercle  bacilli  in  the  lung  follows  the  same 
path  as  the  absorption  of  coal  dust  or  other  pigments,  and  the  work  of 
Aufrecht  proves  that  it  is  next  to  impossible  for  foreign  particles  to  reach 
the  alveoli  of  the  lung  by  inhalation,  save  in  the  possible  case  of  forced 
inspiration.  The  observations  of  Ribbert,  Baumgarten,  and  others,  re- 
ferred to  above,  strongly  support  the  view  that  tubercle  bacilli  do  not 
reach  the  lungs  directly,  but,  entering  by  way  of  the  nasopharyngeal 
mucosa,  pass  into  the  cervical  chain  of  lymph-nodes  and  thence  into  the 
tracheobronchial  and  peribronchial  group.  The  exact  method  by  which 
the  lungs  eventually  become  infected  is  less  certain,  but  it  is  probable 
that  it  is  either  by  retrograde  metastasis  in  some  cases,  or,  in  others, 
by  the  rupture  of  a  caseous  focus  into  one  of  the  pulmonary  arteries. 
The  presence,  then,  of  infected  lymph-nodes  opens  up  several  possibilities. 
Inflammation  may  spread  throughout  the  mediastinum  and  eventually 
involve  important  structures.  Enlarged  masses  of  nodes  may  press 
upon  the  trachea,  bronchi,  oesophagus,  or  large  vessels.  Cicatricial 
contraction  of  the  chronically  inflamed  groups  may  lead  to  traction  upon 
the  same  structures.  In  this  way  diverticula  of  the  hollow  viscera 
are  not  uncommonly  produced.  Caseous  or  suppurating  foci  may 
discharge  into  the  mediastinal  space  or  into  the  trachea,  bronchi,  arteries, 
or  veins.  Further,  abscesses  have  been  known  to  rupture  externally, 
or  into  various  cavities  and  viscera.  The  results  are  often,  therefore, 
far-reaching. 

Parasites. — Simple  and  echinococcus  cysts  of  the  mediastinum  have 
been  described. 

PROGRESSIVE  METAMORPHOSES. 

Tumors. — Tumors  of  the  mediastinum  may  be  primary  and  sec- 
ondary. The  secondary  growths  originate  in  the  bronchi,  lungs,  or 
oesophagus  and  involve  the  region  by  direct  extension,  or,  again,  are 
metastatic.  The  primary  neoplasms  begin  in  the  lymph-nodes,  connec- 
tive tissue,  thymuSj  or  in  the  thyroid  or  an  accessory  thyroid, 


TUMORS  337 

The  primary  benign  growths  reported  are  lipoma,  fibroma,  adenoma, 
lymphoma,  chondroma,  and  teratoma.  In  some  cases,  however,  it  is 
impossible  to  determine  whether  such  tumors  have  developed  primarily 
in  the  mediastinum  or  not. 

Among  the  most  common  of  the  benign  tumors  are  the  teratomas 
(dennoid  cysts),  of  which  Christian1  has  made  the  most  recent  study. 
They  are  usually  benign,  but  occasionally  show  evidences  of  malignancy. 
They  originate,  according  to  Marchand,  Koster,  and  Finders,  from  the 
thymus  gland.  Waldeyer  describes  one  which  contained  thyroid  tissue. 
1  Vrmoid  cysts  are  usually  soft,  fluctuating,  occasionally  pulsating,  and 
are  situated  under  one  clavicle  or  on  both  sides  of  the  sternum.  The 
pulsation,  which  may  be  due  to  their  own  vascularity  or  to  transmitted 
impulse  from  the  aorta,  has  led  to  their  being  mistaken  for  aortic  aneu- 
rism. The  contents  of  the  cyst  are  similar  to  those  of  dermoids  elsewhere. 
These  cysts  are  dangerous,  inasmuch  as  they  may  ruture  into  some  im- 
portant structure,  such  as  the  pericardium,  pleura,  left  lung,  the  bronchus, 
or  aorta.  In  about  20  per  cent,  of  cases  they  have  been  diagnosticated 
by  the  presence  of  hair  in  the  sputum. 

Tumors  of  the  mediastinum  may  arise  from  thyroid  tissue.  Occa- 
sionally, the  thyroid  gland  is  situated  much  lower  down  than  usual, 
lying  behind  the  sternum  between  the  trachea  and  cesophagus.  Ac- 
cessory thyroids  may  also  at  times  be  found  in  the  superior  mediastinum. 
Wuhrmann  has  recorded  91  tumors  of  thyroid  origin  in  the  mediastinum, 
75  benign  and  16  malignant.  Such  tumors  may  assume  the  type  of 
an  adenoma,  carcinoma,  or  sarcoma.  They  may  attain  a  large  size. 
Dittrich  mentions  a  substernal  "  endothoracic  struma,"  the  size  of  a 
man's  head,  which  had  compressed  the  right  lung. 

Altogether,  the  most  common  primary  newgrowths  of  the  mediastinum 
are  those  originating  in  the  lymph-nodes.  These  are  by  far  the  most 
frequently  malignant  and  sarcomatous  in  type,  but  benign  lymphoma 
has  been  described. 

Benign  lymphomas  are  to  be  distinguished  from  malignant  lymphomas 
or  lymphosarcomas,  on  the  one  hand,  by  their  localized  non-infiltrating 
character,  and  from  leukemia  and  pseudoleukemia,  on  the  other,  by  the 
absence  of  the  peculiar  blood  changes  and  of  enlargement  of  the  spleen 
and  liver. 

The  most  common  primary  tumor  of  the  mediastinum  is  the  sarcoma, 
which  may  assume  the  form  of  lymphosarcoma,  fibrosarcoma,  round  or 
spindle-celled,  and  alveolar.  Endothelioma  is  also  met  with.  These 
growths  originate  either  in  the  mediastinal  lymph-nodes  or  in  the  areolar 
connective  tissue ;  it  is  not  always  possible  to  determine  which. 

Lymphosarcomas  of  the  mediastinum  are  soft,  yellowish-white  in 
c:>lor,  with  thin- walled  vessels.  They  usually  infiltrate  somewhat 
rapidly,  but  occasionally  are  of  slow  growth.  The  newgrowth  begins  in 
the  lymph-nodes,  bursts  through  the  fibrous  investiture  of  these  structures, 
eventually  invading  all  the  tissues  of  the  mediastinum  and  fusing  them 

1  Dermoid  Cysts,  Jour,  of  Med.  Research,  ii  (N.  S,):  1902: 541. 
22 


338  THE  MEDIASTINUM 

into  a  more  or  less  homogeneous  mass.  The  resulting  tumor  is  often  of 
enormous  size  and  extent,  and  may  involve  the  heart,  lungs,  bronchi, 
oesophagus,  the  sternum,  and  vertebral  column.  It  has  been  known  to 
reach  even  the  meninges  by  way  of  the  intervertebral  foramina.  Un- 
like other  forms  of  sarcoma,  the  mediastinal  lymphosarcoma  gives  rise 
to  metastases  by  preference  in  the  intestinal  tract,  less  often  in  the  paren- 
chymatous  organs,  such  as  the  liver,  spleen,  and  kidneys.  On  the  whole, 
however,  mediastinal  sarcoma  tends  to  spread  by  local  diffusion,  and 
distant  metastases  are  not  common. 

Histologically,  we  find  an  aggregation  of  small,  round-cells,  held 
together  by  a  variable  amount  of  fibrous  reticulum,  and,  if  small,  pre- 
senting evidence  of  a  capsule.  Multinucleated  and  spindle-shaped  cells 
are  not  often  seen. 

According  to  Hare,1  the  most  frequent  malignant  newgrowth  of  the 
mediastinum  is  the  carcinoma.  This,  however,  is  almost  always  second- 
ary in  character,  the  few  exceptions  being  those  forms  derived  from 
thyroid  tissue  above  referred  to.  Some  of  the  cases  reported  by  the 
older  pathologists  as  carcinomas  probably  would  now  be  placed  in  the 
category  of  endotheliomas. 

The  secondary  tumors  of  the  mediastinum  are  carcinomatous  or  sar- 
comatous,  and  are  confined  to  the  lymph-nodes.  In  the  case  of  carci- 
noma, the  primary  growth  is  usually  to  be  found  in  the  breast  or  lung, 
less  often  in  gall-bladder,  kidney,  pancreas,  or  stomach. 

Secondary  sarcoma  is  not  very  frequent,  but  has  been  observed  in 
cases  of  sarcoma  of  the  upper  extremity. 

The  symptoms  resulting  from  the  presence  of  mediastinal  tumors 
of  all  kinds  depend  largely  on  their  size  and  position,  and,  in  general, 
are  those  of  pressure  and  irritation,  together  with,  in  some  instances, 
the  ordinary  features  of  malignancy.  At  first,  there  is  usually  a  sub- 
jective sensation  of  fulness  and  pressure,  generally  referred  to  the  neck, 
with  some  palpitation  of  the  heart,  but  with  at  first  no  pain.  Later, 
actual  dyspnoea  sets  in,  due  to  pressure  upon  one  or  more  of  the  impor- 
tant structures  within  the  thorax,  trachea,  bronchi,  the  veins  of  the  heart, 
nerve  or  lungs,  and  the  recurrent  laryngeal  nerves.  Irritation  of  the  vagus 
possibly  accounts  for  the  cough,  vomiting,  palpitation,  regurgitation  of 
food,  and  the  girdle  sensation  complained  of  in  some  cases.  Irritation  of 
the  sympathetic  leads  to  dilatation  of  the  pupil  on  the  affected  side; 
destruction  of  the  nerve  to  contraction  of  the  pupil.  Pressure  on  the 
aorta  will  produce  a  difference  in  the  volume  of  the  radial  or  carotid 
pulse  of  the  two  sides.  Irritation  of  the  phrenic  nerve,  which  is  rare,  pro- 
duces severe  pain  and  singultus.  Compression  of  the  oesophagus  and 
the  thoracic  veins  is  common.  Where  there  is  much  obstruction  to  the 
venous  return,  cyanosis  of  the  head,  chest,  and  arm,  dilatation  of  the 
superficial  veins,  and,  later,  local  oedema,  are  met  with.  Pulsation  may 
be  detected  and  lead  to  the  suspicion  of  an  aneurism. 

1  Affections  of  the  Mediastinum,  Philadelphia,  1889. 


SECTION  III. 
THE    ALIMENTABY   SYSTEM. 


CHAPTER    XVI. 

THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES. 
DEVELOPMENTAL  AND  ANATOMICAL  CONSIDERATIONS. 

BEFORE  we  can  be  prepared  for  an  adequate  conception  of  the  great 
diversity  of  disease  processes  that  occur  in  the  alimentary  tract,  we 
must  be  conversant  with  certain -features  of  the  development  and  ana- 
tomical structure  of  this  system.  Furthermore,  it  is  necessary  to  have 
a  working  knowledge  of  its  normal  functions,  inasmuch  as  it  is  from 
the  physiological  side  alone  that  we  can  attack  many  of  the  problems 
that  confront  us  as  a  result  of  disordered  activity.  We  can  in  this 
way,  and  in  this  way  only,  become  competent  to  recognize  and  to  appre- 
ciate not  only  the  grosser  structural  evidences  of  morbidity,  but  also  the 
internal  and  external  manifestations  of  abnormal  function  to  which 
they  give  rise.  A  very  cursory  examination,  indeed,  would  be  all  that  is 
necessary  to  convince  us  of  the  wide  range  and  importance  of  this  part 
of  our  subject,  for  its  complexity  is  evident  at  once. 

The  alimentary  tract  is  derived  from  the  invagination  of  the  endo- 
dcnnal  layer  of  the  embryo,  supported  by  the  visceral  portion  of  the 
mesoderm.  The  former  provides  the  lining  epithelium  of  the  entire 
digestive  tube,  as  well  as  its  accessory  structures,  the  lungs,  liver,  and 
pancreas.  The  latter  forms  the  muscular  and  serous  coatings,  together 
with  the  mesentery  and  great  omentum.  We  will  not  take  up  time 
in  describing  the  chronological  sequence  of  events,  for  that  would  be 
beside  our  immediate  purpose,  but  will  confine  ourselves  to  indicating 
those  features  that  will  be  of  importance  in  our  subsequent  consideration 
of  our  subject.  The  invagination  of  the  two  tissue  layers  just  mentioned 
continues  until  a  complete  tube  is  formed,  the  primitive  gut  or  archen- 
teron.  This  is  at  an  early  period  of  embryonic  existence  a  simple 
straight  tube,  recalling  the  condition  of  things  found  in  certain  adult 
fishes  and  amphibians,  but  terminating  blindly  at  each  end  without 
external  communication.  The  central  portion,  or  mid-gut,  as  it  is  called, 
is  connected  with  the  yolk-sac  by  a  wide  passage,  which  ultimately 
becomes  contracted  to  form  the  omphalomesenteric  or  mtelline  duct. 


340     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

From  the  posterior  part,  or  hind-gut,  a  diverticulum  grows  out,  forming 
a  thick-walled  stem,  the  allantoic  stalk.  These  two  structures  eventually 
become  approximated,  and,  together  with  the  umbilical  arteries  and  vein, 
form  the  umbilical  cord.  As  development  proceeds,  the  primitive 
gut  increases  in  length,  but  the  body  cavity  enlarges  disproportionately, 
so  that  the  tissues  uniting  the  dorsal  and  ventral  aspects  of  the  gut  to 
the  body  wall  become  elongated  to  form  ultimately  two  ligaments,  each 
composed  of  two  serous  layers  united  by  connective  tissue.  These 
are  the  dorsal  and  the  ventral  mesenteries.  During  the  fourth  week  of 


FIG.  81 


Diagrammatic  schema  of  the  alimentary  canal  of  a  human  embryo,  twenty-eight  days  old:  Pf, 
pituitary  fossa;  Tg,  tongue;  Lx,  larynx;  T,  trachea;  O,  (Esophagus;  L,  lung;  S,  stomach;  P, 
pancreas;  HD,  hepatic  duct;  Lv,  liver;  VD,  vitelline  duct;  Al,  allantois;  HG,  hind-gut;  K,  kid- 
ney; WD,  Wolffian  duct. 

foetal  life  the  various  parts  of  the  alimentary  tract  begin  to  be  differenti- 
ated. The  dorsal  aspect  of  the  tube  toward  the  head  gradually  bulges 
backward  to  form  the  primitive  stomach.  The  liver  begins  as  a  diverti- 
culum, which  arises  on  the  ventral  aspect  of  the  archenteron  just  below 
a  point  corresponding  to  the  future  duodenum.  Sometime  later,  a 
similar  pouching  of  the  dorsal  side  of  the  same  portion  gives  rise  to  the 
pancreas.  The  accompanying  diagram  shows  very  clearly  the  position 
of  things  at  this  stage. 

The  mouth  is  at  first  indicated  by  a  pit  (stomodceum)  on  the  under 
surface  of  the  primitive  head,  which  gradually  deepens  until  it  meets 


DEVELOPMENTAL  AND  ANATOMICAL  CONSIDERATIONS     341 

the  blind  end  of  the  foregut.  At  first,  a  thin  membrane,  composed  of 
ectoderm  and  endoderm,  the  pharyngeal  membrane,  separates  the  two, 
but  this  finally  ruptures  and  communication  is  thus  established.  The 
amis  is  formed  in  a  somewhat  similar  way.  The  tissues  in  a  small  area 
on  the  ventral  aspect  of  the  body,  in  front  of  the  neurenteric  canal, 
become  thinned  and  again  form  a  depression  (proctodoeum)  which 
gradually  approximates  to  the  hind  gut  until  it  is  separated  from  it  by 
an  anal  membrane,  which,  in  time,  also  disappears. 

The  intestine  thus  constituted  soon  loses  its  primitive  simplicity.  It 
becomes  somewhat  folded,  and  we  can  early  distinguish  four  divisions: 
The  first  becomes  the  duodenum;  the  second,  the  small  intestine;  the 
third,  the  colon;  and  the  fourth,  the  sigmoid  and  rectum.  There  is  at 
first  no  ascending  colon,  for  the  cecum  is  situated  high  up  under  the 
liver.  It  gradually  descends,  however,  and  this  portion  of  the  gut 
elongates  to  form  the  ascending  portion.  Finally,  the  small  intestine 
becomes  extremely  long  and  convoluted. 

The  stomach,  which  at  first  is  vertical,  its  long  diameter  being  parallel 
to  the  vertebral  column,  a  condition  that  occasionally  persists  into  adult 
life,  alters  its  position  considerably  by  rotation  in  two  axes.  The  long 
axis  becomes  oblique  and,  later,  almost  transverse,  owing  to  rotation  on 
the  dorsoventral  axis.  The  pylorus,  thus,  comes  to  be  on  the  right  side 
and  lies  somewhat  higher  than  the  cardia.  During  the  same  period 
the  stomach  also  rotates  on  its  longitudinal  axis,  the  left  aspect  becoming 
anterior  and  the  right  posterior.  Thus,  the  greater  curvature  assumes 
the  lower  position,  and  the  lesser  the  upper.  This  torsion,  also,  to  some 
extent  affects  the  lower  part  of  the  oesophagus. 

A  recollection  of  the  facts  just  mentioned  will  suggest  an  explanation 
for  many  of  the  anomalies  of  development  that  are  met  with  in  later 
life;  for  example,  atresia  oris,  imperf orate  anus,  tracheo-cesophageal 
fistula,  omphalomesenteric  fistula,  preperitoneal  cyst,  enterocystoma, 
aberrant  pancreas  and  liver,  certain  regional  hypoplasias,  and  the  like. 

Apart  from  the  embryological  considerations  that  we  have  just  dis- 
cussed, there  are  certain  other  points  which  have  an  important  bearing 
on  the  etiology  of  disease  of  the  alimentary  tract  and  its  accessories,  to 
which  a  brief  reference  should  be  made.  These  have  to  do,  on  the 
one  hand,  with  the  anatomical  structure  and  peculiarities  of  the  tract 
itself,  and,  on  the  other,  with  the  relationships  which  the  tract  bears  to 
other  systems  and  to  the  body  at  large. 

The  first  important  fact  is  that  the  mucous  membrane  of  the  alimentary 
system  is  lined  with  cells  that  are  of  different  types  in  various  parts. 
Thus,  in  the  mouth  and  oesophagus  we  have  stratified  squamous  epi- 
thelium; in  the  stomach  we  have  columnar  cells  of  certain  specialized 
types;  in  the  intestine,  columnar  cells,  of  a  different  character  still, 
arranged  in  a  characteristic  and  complicated  way.  The  squamous 
cells  have  little  to  do  with  absorption  or  with  secretion,  and  from  their 
nature  are  adapted  rather  to  a  protective  function.  Therefore,  they  are 
but  little  likely  to  get  out  of  gear  and  are  relatively  insusceptible  to  irrita- 
tion and  other  forms  of  trauma.  The  more  highly  specialized  and  deli- 


342     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

cate  cells  of  the  stomach  and  intestine  are  much  more  easily  deranged 
and  are  liable  to  disorder  from  a  great  variety  of  sources.  Any 
derangement,  too,  of  these  structures  will  be  likely  to  be  attended  by 
far-reaching  results. 

Another  point  is,  that  there  are  several  places  at  which  the  epithelium 
is  transitional,  for  example,  at  the  lip,  the  passage  of  the  oesophagus  into 
the  stomach,  the  pyloric  ring,  and  the  anus.  These  are  points  of  danger, 
because  the  lining  cells  at  these  points  are  more  or  less  unstable.  Con- 
sequently, we  find  that  they  are  the  favorite  seats  of  carcinoma.  Again, 
at  certain  points,  the  lumen  of  the  alimentary  tube  is  narrowed,  for 
instance,  at  the  level  of  the  cricoid  cartilage,  the  pylorus,  and  the  anus. 
Mechanical  irritation  and  stasis  are,  therefore,  more  likely  to  occur  at 
such  places  and  set  up  inflammation  and  newgrowth.  A  similar  result 
is  apt  to  occur  at  points  where  there  is  an  abrupt  turn  in  the  direction  of 
the  tube,  at  the  cardia  of  the  stomach,  the  duodenum,  the  ileocecal  valve, 
the  hepatic  and  splenic  flexures  of  the  colon. 

The  extreme  mobility  of  the  gastro-intestinal  tract  is  also  an  element  of 
danger,  in  that  dilatation  and  obstruction  of  the  lumen,  displacements, 
kinks,  twists,  and  invaginations  are  comparatively  easily  brought  about, 
any  of  which  may  be  most  of  serious  moment. 

Moreover,  the  alimentary  tube,  being  in  direct  communication  with  the 
external  air,  is  a  breeding  place  for  bacteria,  some  harmless  or  possibly 
even  beneficial;  others  potentially  dangerous  and  at  times  working 
havoc,  not  only  on  the  alimentary  tract  itself,  but  in  the  general  bodily 
system. 

The  digestive  tract,  finally,  is  brought  into  touch  with  the  body, 
as  a  whole,  through  the  medium  of  the  blood  and  lymph-circulatory 
systems  and  the  nervous  system.  Toxic  substances  and  infective  agents 
may  be  carried  to  the  tract  or  away  from  it,  congestion  may  occur  and 
lead  to  lowered  vitality,  catarrh,  and  impaired  function.  Disorders  of  the 
nervous  mechanism  may  lead  to  impaired  motility  and  secretion.  The 
absorption  of  toxic  matters,  in  turn,  may  affect  the  nerve  trunks  and 
centres.  These  relationships  will  be  dealt  with  in  more  detail  later. 

The  functions  of  the  alimentary  system,  stated  briefly,  are:  (1)  to 
ingest  foodstuffs,  and,  by  mechanical  action,  to  render  them  more 
easily  acted  upon  by  the  various  digestive  ferments;  (2)  To  convert 
by  the  secretory  activities  of  the  mucous  membranes  lining  the  digestive 
tube,  and  of  the  parenchymatous  cells  of  its  accessory  glands,  substances 
largely  insoluble  into  those  that  are  largely  soluble,  thereby  preparing 
them  for  incorporation  into  living  cells  and  vital  fluids;  (3)  to  absorb 
and  assimilate  the  substances  thus  transformed;  and  (4)  to  eliminate 
from  the  body  those  products  that  are  unnecessary  or  even  harmful 
to  the  economy.  We  may,  therefore,  consider  this  subject  under  the 
headings  of  mastication  and  propulsion,  digestion,  absorption  and  assimi- 
lation, and  finally,  excretion  and  elimination.  Or,  in  other  words,  it 
may  be  discussed  from  a  mechanical,  a  chemical,  and  a  vitalistic  point  of 
view.  While  this  method,  however,  conduces  to  precise  thought,  it 
must  not  be  imagined  that  the  subject  in  hand  is  so  simple  as,  at  first 


/'///;  MECHANICS  Ob'  blGfiSTlON  ;^4o 

sight,  it  might  appear.  Even  the  normal  processes  connected  with  the 
function  of  alimentation  are  highly  complicated,  and  this  complexity 
and  confusion  become  still  more  confounded  when  we  come  to  deal  with 
diseased  conditions.  While  in  the  alimentary  system  we  have  particularly 
well  exemplified  the  peculiar  features  of  a  division  of  labor,  the  factors 
above  mentioned,  while  separate  and  distinct,  are  still  in  a  large  measure 
mutually  complementary.  A  disorder  of  one  function  is  liable  to  be 
followed  by  disorder  of  another,  and  may  even  lead  to  a  derangement  of 
the  general  system.  Thus,  an  insufficiency  in  the  motor  power  of  the 
digestive  tube  leads  to  abnormal  fermentation  of  its  contents,  impaired 
digestion,  and  systemic  intoxication.  Defects  in  its  secretory  functions, 
again,  may  alter  its  motor  functions.  Not  infrequently  a  "  vicious 
circle"  is  thus  produced.  Or,  again,  a  local  condition  of  the  tract  will 
produce  an  effect  for  good  or  evil  on  a  distant  portion  of  it  or  on  the 
economy  at  large.  A  correlation,  more  or  less  intimate,  therefore,  exists 
between  its  various  functions  and  between  its  anatomical  divisions. 


THE  MECHANICS  OF  DIGESTION. 

The  purely  mechanical  functions  of  the  alimentary  tract  are  con- 
cerned with  the  duties  of  ingestion,  mastication,  deglutition,  admixture, 
propulsion,  and  defecation. 

Food  is  taken  into  the  mouth  through  the  mutual  cooperation  of  the 
lips,  teeth,  cheeks,  and  tongue.  It  is  ground  up  by  the  teeth  and  jaws 
so  as  to  provide  a  greater  surface  for  the  action  of  the  digestive  ferments. 
It  is,  by  the  tongue  and  cheeks,  intermingled  with  saliva  and  mucus, 
and  rolled  into  a  bolus  convenient  for  swallowing.  The  process  of 
deglutition  is  somewhat  intricate,  for  during  the  act  the  larynx  must  be 
shut  off  by  becoming  raised  up  under  the  back  of  the  tongue  and 
by  the  epiglottis;  the  nasal  cavity,  by  the  soft  palate  and  the  superior 
constrictors  of  the  pharynx.  This  part  of  the  mechanism  is  a  reflex 
one,  the  centripetal  impulses  originating  in  the  pharyngeal  mucous  mem- 
brane, being  conveyed  to  the  appropriate  centre  in  the  medulla,  and 
from  thence  reflected  to  the  muscles  concerned  by  means  of  the  tri- 
geminal  and  vagus  nerves. 

When,  by  the  contraction  of  the  muscular  wall  of  the  oesophagus, 
the  bolus  is  conveyed  to  the  stomach,  it  is  stored  up  in  the  fundal  portion. 
There,  after  a  brief  period  of  rest,  it  is  intimately  mixed  with  the  gastric 
secretion,  owing  to  a  quiet,  rhythmical  churning  action  of  the  cardia, 
and  is  then  passed  on.  The  antrum  pyloricum  seems  to  have  the  power 
of  picking  out  the  water  and  the  finer  particles  of  food  from  the  rest 
before  permitting  it  to  enter  the  intestine.  By  this  selective  action  the 
more  delicate  bowel  is  protected  from  possible  injury  by  the  larger 
masses  of  food.  Only  a  small  proportion  of  the  total  meal  can  be  acted 
on  in  the  duodenum,  so  that  an  important  function  of  the  stomach  is 
to  act  as  a  receptacle  for  food.  It  is  known  that  the  action  of  the  pyloric 
sphincter  is  intermittent,  periods  of  relaxation  alternating  with  periods 


344     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

of  contraction,  thus  allowing  only  small  amounts  of  food  to  pass  at  a 
time.  How  this  is  brought  about  is  even  yet  not  well  understood,  and 
has  led  to  considerable  theorizing.  The  most  we  can  say  positively  is 
that  the  act  appears  to  be  reflex  and  dependent  on  the  amount  and 
condition  of  the  food  in  the  pyloric  antrum  and  its  degree  of  acidity. 
Once  in  the  duodenum,  the  acidity  of  the  partially  digested  food  is  quickly 
neutralized  by  the  alkaline  contents  of  that  portion  of  the  bowel,  and  it 
is  thus  prepared  for  the  action  of  the  pancreatic  secretion,  the  succus 
entericus,  and  the  bile. 

The  semisolid  and  partially  digested  food,  or  chyme,  as  it  is  now 
called,  is  rapidly  passed  on  through  the  small  intestine  by  peristalsis. 
The  movements  connected  with  peristalsis  appear  to  be  threefold.  There 
is,  first,  a  simultaneous  contraction  of  the  circular  and  longitudinal  fibers 
of  the  muscular  wall,  which  has  the  effect  of  thoroughly  mixing  the 
chyme  and  bringing  every  part  of  it  in  contact  with  the  mucous  mem- 
brane; secondly,  waves  of  contraction,  affecting  the  circular  layer  of 
muscle,  which  tend  to  progress  forward  and,  as  a  consequence,  carry 
along  with  them  the  intestinal  contents;  thirdly,  simultaneous  contrac- 
tions and  relaxations  of  the  muscle,  producing  a  rhythmical  segmenta- 
tion of  the  bowel  and  its  lumen. 

The  ileocecal  valve  is  the  boundary  between  the  large  and  small 
intestine.  It  is  slit-like  in  shape,  and  when  the  cecum  becomes  dis- 
tended with  food  products,  or  is  the  site  of  muscular  contraction,  it  is 
closed,  thus  preventing  the  return  of  the  contained  material  into  the 
small  bowel.  The  movements  of  the  large  intestine  are  not  unlike  those 
of  the  small,  but  are  slower  and  less  vigorous.  In  the  sacculated  portion, 
they  result  largely  in  the  transference  of  material  through  contraction 
from  one  sacculus  to  another,  which  dilates  to  receive  it.  The  move- 
ments of  the  sigmoid  and  rectum  are  comparatively  infrequent. 

Defecation,  or  the  expulsion  of  the  unused  residue  of  the  food  from  the 
body,  is,  or  at  least  may  be,  a  purely  reflex  act.  The  sphincter  ani  is 
usually  kept  in  a  state  of  tonic  contraction  through  the  action  of  a  centre 
in  the  lumbar  cord.  The  accumulation  of  feces  in  the  large  bowel  leads 
to  increased  peristaltic  action,  and,  therefore,  increased  pressure  against 
the  sphincter.  The  lumbar  centre  is  then  inhibited,  and  through  the 
combined  action  of  the  intestinal  and  abdominal  muscles  and  the  levator 
ani,  the  contents  of  the  bowel  are  ejected.  It  should  be  remarked, 
however,  that  in  human  beings,  at  all  events,  the  act  of  defecation  is 
largely  controlled  by  the  will.  The  glottis  is  closed,  the  diaphragm 
is  fixed,  and  with  the  chest  thus  splinted,  as  it  were,  the  abdominal 
muscles  can  contract  effectively,  and  drive  the  contents  of  the  large  bowel 
forward.  The  lumbar  centre  also  appears  to  be  to  some  extent  under 
the  control  of  the  volition.  The  power  of  the  will  to  hasten  or  to  delay 
defecation  is  too  well  known  to  require  comment. 


DEGLUTITION  345 


DISORDERS  OF  THE  DIGESTIVE  MECHANISM. 

Serious  results  may  follow  the  imperfect  performance  of  any  of  the 
motor  functions  of  the  alimentary  canal,  results  that  are  often  far- 
reaching  in  their  character. 

(1)  Mastication. — Insufficient  mastication  may,  for  example,  result 
from  deficient  or  painful  teeth,  inflammation  or  injury  to  the  maxillae 
or  temporomaxillary  joints,  spasm  of  the  muscles  of  the  jaw,  or  paresis 
of  the  muscles  concerned  with  the  movement  of  the  food  within  the 
mouth.  Moreover,  painful  affections  of  the  mouth,  tonsils,  or  parotid 
glands  will  render  patients  averse  to  taking  a  sufficient  amount  of  food. 
If  such  conditions  be  of  some  standing,  malnutrition  of  the  body,  as  a 
whole  will  inevitably  result.  When  food  is  imperfectly  chewed  or 
insalivated,  the  burden  of  the  stomach  is  greatly  increased,  and  digestion 
may  be  greatly  delayed.  In  aggravated  cases  even  gastritis  may  be 
induced. 

Deglutition. — Deglutition  may  be  rendered  difficult  or  impossible  from 
defect  in  the  palate,  and  through  paralysis  of  the  tongue,  cheeks,  or 
oesophagus.  Thus,  the  motor  nuclei  in  the  medulla  may  be  destroyed, 
as  in  bulbar  paralysis  and  tumors  of  the  medulla,  or  there  may  be  a 
peripheral  neuritis,  such  as  is  not  infrequently  met  with  in  diphtheria. 
Similar  results  may  follow  diminished  excitability  of  the  centre  or  of  the 
sensory  nerves.  Spasm  of  the  necessary  muscles,  again,  such  as  we  meet 
with  in  tetanus,  strychnine  poisoning,  hysteria,  and  hydrophobia,  may  pre- 
vent deglutition.  The  appropriate  movements,  not  being  properly  cor- 
related, or  being  rendered  impossible  through  disease  or  physical  defect, 
may  result  in  the  food  taking  an  abnormal  course,  for  example,  into  the 
larynx,  trachea,  or  bronchus,  or,  again,  into  the  nasal  cavity.  Obstruc- 
tion of  the  larynx  leads  rapidly  to  suffocation,  if  the  condition  be  not 
promptly  relieved,  or,  should  the  offending  substance  become  lodged  in  the 
lung,  an  infective  pneumonia,  with  not  infrequently  gangrene,  is  almost 
certain  to  result.  The  passage  of  food  into  the  nasal  cavity  is  attended 
with  discomfort  rather  than  danger,  but  may  be  so  disagreeable  that  the 
person  so  affected  refrains  from  eating.  A  large  proportion  of  the  food 
may  be  lost  in  this  way.  Pain  in  swallowing,  like  pain  in  chewing, 
may  lead  the  patient  to  take  insufficient  nourishment  and  thus  induce 
marasmus. 

In  the  case  of  the  oesophagus,  difficulties  in  the  matter  of  deglutition 
are  usually  to  be  referred  to  positive  obstruction.  This  may  be  due, 
on  the  one  hand,  to  inability  of  the  muscle  to  force  the  food  onward,  or, 
on  the  other,  to  a  narrowing  of  the  lumen,  which  renders  the  onward 
passage  of  the  bolus  difficult  or  impossible.  Not  infrequently,  both 
conditions  are  combined. 

Muscular  insufficiency  of  the  oesophagus  is  always  associated  with 
dilatation  of  its  lumen.  Some  of  the  acute  cases  are,  beyond  doubt, 
due  to  a  primary  paralysis  of  the  musculature,  for  the  condition  has  been 
produced  experimentally  in  the  dog  by  cutting  both  vagi  in  the  neck. 


346     ME  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

As  a  result,  though  the  cardia  remains  open,  the  food  does  not  pass  into 
the  stomach,  but  accumulates  in  the  oesophagus,  decomposes  there,  and 
eventually  causes  death.  There  are  some  cases,  also,  of  so-called  "idio- 
pathic"  dilatation  that  are  apparently  congenital  and  developmental. 

Narrowing  of  the  lumen  of  the  oesophagus  arises  from  a  variety  of 
causes.  Some  few  cases,  such  as  those  found  in  hysteria  and  hypochon- 
driasis,  appear  to  be  due  to  a  functional  disorder  of  the  nature  of  a  spasm 
of  the  lower  end  of  the  organ.  The  majority,  however,  result  from  some 
demonstrable  physical  condition,  cicatricial  contraction,  the  pressure 
or  traction  of  inflammatory  bands,  the  pressure  of  enlarged  glands, 
tumors,  aneurismal  sacs,  or  diverticula.  When  the  stenosis  is  gradual 
in  its  onset,  the  walls  of  the  oesophagus  above  the  stricture  hypertrophy 
and  for  a  time  may  be  able  to  force  the  food  onward.  Sooner  or  later, 
however,  disability  becomes  apparent,  only  the  smaller  particles  of  food 
being  able  to  pass,  and,  finally,  liquids  alone.  Hypertrophy  is  succeeded 
by  dilatation  and  the  inability  may  then  become  complete.  Inflam- 
mation, and  even  ulceration,  of  the  oesophagus  may  be  induced,  and 
death  result  from  infection  or  from  starvation. 

The  symptoms  are  those  of  obstruction  to  the  passage  of  food,  stasis 
and  decomposition  of  the  contents  of  the  sac,  and  regurgitation.  The 
regurgitation  is  quite  different  from  vomiting,  inasmuch  as  the  food 
appears  to  return  of  itself;  there  is  no  nausea,  and  the  abdominal  muscles 
are  not  called  into  play.  The  symptoms  occasionally  resemble  those 
of  rumination.  In  the  cases  where  there  is  only  a  partial  anatomical 
obstruction  or  an  intermittent  functional  stenosis,  symptoms  may  occur 
irregularly,  and  the  condition  is  compatible  with  prolonged  periods  of 
perfect  health.  A  persistent  close  stricture,  on  the  other  hand,  which 
cannot  be  relieved,  results  in  death. 

Apart  from  muscular  weakness  or  stenosis,  deglutition  may  be  rendered 
impossible  through  a  solution  of  the  continuity  of  the  oesophagus.  Rup- 
ture of  this  organ  from  traumatism,  cancerous  infiltration,  or  peptic  self- 
digestion  and  ulceration  has  occasionally  been  met  with. 

(3)  Gastric  Motility. — The  disturbances  of  the  motor  functions  of 
the  stomach  take  the  form  of  overactivity  and  insufficiency. 

Overactivity  may  be  the  result  of  excessive  irritability  of  the  nervous 
mechanism  of  the  stomach,  but  is  much  more  often  due  to  some  difficulty 
in  discharging  its  contents.  Any  obstruction  at  the  pyloric  orifice  may 
bring  this  about,  whether  it  be  from  newgrowth,  the  pressure  of  enlarged 
glands,  the  traction  of  inflammatory  adhesions,  hypertrophy  of  the 
pyloric  ring,  or  spasm  of  the  musculature  of  the  pylorus.  In  such  cases 
the  efforts  of  the  stomach  to  empty  itself  become  very  forcible,  and  the 
increased  peristalsis  may,  in  thin  subjects,  become  visible  on  the  surface 
of  the  abdominal  wall.  Should  the  stomach,  in  spite  of  its  more  powerful 
contractions,  be  unable  to  force  its  contents  onward  within  the  usual 
time,  its  muscle  gradually  weakens  and  dilatation  supervenes  upon  the 
motor  inadequacy. 

Motor  insufficiency,  or  atony  of  the  stomach,  is  a  not  infrequent  condi- 
tion, and  almost  invariably  becomes  associated  with  dilatation.  It  may 


GASTRIC  MOTILITY  ;\\~ 

be  acute  or  chronic.  The  chronic  form  is  usually  the  result  of  some  form 
of  obstruction  to  the  outflow  of  the  gastric  contents  through  the  pylorus, 
or  to  habitual  overeating  or  overdrinking.  The  causes  at  work  are  either 
operative  over  a  prolonged  period  of  time  and  are  gradual  in  their  effects, 
or  are  frequently  repeat ed. 

The  acute  form  may  supervene  upon  the  chronic,  but  is  more  often 
spontaneous.  Here  pyloric  obstruction  is  rare,  the  fault  in  most  cases 
being  in  the  duodenum.  Compression  of  the  duodenum  by  the  root  of 
the  mesentery,  or  by  the  traction  of  adhesions,  or  kinks  leading  to  oblitera- 
tion of  its  lumen,  are  the  usual  lesions  found.  Not  a  few  cases,  how 
many  we  cannot  very  well  say,  appear  to  be  the  result  of  a  primary 
paralysis  of  the  gastric  muscle,  either  due  to  some  toxic  effect  upon  the 
nerve  terminals,  or  to  disease  of  the  vagi,  or,  again,  to  inhibition  of  the 
cerebral  centres.  Conditions  predisposing  to  acute  gastric  dilatation  are 
anesthesia,  prolonged  and  wasting  disease,  indiscretions  in  diet,  deformity 
of  the  spine,  injuries  to  the  head,  back,  and  abdomen.1 

The  results  of  motor  insufficiency  depend  largely  on  the  cause  of  the 
condition  and  its  extent.  In  the  milder  grades,  beyond  some  slight  delay 
in  the  discharge  of  the  gastric  contents,  the  consequences  are  but  slight. 
Should,  however,  food  particles  remain  continuously  in  the  stomach, 
decomposition  sets  in,  with  the  production  of  abnormal  acids  and  gases, 
and  leads  to  serious  disturbance  of  the  secretory  power  of  the  mucosa,2 
and  to  dilatation  of  the  organ.  Gastritis  is,  also,  a  not  infrequent 
result.  The  presence  of  an  excessive  amount  of  food,  the  retention  of 
fluid,  which  is  normally  secreted  by  the  stomach  in  considerable  quantity, 
the  degeneration  of  the  muscle  fibers,  all  contribute  to  the  production 
of  dilatation.  In  cases  of  functional  obstruction,  such  as  may  be  due 
to  spasm,  the  dilatation  is  not  necessarily  associated  with  inability  of 
the  stomach  to  discharge  its  contents.  Examples  of  this  are  found  in 
connection  with  chronic  gastritis,  peptic  ulcer,  and  carcinoma,  and  in 
hyperacidity  and  hypersecretion.3  Possibly,  here,  the  condition  is  an 
intermittent  one,  giving  the  gastric  musculature  time  to  recover.  It 
should  not  be  forgotten,  either,  in  this  connection,  that,  like  other  organs, 
the  stomach  has  a  certain  amount  of  reserve  force,  so  that  for  a  time  a 
slightly  dilated  stomach  may  be  able  to  empty  itself  within  a  reasonable 
period,  and,  so,  continuous  retention  of  food  does  not  occur. 

Where  obstruction  to  the  onward  flow  of  the  stomach  contents  is 
complete  or  nearly  so,  death  will  eventually  take  place  from  inanition, 
unless  the  condition  be  relieved  by  operation.  In  the  less  extreme 
instances,  in  which  there  is  stagnation  of  food  and  the  products  of  secre- 
tion and  digestion,  conditions  are  favorable  for  the  growth  of  micro- 
organisms, so  that  we  have  abnormal  fermentations  going  on  with  all 
that  implies.  Not  only  is  the  normal  process  of  secretion  impaired  by 
the  retention  of  products  that  should  be  removed,  but  the  abnormal  pro- 
duction of  fatty  acids,  gases,  and  other  chemical  substances,  leads  to 

1  Xicholls,  Acute  Dilatation  of  the  Stomach,  International  Clinics,  4: 1908:  80. 

2  Kausch,  Mittheil.  aus  d.  Grenzgeb.,  4 : 347.  3  Kausch,  Ibid.,  7. 


348     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

irritation  of  the  mucosa,  dilatation,  pain,  eructations,  and  vomiting. 
When  the  decomposed  materials,  with  the  enormous  accumulation  of 
bacteria  that  accompanies  them,  is  in  course  of  time  passed  on  into  the 
intestine,  further  irritation  and  further  decomposition  are  initiated. 

In  the  acute  cases  a  lethal  termination  comes  on  more  or  less  rapidly 
in  cases  that  are  not  recognized  and  treated  judiciously,  apparently  from 
the  combined  effects  of  inanition,  shock,  and  collapse. 

Some  curious  nervous  effects  are  produced  by  gastric  dilatation,  such 
as  tetany,  epileptiform  convulsions,  tetanoid  muscular  contractions, 
general  depression,  and  collapse.  Whether  these  are  the  result  of  systemic 
intoxication  owing  to  the  absorption  of  poisonous  substances  from  the 
alimentary  tract,  or  to  more  mechanical  causes,  such  as  the  distension 
of  the  gastric  muscle,  or  the  loss  of  fluids  from  the  body,  is  by  no  means 
definitely  established.  The  striking  observations  of  Verstraeten,  Vander- 
linden,  Halstead,  Loeb,  and  the  MacCallums,1  however,  suggest  an 
explanation  for  a  hitherto  obscure  condition,  for  they  have  proved 
conclusively  that  the  important  factor  in  the  etiology  of  tetany  and 
various  convulsive  phenomena  is  a  deficiency  of  calcium  salts  in  the 
system,  in  some  cases,  at  least,  dependent  on  parathyroid  insufficiency. 

Vomiting  is  a  common  feature  in  dilatation  of  the  stomach.  In  the 
slowly  progressive  chronic  cases  vomiting  occurs  at  comparatively  rare 
intervals,  an  enormous  quantity  of  offensive  material,  consisting  of  un- 
digested food,  fluid,  fermenting  and  decomposing  matter,  being  brought 
up,  the  accumulation  of  several  days.  The  vomiting  in  the  acute  forms 
is  essentially  different,  being  more  of  the  nature  of  a  regurgitation,  the 
material  coming  up  with  little  effort  and  at  brief  intervals.  Food 
has  no  time  to  accumulate,  as  it  is  immediately  rejected,  and  the  vomitus 
consists  chiefly  of  watery  secretion  from  the  stomach,  with  greenish  or 
blackish,  curdy  flakes,  bile,  and  often  a  diastatic  ferment.  The  amount 
brought  up  is  quite  enormous,  as  the  vomiting  is  persistent  and  uncon- 
trollable. 

The  act  of  vomiting  is  a  somewhat  complicated  one,  and  is  governed 
by  a  special  "vomiting  centre"  in  the  medulla,  situated  not  far  from  the 
respiratory  centre.  This  centre  may  be  stimulated  directly  by  toxic 
substances  circulating  in  the  blood,  and  by  a  variety  of  intracranial  condi- 
tions. It  may,  also,  be  affected  reflexly  by  stimuli  reaching  it  from 
other  organs,  such  as  the  nose,  stomach,  peritoneum,  and  uterus.  Vomit- 
ing begins  with  a  deep  inspiration.  The  glottis  is  closed,  the  diaphragm 
is  depressed  and  fixed,  and  the  abdominal  respiratory  muscles  are  con- 
tracted. The  stomach,  in  some  cases  at  least,  undergoes  antiperistaltic 
movements,  and  a  small  amount  of  its  contents  is  aspirated  into  the 
oesophagus.  Finally,  the  abdominal  muscles  contract  vigorously  and 
the  contents  of  the  stomach  and  oesophagus  are  forcibly  expelled  through 
the  mouth.  The  act  of  vomiting,  however,  includes  more  than  this,  for 
it  has  a  profound  systemic  effect.  Salivation  and  sweating  are  common 
occurrences  at  its  inception,  the  blood  pressure  falls,  and  the  pulse  is 

1  W.  G.  MacCallum,  Johns  Hopkins  Hosp.  Bull.,  9:1908:91. 


INTESTINAL  PERISTALSIS  349 

slowed,  owing  to  vagus  stimulation.  Later,  the  blood  pressure  and  the 
rate  of  the  pulse  are  markedly  increased. 

Vomiting  is  always  an  indication  of  disordered  function,  whether  of 
the  stomach  itself  or  of  some  remote  organ  with  which  it  is  connected  by 
nerve  paths.  It  is  essentially  a  nervous  phenomenon,  the  stimulus  of 
which  is  either  peripheral  or  central.  Organic  disease  is  not  necessarily 
present,  and  many  cases  are  purely  functional.  The  power  of  un- 
pleasant sights  or  smells,  for  example,  is  well  known.  The  influence 
of  the  mind  is,  therefore,  apparent. 

Akin  to  vomiting  is  belching,  by  which  is  meant  the  expulsion  of 
gas  through  the  mouth.  This  gas  is  either  air  that  has  been  swallowed 
or  the  product  of  fermentative  processes  going  on  in  the  stomach.  Very 
often  the  gas  brings  with  it  small  quantities  of  liquid,  containing  fatty 
acids  or  hydrochloric  acid,  which  gives  rise  to  unpleasant  burning  sensa- 
tions in  the  mouth  and  gullet.  This  is  known  as  pyrosis  or  "  heart-burn." 
Apparently,  there  is  a  preliminary  relaxation  of  the  cardiac  sphincter  of 
the  stomach,  which  permits  gas  to  be  forced  through  in  consequence 
of  compressive  action  of  the  diaphragm,  abdominal  muscles,  or,  possibly, 
of  the  stomach  wall  itself.  The  eructation  of  gas  in  certain  hysterical 
persons  may  attain  extraordinary  proportions,  the  belching  being  almost 
explosive  in  character  and  recurring  at  frequent  intervals.  The  condition 
appears  to  be  due  in  these  cases  to  the  habit  of  "air-swallowing."  Re- 
flexes from  the  stomach  and  peritoneum  play  an  important  role  in  the 
causation. 

Hiccoughing  is  somewhat  similar,  but  is  due  to  a  clonic  spasm  of  the 
muscles  in  question.  As  in  the  case  of  belching,  it  may  be  initiated  by 
reflexes  from  the  stomach  or  abdominal  cavity.  The  remarkable 
barking  hiccough,  sometimes  met  with  in  hysterical  conditions,  is  perhaps 
due  to  central  causes. 

(4)  Intestinal  Peristalsis. — During  periods  of  complete  fast  the  entire 
gastro-intestinal  tract  is  at  rest.  With  the  initiation  of  the  digestive 
function,  however,  movements  are  instituted  having  for  their  object  the 
thorough  mixing  of  the  food  and  its  subjection  to  the  action  of  the  various 
digestive  ferments  in  turn.  The  peristaltic  movements  of  the  bowels  are 
normally  unobtrusive,  although  the  individual  is  often  conscious  of  their 
presence.  In  thin  persons  they  may  be  seen  and  felt  in  consequence  of 
transmitted  undulations  of  the  anterior  abdominal  wall.  Usually  no 
sound  is  produced. 

Under  abnormal  conditions  these  peristaltic  movements  may  be 
greatly  increased,  both  in  force  and  frequency.  This  is  especially  well 
seen  in  those  cases  where  there  is  some  obstruction  in  the  lumen  of  the 
bowel  to  be  overcome.  As  illustrations  of  this,  may  be  cited  kinks  in 
the  bowel,  volvulus,  cicatricial  contracture  of  the  lumen,  new-growths 
in  the  bowel,  hernias  and  other  causes  of  external  pressure.  When  the 
obstruction  comes  on  suddenly,  violent  cramp-like  movements  of  the 
1)0 wel  above  the  seat  of  the  trouble  are  induced.  Should  the  condition 
be  not  relieved,  however,  these  cease  in  time,  giving  place  to  an  atonic 
and  dilated  condition  of  the  bowel,  which  leads  quickly  to  toxemia, 


350     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

collapse,  and  death.  In  cases  where  the  obstruction  comes  on  more 
gradually,  the  increased  work  of  the  bowel,  in  attempting  to  force  its 
contents  past  the  point  of  narrowing,  results  in  hypertrophy  of  its 
muscular  wall,  though  in  the  later  stages  a  certain  amount  of  dilatation 
is  present  as  well. 

More  common,  and,  fortunately,  less  serious,  are  those  cases  of  in- 
creased peristaltic  action  that  come  on  in  the  absence  of  obstruction. 
To  understand  these  we  must  bear  in  mind  that  the  motor  activities  of 
the  bowel,  like  the  secretory  functions,  are  initiated  by  and  are  largely 
under  the  control  of  the  nervous  system.  Peristalsis  is  commonly  re- 
flex, the  stimulus  being  the  presence  of  solid  and  liquid  matter  within 
the  lumen.  In  some  instances,  however,  the  stimulus  appears  to  be  central, 
as  in  those  cases  due  to  emotion,  excitement,  fear,  worry,  and  the  like. 
The  influence  of  the  mind  over  the  activities  of  the  stomach  and  bowels 
is  a  well-recognized  fact.  Excessive  peristalsis,  then,  may,  conceivably, 
be  due  to  an  increase  in  the  normal  stimulus  within  the  bowel,  to  an 
increased  irritability  of  the  bowel  wall,  whether  nervous  or  muscular, 
or  to  impulses  proceeding  from  the  cerebrum. 

Usually  the  condition  is  accompanied  by  diarrhoea,  though  not  invariably 
so.  In  elderly  persons  and  hysterical  subjects  it  is  not  uncommon  to 
have  gurgling  sounds  (borborygmi)  produced  within  the  intestines,  which 
may  be  audible  at  a  considerable  distance,  without  diarrhoea.  This 
is  more  apt  to  occur  when  the  bowels  contain  gas  but  a  relatively  small 
amount  of  solid  matter.  The  normal  stimulus  to  the  contraction  of  the 
bowels  comes  from  the  coarser,  indigestible  constituents  of  the  food. 
Should  these  be  in  excess,  increased  peristalsis  and  diarrhoea  will  result. 
More  often  chemical  irritants  are  at  work,  either  introduced  with  the 
food  or  the  result  of  faulty  digestion  and  of  abnormal  fermentation. 
The  organic  acids  and  the  various  gases  resulting  from  decomposition 
are  the  chief  offenders.  An  interference  with  the  absorption  of  water  by 
the  bowel,  in  some  instances,  will  cause  diarrhoea. 

Any  condition  that  leads  to  increased  irritability  of  the  intestine, 
either  of  its  mucosa,  muscle,  or  nerves,  favors  the  production  of  diarrhoea, 
for  here  normal  stimuli  may  produce  excessive  response.  In  many  forms 
of  acute  enteritis  such  an  increased  irritability  is  present,  though  we 
have  to  reckon  with  the  stimulating  effects  of  the  products  of  abnormal 
fermentation  as  well.  It  is  singular,  however,  that  a  considerable 
degree  of  inflammation  of  the  bowel  may  be  present  in  some  cases  without 
diarrhoea,  when  we  might  reasonably  have  expected  it.  Typhoid  fever, 
for  example,  is,  in  this  country  at  least,  more  often  associated  with 
constipation  than  with  diarrhoea.  The  same  thing  is  true  also  of  many 
chronic  inflammations  of  the  bowel,  even  when  associated  with  ulceration. 
Such  conditions  are  often  remarkably  sluggish. 

We  have  seen  that,  normally,  the  movements  of  the  gastro-intestinal 
tract  are  more  or  less  influenced  by  the  central  nervous  system.  This 
operates  probably  through  the  agency  of  the  vagus  and  splanchnic 
nerves.  In  hysterical  and  neurasthenic  subjects  comparatively  slight 
stimuli  are  sufficient  to  bring  on  overaction  of  the  bowels.  Even  the 


INTESTINAL  PERISTALSIS  351 

fear  of  a  diarrhoea  may  be  enough  in  certain  susceptible  individuals. 
In  persons  of  a  nervous  type,  worry,  anxiety,  fear,  or  surprise,  in  fact, 
any  sudden  emotion,  will  bring  on  a  watery  evacuation  of  the  bowels. 
l"nder  such  circumstances  the  slightest  indiscretion  in  diet,  also,  may 
be  enough  to  precipitate  an  attack,  though  at  other  times  it  might  be 
insufficient  to  produce  this  result.  Organic  disease  of  the  central 
nervous  system  may,  occasionally,  produce  diarrhoea,  as,  for  example, 
locomotor  ataxia,  during  an  intestinal  crisis. 

The  act  of  defecation  is  to  a  considerable  extent  dependent  on  peri- 
stalsis of  the  bowels.  The  descending  colon  acts  as  a  storehouse  for  the 
unused  residue  from  the  ingesta,  the  rectum  being  ordinarily  a  closed 
tube,  owing  to  the  action  of  the  so-called  third  sphincter  situated  at  its 
upper  end.  With  the  entry  of  feces  into  the  rectum  the  stimulus  comes, 
and  leads  to  a  desire  for  evacuation.  As  has  been  stated  previously,  the 
act  of  defecation  is  more  or  less  under  the  control  of  the  will,  but  not 
entirely  so.  The  act  may  be  delayed  for  a  time,  but  eventually  the  call 
becomes  imperative.  In  cases  of  diarrhoea  the  contraction  of  the 
external  sphincter  cannot  for  long  be  voluntarily  maintained,  and  evacua- 
tion takes  place,  either  as  a  gentle  oozing,  or  with  explosive  force,  accord- 
ing to  the  force  of  the  expulsive  movements  and  the  retaining  power  of 
the  external  sphincter.  In  some  inflammatory  affections  of  the  bowel, 
such  as  dysentery,  the  acts  of  defecation  are  greatly  increased  in  number 
and  are  accompanied  by  painful  sensations  or  tenesmus.  Hemorrhoids, 
fissures  of  the  anus,  and  fistulse ,  also  render  the  act  exceedingly 
painful. 

Involuntary  evacuation  of  the  bowels,  or  incontinence  of  feces,  is,  also, 
not  infrequently  met  with.  It  may  result,  for  example,  from  organic 
disease  of  the  brain  or  cord,  during  delirium  and  coma,  in  sleep  and 
intoxication,  and  from  strong  mental  impressions,  as  fright  or  fear.  It 
is  presumed  in  these  cases  that  the  cerebral  control  is  inhibited. 

Deficient  action  of  the  bowels  is  a  common  complaint,  being,  indeed, 
with  many  an  almost  habitual  condition.  The  term  constipation  implies 
somewhat  more  than  a  mere  infrequency  of  evacuation,  connoting,  as 
it  does,  an  alteration  in  the  character  of  the  feces.  Owing  to  the  fact 
that  the  food  remains  too  long  within  the  intestine,  the  fluid  parts  are, 
to  a  large  extent,  absorbed,  or,  it  may  be,  the  fluids  in  the  food  may 
have  been  deficient  from  the  beginning.  Consequently,  the  feces  become 
firmer  in  consistency,  and,  in  some  cases,  even  dry,  hard,  and  stony. 
The  latter  result  is  in  great  part  an  effect  of  pressure,  and  the  fecal  masses 
often  are  moulded  to  the  shape  of  the  intestinal  cavity.  What  consti- 
tutes the  condition  of  constipation  is  somewhat  hard  to  define.  Some 
persons  have  an  evacuation  of  the  bowels  regularly  once  or  twice  daily, 
often  at  a  stated  time.  Others  may  go  without  a  movement  for  two  or 
three  days.  Still  others,  women  especially,  may  go  a  week  or  ten  days, 
or  even  longer.  Many  persons  affected  in  this  way  with  sluggishness  of 
the  bowels  preserve  the  manners  and  appearance  of  perfect  health. 
They  may  not  perhaps  suffer  from  the  abdominal  fulness,  the  sallow, 
earthy  complexion,  the  furred  tongue,  the  loss  of  appetite,  and  the  head- 


352     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

ache  that  are  so  often  the  accompaniments  of  this  condition,  but  in 
the  more  marked  cases  it  would  be  unsafe  to  assume  that  no  harm  was 
being  done,  even  when  there  are  no  obvious  external  evidences  of  the 
disorder.  A  certain  amount  of  slow  poisoning  may  be  going  on,  which 
will  make  itself  manifest  in  the  end,  or  may  so  sap  the  vital  energy  that 
the  individual  will  fall  a  prey  to  disease  of  other  kinds. 

Infrequent  evacuation  of  the  bowels  may  result  from  gross  obstruction 
to  the  passage  of  feces  through  the  lumen,  or  from  causes  unassociated 
with  such  obstruction. 

Gross  causes  of  obstruction  are  found  either  in  the  intestinal  tube 
itself  or  in  structures  external  but  adjacent  to  it.  Thus,  in  the  first 
class  of  cases,  the  passage  may  be  blocked  by  fecal  masses,  gallstones, 
bones,  or  other  foreign  substances  that  have  been  swallowed,  or  may 
be  completely  obliterated  at  a  given  point  by  volvulus.  The  lumen 
may  be  narrowed  as  a  result  of  chronic  inflammation,  cicatricial  con- 
traction, intussusception,  tumors,  or  hemorrhoids. 

In  the  second  class,  the  obstruction  is  caused  either  by  pressure  or 
traction  exerted  from  without.  The  lumen  may,  for  example,  be 
encroached  upon  or  obliterated  by  hernial  sacs,  fibrous  adhesions,  a 
retroverted  uterus,  or  other  misplaced  organs,  or  tumors.  The  traction 
of  adhesions,  the  dilatation  and  descent  of  the  stomach,  and  the  prolapse 
of  certain  portions  of  the  bowel  owing  to  the  weight  of  tumors  in  the  wall, 
may  result  in  kinking  and  obstruction. 

Much  more  common  are  those  cases  of  constipation  that  are  unassoci- 
ated with  mechanical  obstruction.  As  we  have  seen,  the  function  of 
peristalsis  is  commonly  reflex,  the  stimulus  being  the  presence  of  food 
in  the  bowel  of  sufficient  bulk,  and  especially  the  presence  of  an  indigest- 
ible residue.  An  insufficient  stimulus,  affections  of  the  muscular  wall 
of  the  bowel  interfering  with  its  contractility,  and  derangements  of 
the  nervous  apparatus,  have  all  to  be  reckoned  with  in  a  discussion  of 
the  etiology  of  constipation. 

Unsuitable  diet  plays  an  important  role  in  many  cases.  Food  that 
is  too  concentrated  and  easily  assimilable,  such  as  milk,  eggs,  and  meat, 
will  often  produce  constipation.  A  deficiency  in  the  watery  constituents 
or  of  the  organic  acids  will  have  the  same  effect. 

Deficient  muscular  power  is  a  potent  cause  of  constipation.  We  see 
this  in  elderly  persons  and  those  of  a  sedentary  disposition,  and,  also, 
during  the  convalescence  from  acute  disease.  The  muscle  in  these 
cases  seems  to  undergo  a  certain  amount  of  degeneration  or  atrophy, 
which  considerably  lessens  its  effectiveness.  It  should  be  pointed  out 
in  this  connection  that  muscular  contraction  of  the  intestines  often 
exhibits  a  definite  periodicity,  the  call  for  evacuation  and  the  resulting 
peristalsis  occurring  at  about  the  same  hour  each  day.  Neglect  of  these 
calls  is  one  of  the  most  potent  causes  of  constipation.  The  stimulus 
becomes  increasingly  ineffective,  and,  owing  to  the  accumulation  of 
feces,  the  lower  portion  of  the  large  bowel  becomes  dilated,  its  muscle 
thinned,  and  its  power  of  contraction  correspondingly  diminished. 
Ultimately,  the  power  to  evacuate  by  natural  means  is  entirely  lost,  and 


DISORDERS  OF  SECRETION  353 

can  only  be  excited  by  the  use  of  cathartics.  It  has  been  found  by 
experiment  that  a  certain  amount  of  material  within  the  bowel  is  neces- 
sary to  induce  its  contraction,  but  if  the  bowel  be  greatly  distended  the 
contractility  is  lessened  and  finally  abolished.  The  power  to  contract 
will,  however,  return  if  the  dilatation  be  relieved.  The  constipation  that 
accompanies  peritonitis  appears  to  be  due  to  a  combination  of  causes, 
cloudiness  and  degeneration  of  the  muscle  fibers,  inflammatory  oedema, 
and  inhibition  of  the  nervous  impulses.  A  somewhat  similar  association 
of  morbid  conditions  is  met  with  in  enteritis  and  colitis. 

Reference  should  be  made  here,  also,  to  a  form  of  constipation  thought 
by  many  authorities  to  be  due  to  a  condition  of  hypertonusof  the  intestinal 
muscle.  Thus,  persons  enjoying  robust  health  may  be  the  subjects 
of  constipation.  This  may,  in  part,  be  due  to  the  fact  that  the  assimilative 
functions  of  the  bowel  are  particularly  active,  but,  perhaps,  also,  to  an 
increased  tone  of  the  whole  intestinal  tract.  Relaxation  of  the  bowel 
is  rendered  more  difficult,  and  the  wave-like,  propulsive  movements 
characteristic  of  peristalsis  are  not  so  easily  set  up.  Sometimes  this 
hypertonus  is  so  excessive  as  to  lead  to  actual  spasm  of  certain  portions 
of  the  tract.  The  condition  is  often  associated  with  colic,  and  the  pro- 
pulsion of  the  feces  for  the  time  being  is  rendered  impossible.  This 
form  is  met  with  in  chronic  lead  poisoning. 

The  disorders  of  the  nervous  mechanism  of  the  bowel  and  their  rela- 
tion to  peristalsis  are  as  yet  very  imperfectly  understood.  The  ganglia 
and  plexuses  of  nerves  in  the  intestinal  wall  govern  peristalsis,  and 
changes  in  these  structures  have  been  described  in  connection  with  lead 
poisoning  and  constipation,1  but  similar  appearances  have  been  observed 
in  other  affections. 

Constipation  is  occasionally  met  with  in  both  functional  and  organic 
disease  of  the  central  nervous  system,  as,  for  example,  in  hysteria, 
neurasthenia,  melancholia,  and  meningitis.  Why  this  should  be  we 
do  not  know.  Perhaps  some  centrifugal  impulse  is  interfered  with 
which  is  required  to  bring  about  normal  peristalsis.  Or,  again,  an 
inhibitory  mechanism  is  possibly  set  in  action. 

Constipation  may,  in  some  cases,  be  unattended  by  obvious  signs 
of  disorder,  but  the  condition  undoubtedly  interferes  with  the  proper 
conduct  of  digestion.  Abnormal  fermentation  of  the  intestinal  contents 
takes  place,  leading  to  flatulency.  Poisonous  substances  are  produced 
which  may  enter  the  circulation  and  produce  far-reaching  results. 
Inflammation  and  even  ulceration  of  the  bowel  may  occur,  or,  again, 
the  weight  of  the  retained  material  may  lead  to  prolapse  of  the  bowel 
and  traction  on  important  structures. 

DISORDERS  OF  SECRETION. 

Foodstuffs,  when  reduced  to  their  simplest  constituents,  consist  of 
proteins,  carbohydrates,  fats,  water,  and  certain  mineral  salts.  The 

1  Jurgens,  Berl.  klin.  Woch.,  23:  1882:  357;  Maier,  Virch.  Archiv,  90:1882:455. 
23 


354     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

digestive  fluids  concerned  in  the  transformation  of  food  are  the  saliva, 
gastric  juice,  bile,  pancreatic  secretion,  and  the  succus  entericus.  The 
food  materials  and  the  different  ferments  act  and  react  upon  one  another 
variously  in  different  parts  of  the  alimentary  tract,  so  that  it  is  convenient 
to  discuss  the  process  of  digestion  as  it  takes  place  in  the  mouth,  stomach, 
and  intestines. 

The  Saliva. — In  the  mouth  the  digestive  fluid  is  the  saliva.  This 
consists  of  the  excretions  of  the  various  salivary  glands  discharging  into 
the  buccal  cavity,  and  contains  mucin  and  a  digestive  ferment — ptyalin. 
The  chief  function  of  the  saliva  is,  apparently,  to  facilitate  deglutition,  for 
the  ptyalin,  the  only  chemically  active  substance,  is  absent  in  certain  of 
the  lower  groups  of  animals,  notably  the  carnivora.  In  the  course  of 
mastication  the  food  is  ground  into  small  particles,  thus  facilitating 
the  action  of  the  saliva.  The  watery  constituents  of  the  secretion  render 
the  food  of  softer  consistence,  and  the  mucin  provides  it  with  a  slippery 
coating,  so  that  swallowing  of  the  bolus  is  greatly  facilitated.  The  saliva, 
further,  dissolves  certain  mineral  substances  that  are  soluble  in  weak 
alkaline  solutions,  feebly  emulsifies  fats,  but  exerts  no  action  upon 
proteids,  except  that  of  maceration.  The  active  constituent  of  the  saliva 
is  the  ptyalin,  which  is  a  diastatic  enzyme  acting  on  the  starches.  In  the 
chemical  transformation  that  ensues,  the  ptyalin  initiates  hydrolysis 
of  the  complex  starch  molecule,  so  that  it  is  reduced  to  simpler  con- 
stituents. The  first  action  of  the  ptyalin  is  to  render  the  viscid  starch 
more  fluid,  and  then  to  convert  it  into  a  variable  mixture  of  dextrin, 
maltose,  and  isomaltose.  The  conversion  of  the  starch  into  a  thin, 
watery  fluid  takes  place  very  rapidly,  only  a  few  seconds  being  required 
to  bring  about  the  result.  Thorough  mastication  of  the  food  and  its 
admixture  with  the  saliva  are  very  important,  for  the  amount  of  salivary 
digestion  is  quite  considerable,  and  renders  the  food  to  that  extent  more 
suitable  for  the  action  of  the  gastric  juice.  In  fact,  the  conversion  of 
the  starches  into  dextrin  and  sugars  continues  for  some  time  after  the 
food  enters  the  stomach,  even  when  there  is  considerable  acidity  of  the 
gastric  contents. 

A  diminution  of  the  amount  of  saliva,  then,  materially  interferes  with 
the  rapidity  of  the  digestive  process,  and,  moreover,  interferes  with 
mastication,  deglutition,  and  speaking.  It  occurs  in  many  febrile  condi- 
tions, notably  in  typhoid  fever  and  pneumonia,  as  Hosier  has  pointed 
out,  and  in  all  conditions  in  which  there  is  an  increased  elimination  of 
water  from  the  system,  as,  for  example,  diabetes,  cholera,  and  chronic 
interstitial  nephritis.  Some  few  cases  are  definitely  nervous  in  origin, 
e.  g.,  those  due  to  certain  paralyses  of  the  facial  nerve  involving  the  chorda 
tympani.  As  is  well  known,  in  many  emotional  states,  such  as  fear  and 
"nervousness,"  the  mouth  becomes  for  the  time  being  quite  dry  (xeros- 
tomia). Dryness  of  the  mouth  frequently  leads  to  the  retention  within 
the  buccal  cavity  of  small  particles  of  food  which  decompose  and  lead  to 
a  great  multiplication  of  bacteria.  In  this  way  irritation  and  eventually 
inflammation  are  not  infrequently  produced.  Not  only  do  the  bacteria 
produce  their  local  effects  in  the  mouth,  but  they  may  be  introduced  into 


PTYALISM  355 

the  stomach  in. such  numbers  that  this  organ  is  unable  to  cope  with  them, 
and  serious  disturbance  may  be  set  up.  The  tongue,  lips,  and  gums  are 
dry,  coated  with  decomposing  secretion,  desquamated  cells,  and  decaying 
food  (sordes),  and  may  then  become  fissured  and  inflamed. 

An  increased  flow  of  saliva  (ptyalism)  may  be  produced  experimentally 
by  irritation  of  the  chorda  tympani  nerve  or  by  cutting  the  salivary 
nerves.  The  nervous  element  is  of  considerable  importance,  for  we  find 
a  notable  increase  in  the  saliva  in  cases  of  bulbar  paralysis,  in  which 
there  is  a  degeneration  of  the  ganglion  cells  of  the  medulla.  This  has 
been  considered  by  some  authorities  as  being  analogous  to  the  paralytic 
secretion  that  results  when  the  salivary  nerves  are  cut.  Krehl1  is  in- 
clined to  think,  however,  that  this  is  not  the  correct  explanation,  but  that 
the  phenomenon  is  an  irritative  one  due  to  the  degeneration  of  the  cells 
in  the  medulla.  Very  susceptible  people  will  also  manifest  salivation 
at  the  sight  or  thought  of  food,  or  even  when  they  think  they  have  taken 
calomel.  Other  cases  are  undoubtedly  reflex,  as,  for  example,  those 
associated  with  ulcer  of  the  stomach,  pregnancy,  and  trifacial  neuralgia. 

Ptyalism  is  also  met  with  in  all  forms  of  stomatitis  and  in  mercurial 
poisoning.  In  these  cases  not  only  does  there  seem  to  be  a  reflex 
stimulation  of  the  salivary  and  mucous  glands,  but  there  are  local  changes 
in  the  mucous  membrane,  in  the  form  of  degeneration  of  cells,  hyperemia, 
and  inflammatory  exudation. 

Ptyalism  must  be  carefully  distinguished  from  the  apparent  increase 
in  the  salivary  secretion  which  is  present  in  some  forms  of  paralysis  of 
the  muscles  of  the  mouth,  in  which  the  patient  is  unable  to  swallow  and 
the  saliva  simply  dribbles  out. 

In  cases  of  ptyalism  the  character  of  the  secretion  may  be  altered. 
Thus,  in  the  reflex  varieties,  the  secretion,  while  it  is  increased  in  amount, 
is  deficient  in  solids.  In  others,  the  amount  of  ptyalin  may  be  reduced. 
( )ccasionally,  as  has  been  noted  in  diabetes,  fevers,  and  certain  dyspeptic 
conditions,  the  reaction  is  acid  instead  of  alkaline.  This  is  due  to  the 
activity  of  microorganisms,  whereby  lactic  acid  is  produced.  The 
alteration  in  the  characters  of  the  saliva  occasionally  leads  to  curious 
results,  such  as  the  formation  of  calculi  in  the  ducts  of  the  salivary 
glands.  Probably,  infection  and  inflammation  are  here  the  primary 
causes,  and  lead  to  abnormal  chemical  reactions. 

The  swallowing  of  large  amounts  of  secretion  may  be  injurious, 
especially  when  alkaline,  by  diluting  the  gastric  juice  and  neutralizing 
its  acidity.  Where  multitudes  of  septic  microorganisms  have  been 
swallowed,  serious  changes  in  the  stomach  may  occur,  as  Hunter  has 
pointed  out,  in  the  form  of  catarrh  and  atrophy  of  the  glands. 

The  Gastric  Juice. — The  primary  function  of  the  stomach  appears  to 
be  to  serve  as  a  storehouse  for  the  food  ingested.  This  is  proved  by  the 
fact  that  the  stomach  has  been  removed  in  dogs  and  digestion  goes  on 
perfectly,  provided  that  food  is  supplied  in  small  particles  and  frequently. 
After  a  time  the  bowel  distends  at  the  site  formerly  occupied  by  the 

1  Principles  of  Clinical  Pathology,  1907:  248:  Lippincott. 


356     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

stomach,  to  accommodate  increased  quantities  of  food.  This  view  is 
corroborated  by  the  results  in  the  few  cases  where  the  stomach  has  been 
successfully  removed  in  man.  An  important,  though  secondary,  duty  of 
the  stomach  is  to  thoroughly  mix  the  food  with  the  gastric  juice  and  pass 
it  on  to  the  intestines  in  quantities  which  they  can  comfortably  deal  with. 
Thus,  the  delicate  mucosa  of  the  bowel  is  protected  from  contact  with, 
and  possible  injury  from,  coarse  masses  of  food.  Only  a  small  propor- 
tion of  the  food  ingested  is  absorbed  in  the  stomach.  Most  of  it,  and 
nearly  all  the  water,  is  passed  on  into  the  duodenum. 

The  ferments  of  the  gastric  secretion  are  pepsin  and  rennin.  Some 
authorities  believe,  also,  in  the  existence  of  a  fat-splitting  ferment,  but 
others  dispute  this.  Pepsin  acts  only  in  an  acid  medium,  and  this  is 
supplied  by  the  production  of  hydrochloric  acid. 

It  is  extremely  difficult  to  study  the  phenomena  of  digestion  in  the 
human  subject,  inasmuch  as  it  is  rarely  possible  to  obtain  pure  gastric 
juice  unmixed  with  food.  Pawlow's  work  on  dogs1  has,  however, 
thrown  a  flood  of  light  on  the  problem,  and  many  of  his  conclusions 
have  been  substantiated  by  later  observations  on  human  beings,  who  were 
the  subjects  of  gastric  and  oesophageal  fistulse.  The  normal  stimulus 
to  the  secretion  of  gastric  fluid  in  the  dog  is  the  appetite,  but  it  is  probable 
that  this  element  has  a  much  slighter  influence  in  man.  Here,  the  chief 
factor  is  the  direct  stimulation  of  the  gastric  mucous  membrane  by  the 
presence  of  food  in  the  stomach.  As  the  French  proverb  has  it,  "L'appe- 
tite  vient  en  mangeant."  Pawlow  found  that  beef  extracts  and  small 
quantities  of  alcohol  produced  the  same  effect,  but  that  alkalies  inhibited 
the  secretion.  The  stimulation  of  the  special  nerves  of  taste  and  smell 
also  seems  to  play  an  important  role  in  this  connection.  The  mere 
act  of  chewing  probably  produces  no  effect.  Nevertheless,  there  is  a 
close  correlation  between  the  appetite  and  the  gastric  secretion.  Under 
ordinary  circumstances,  the  majority  of  people  can  digest  the  food  they 
have  a  fancy  for.  The  hungry  man  is  rarely  a  dyspeptic.  Shakespeare, 
then,  had  an  insight  into  a  deep  physiological  truth  when  he  makes 
Macbeth  exclaim,  "Now  good  digestion  wait  on  appetite,  and  health, 
on  both."  The  influence  of  the  central  nervous  system  is  here  again 
apparent.  The  benumbing  of  the  sensorium  in  fevers  lessens  the 
demand  for  food,  in  spite  of  the  fact  that  the  tissues  are  being  rapidly 
burned  up  and  the  cells  have  every  need  for  increased  nourishment. 
The  emotions  also  play  an  important  role.  Anger  lessens  the  flow  of 
gastric  juice.  Worry,  anxiety,  hysterical  and  neurasthenic  conditions 
often  cause  a  loss  of  appetite  (anorexia  nervosd). 

When  the  food  is  swallowed,  after  the  preliminary  mastication  and 
insalivation,  there  is  a  perceptible  interval  before  the  gastric  juice  begins 
to  be  secreted.  During  this  period,  which  averages  from  twenty  to 
thirty  minutes,  depending  upon  the  nature  of  the  meal,  the  thoroughness 
of  the  insalivation,  and  the  vigor  of  the  secretion  of  the  hydrochoric  acid, 

1  Pawlow,  The  Work  of  the  Digestive  Glands.  Eng.  edit.,  C.  Griffin  &  Co.,  London, 
1902. 


THE  GASTRIC  JUICE  357 

the  conversion  of  the  starches  into  dextrins  and  sugars  proceeds  rapidly, 
for  ptyalin  is  even  more  efl'eetive  in  a  neutral  solution  than  in  an  alkaline 
one.  With  the  appearance  of  hydrochloric  acid,  however,  the  alkalinity 
gradually  gives  way  to  acidity  and  ptyalin  digestion  stops. 

The  further  steps  in  the  process  of  digestion  may  be  briefly  stated  to 
be  as  follows:  The  hydrochloric  acid  provides  a  suitable  medium  for 
the  operation  of  the  peptic  ferment,  which  it  thus  assists  in  hydrolyzing 
the  proteins.  It  also  converts  cane  sugar  into  dextrose  and  levulose.  It 
inhibits  the  growth  of  microorganisms  and  thus  prevents  or  lessens  the 
acetic  and  lactic  fermentation  of  the  carbohydrates. 

Rennin  exerts  its  special  action  on  milk,  coagulating  it  and  thus  pro- 
moting its  digestion  by  causing  it  to  be  retained  for  a  longer  time  in  the 
stomach. 

The  pepsin  acts  on  proteins,  and,  assisted  by  the  hydrochloric  acid, 
converts  them  into  acid  albumin  and  eventually  into  albumoses.  Accord- 
ing to  Neumeister,  there  are  two  sets  of  albumoses,  proto-albumose 
and  hetero-albumose  (primary  albumoses),  which  are  gradually  con- 
verted into  deutero-albumoses  (secondary  albumoses).  Finally,  some 
of  the  deutero-albumoses  are  transformed  into  peptones. 

Hammersten  has  noted  the  presence  in  the  gastric  mucous  membrane 
of  an  oxidizing  ferment  (oxidase)  which  converts  milk  sugar  into  lactic 
acid. 

At  the  height  of  the  digestive  process  the  hydrochloric  acid  is  present 
in  various  conditions.  Some  of  it  is  free  and  uncombined;  some  has 
united  with  the  inorganic  bases  or  basic  salts  of  the  food,  and  has  broken 
up  salts  of  the  weaker  acids;  some,  again,  has  combined  with  organic 
basic  compounds,  of  which  the  proteids  are  the  most  important.  Toward 
the  close  of  gastric  digestion,  the  stomach  contains  a  variable  mixture 
of  starches,  dextrin,  sugars,  peptones,  and  unconverted  albumoses. 

The  length  of  time  that  the  food  remains  in  the  stomach  varies  con- 
siderably in  different  cases.  The  character  of  the  food,  the  state  of 
health,  and  the  type  of  animal  have  much  to  do  with  it.  Under  ordinary 
circumstances,  the  stomach  in  the  herbivora  is  never  empty.  In  the 
carnivora  the  food  may  pass  on  within  one  or  two  hours.  In  man,  with 
his  mixed  diet,  gastric  digestion  takes  from  four  to  six  hours,  fatty 
materials  being  retained  longest  in  the  stomach,  and  carbohydrates 
the  shortest.  Under  pathological  conditions,  however,  food  may  be 
retained  very  much  longer  than  this.  In  chronic  dilatation  of  the  stomach 
from  pyloric  carcinoma,  for  example,  the  stomach  may  be  found  to  con- 
tain food  that  was  taken  days  before.  In  such  cases  the  power  of  gastric 
digestion  is  much  impaired,  and  the  retained  peptones  and  albumoses 
cause  direct  irritation  of  the  mucous  membrane. 

The  subject  of  disorder  of  the  gastric  secretion  is  an  extremely  difficult 
one  to  deal  with.  Our  knowledge  of  the  normal  phenomena  of  diges- 
tion is  still  imperfect,  for  it  is  gathered  from  observations  carried  out  on 
experimental  animals  and  but  rarely  on  human  beings,  in  all  cases, 
under  conditions  which  present  wide  deviations  from  the  normal.  Our 
ideas,  therefore,  are  based  on  insufficient  data  and,  not  infrequently, 


358     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

on  inference  rather  than  fact.  This  being  the  case,  it  is  not  surprising 
that  our  discussion  of  the  various  disturbances  of  secretion  will  be  quite 
inadequate. 

Disorders  of  gastric  digestion  involve  the  quantity  or  the  quality  of 
the  gastric  juice,  or  both.  Thus,  the  total  amount  of  the  secretion  may 
be  less  than  normal  or  it  may  even  be  absent  altogether.  Or,  again,  it 
may  be  increased.  The  qualitative  changes  have  to  do  with  the  pro- 
portions of  hydrochloric  acid  and  the  ferments.  As  a  rule,  the  zymogens 
of  pepsin  and  rennin  continue  to  be  produced,  even  if  the  secretion  of 
the  acid  has  partially  or  completely  ceased.  But  in  advanced  organic 
disease  of  the  stomach  both  acids  and  ferments  may  be  lacking. 

As  we  have  seen,  the  secretion  of  the  gastric  fluid  is  chiefly  reflex, 
the  stimuli,  which  are  rather  complex  in  nature,  being  the  presence  of 
food  in  the  stomach,  the  excitation  of  the  nerves  of  taste  and  smell,  and 
the  appetite.  Conceivably,  then,  a  defect  in  the  necessary  stimuli  would 
result  in  a  diminished  secretion.  Thus,  organic  or  functional  dis- 
orders of  the  special  nerves  of  taste  and  smell,  a  benumbing  of  the 
cerebral  centres,  certain  emotions,  like  anger,  insufficient  or  uncongenial 
food,  would  all  play  a  part.  In  this  category  may  be  placed  most  of  the 
acute  infective  diseases  associated  with  high  temperature,  in  which 
anorexia  is  a  well-marked  feature;  delirium  and  coma;  tumors  at  the 
base  of  the  brain;  hysteria,  neurasthenia,  and  melancholia.  Deficient 
secretion  (hyposecretion)  and  absent  secretion  (achylia  gastrica)  are 
known  to  occur  in  neurotic  individuals.  The  same  thing  has  been 
observed  in  certain  cases  of  tabes  dorsalis.  Here,  possibly,  the  cause 
is  to  be  referred  to  interruption  of  the  reflex  arc. 

In  a  second  set  of  cases,  organic  changes,  involving  more  or  less  de- 
struction of  the  secretory  glands,  may  be  demonstrated.  Thus,  extensive 
carcinoma  of  the  stomach,  amyloid  disease,  atrophy  of  the  mucosa, 
whether  senile  or  that  form  resulting  from  chronic  inflammation,  may  be 
cited  as  examples  in  point. 

In  still  another  series  of  cases  there  is  some  serious  systemic  disease. 
Thus,  profound  anemias  result  in  diminished  gastric  secretion.  Or 
there  may  be  an  excessive  output  of  fluid  from  the  body,  as  in  diabetes 
and  chronic  interstitial  nephritis.  Or,  again,  a  deficiency  of  chlorides 
is  the  cause  at  work. 

When  the  amount  of  hydrochloric  acid  in  the  gastric  juice  is  diminished 
or  absent,  we  speak  of  subacidity  (hypochlorhydria)  and  anacidity 
(achlorhydria)  respectively.  The  total  quantity  of  acid  produced  ap- 
pears to  bear  some  relation  to  the  quantity  and  character  of  the  food 
ingested.  How  much  acid  should  be  found  normally  appears  to  be  still 
under  discussion,  for  different  authorities  give  different  statements 
in  this  regard.  According  to  Krehl,1  the  secretion  probably  continues 
until  the  free  and  combined  hydrochloric  acid  in  the  gastric  contents 
amounts  to  about  0.2  to  0.3  per  cent.  Bickel2  gives  considerably  higher 

1  The  Principles  of  Clinical  Pathology,  1907:  254,  Lippincott. 

2  Kongr.  f.  in.  Medizin,  1906. 


THE  GASTRIC  JUICE  359 

figures.  He  found,  in  individuals  the  subjects  of  oesophageal  and  gastric 
fistulse,  that  the  pure  gastric  juice  contained  from  0.35  to  0.5  per  cent. 
It  is  important  to  remember  in  this  connection  that  our  ordinary  clinical 
analyses  of  the  stomach  contents  are  made  on  mixtures  of  food  and 
gastric  juice.  Consequently  we  are  apt  to  underestimate  the  extent  of 
acid  production.  An  estimation  of  the  free  hydrochloric  acid  is  of  little 
or  no  practical  value.  We  can  only  obtain  accurate  information  by 
determining  the  total  quantity  of  acid,  both  free  and  combined.  To 
do  this,  we  have  to  find  out  the  total  quantity  of  chlorides  in  the  gastric 
contents  and  subtract  from  this  the  quantity  of  chlorides  contained  in 
the  food.  When  the  hydrochloric  acid  in  the  gastric  contents  is  found 
to  be  diminished,  we  have  to  exercise  some  care  in  the  interpretation  of 
our  results,  for  the  condition  may  be  accounted  for  in  two  ways,  either 
there  is  a  diminished  secretion  of  the  acid,  or  else  it  is  neutralized  in  some 
abnormal  way  after  its  secretion. 

In  cases  where  the  gastric  secretion  is  inhibited,  either  partially  or 
completely,  the  amount  of  hydrochloric  acid  will,  of  course,  be  propor- 
tionately diminished.  The  ferments  will  naturally  be  diminished  in 
quantity  as  well.  But  instances  are  not  infrequent  where  the  secretion 
of  the  acid  alone  is  defective. 

Free  hydrochloric  acid  may  be  absent  in  a  variety  of  conditions. 
For  example,  certain  acute  functional  and  organic  disturbances  of  the 
stomach,  notably,  the  acute  infectious  diseases,  may  be  mentioned. 
Perhaps  more  often,  anacidity  is  found  in  chronic  conditions  affecting 
the  integrity  of  the  mucous  membrane,  such  as  atrophy,  carcinoma^ 
and  amyloid  disease.  Curiously  enough,  diseases  in  parts  external 
to  the  stomach  may  produce  the  same  result.  Pernicious  anemia,  ab- 
dominal carcinoma,  advanced  tuberculosis,  and  cachexia  are  cases  in 
point.  The  exact  condition  of  things  has  been  more  carefully  studied 
in  connection  with  gastric  carcinoma  than  in  other  diseases.  Where 
a  large  amount  of  the  secreting  surface  is  involved  in  the  newgrowth, 
especially  where  there  is  marked  ulceration,  it  would  not  be  surprising 
to  find  a  total  defect  or  a  diminution  in  the  amount  of  acid  secreted. 
In  some  instances,  however,  it  is  unquestionable  that  hydrochloric  acid 
is  secreted,  though  it  is  not  found  in  the  free  state,  for  the  total  amount 
of  the  combined  chlorides  may  equal  or  exceed  the  normal.  Apparently, 
the  carcinoma  produces  substances  that  have  the  power  of  neutralizing 
the  acid  produced.  These  are  presumably  of  the  nature  of  enzymes, 
inasmuch  as  they  are  destroyed  by  heat.1  Moore  has  laid  down  that 
in  all  carcinomatous  states  there  is  a  reduction  of  acid;  this  needs  con- 
firmation. 

A  deficiency  in  the  amount  of  hydrochloric  acid  secreted  will  of 
necessity  inhibit  to  a  corresponding  extent  the  activity  of  the  pepsin, 
and  so  delay  gastric  digestion.  Provided,  however,  that  the  motility 
of  the  stomach  be  not  impaired,  the  nutrition  of  the  individual  thus 
affected  need  not  be  seriously  disturbed.  With  a  suitable  dietary,  the 

1  Emerson,  Arch.  f.  klin.  Med.,  72:  426. 


360     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

intestine  may  be  able  to  compensate  the  inefficiency  of  the  stomach. 
It  is,  further,  worthy  of  note  that  carcinomas  of  the  stomach  are  able 
to  produce  ferments  competent  to  digest  proteids  more  quickly  even 
than  the  normal  gastric  fluid. 

One  of  the  most  important  results  of  diminished  gastric  acidity  is 
the  multiplication  of  bacteria  within  the  stomach.  It  is  well  established 
that  the  stomach,  under  normal  conditions,  contains  numerous  micro- 
organisms derived  from  the  ingested  food  and  external  air.  Provided 
that  gastric  motility  is  unimpaired  and  the  food  is  passed  regularly  along 
into  the  intestines,  no  great  multiplication  of  these  organisms  can  occur. 
Should,  however,  stagnation  of  the  stomach  contents  be  present,  more 
or  less  growth  takes  place  and  fermentative  processes  are  set  up.  Thus, 
for  example,  sugar  is  converted  into  alcohol  and  carbon  dioxide;  alcohol, 
into  acetic  acid;  dextrose,  into  lactic  acid,  butyric  acid,  hydrogen,  and 
carbon  dioxide.  Gases  are  set  free,  such  as  carbon  dioxide,  hydrogen, 
and  methane,  which,  together  with  the  air  that  has  been  swallowed,  pro- 
duce flatulency  and  distension  of  the  stomach.  These  fermentative 
processes,  however,  are  kept  within  bounds  by  the  presence  of  the  hydro- 
chloric acid,  which  has  decidedly  antiseptic  properties.  A  0.2  per  cent, 
degree  of  acidity  in  a  culture  medium  will,  in  time,  destroy  many  bacteria, 
though  some,  and  especially  spores,  are  not  greatly  affected.  It  should 
be  remembered,  however,  that  the  conditions  in  the  stomach  are  by  no 
means  so  favorable  for  this  inhibitory  action.  Some  of  the  hydrochloric 
acid  secreted  is  neutralized  by  alkalies,  or  enters  into  combination 
with  the  proteids,  which  combinations  are  much  less  effective  than  free 
acid.  Moreover,  many  portions  of  the  food  do  not  come  in  contact 
with  the  gastric  juice  at  all,  for  they  may  be  passed  rapidly  along  into 
the  intestine  or  lie  in  the  centre  of  large  masses.  Consequently,  the 
antiseptic  powers  of  the  gastric  juice  are  somewhat  limited,  and  many 
active  microorganisms  gain  entrance  to  the  bowel. 

In  cases  of  subacidity  and  anacidity  an  opportunity  is  afforded  for 
the  enormous  increase  of  bacteria,  both  in  numbers  and  variety,  with  a 
concomitant  increase  in  the  amount  of  fermentation.  Under  these 
circumstances,  processes,  similar  to  those  just  referred  to,  are  set  in 
operation,  but  there  is  a  special  tendency  to  the  formation  of  lactic, 
butyric,  and  other  volatile  organic  acids.  Lactic  acid  fermentation  is 
a  chief  and  characteristic  feature  of  anacidity  with  stagnation  of  the 
stomach  contents.  In  fact,  the  lactic  acid  may  be  so  abundant  as  to 
restrain  the  development  of  other  bacteria  that  ordinarily  would  produce 
their  own  peculiar  form  of  fermentation.  In  such  cases,  the  Oppler-Boas 
bacillus  is  usually  present  in  the  stomach  in  enormous  numbers  and  is 
the  cause  of  the  lactic  acid  production.  In  marked  cases  of  anacidity 
putrefaction  of  the  proteins  may  occur  as  well.  In  some  few  instances 
fermentation  occurs  in  the  stomach  in  the  absence  of  subacidity  and 
impaired  muscular  power.  Possibly,  here  we  have  to  do  with  the  inges- 
tion  of  excessive  amounts  of  fermentable  material  together  with  agents 
that  are  competent  to  produce  fermentation. 

It  should  be  pointed  out  that,  in  the  present  state  of  our  knowledge, 


THE  GASTRIC  JUICE  361 

there  is  no  pathognomonic  relationship  between  any  one  kind  of  bacterial 
decomposition  and  any  particular  clinical  state.  Our  findings  have  to 
be  interpreted  in  such  cases  in  the  most  general  way.  The  chief  factors 
are,  the  character  of  the  food,  impaired  gastric  motility,  the  amount 
of  hydrochloric  acid  secreted,  and  the  multiplication  of  bacteria.  On 
the  correlation  of  these  depend  the  result. 

Abnormal  fermentation  in  the  stomach  is  injurious  in  a  variety  of 
ways.  The  mucous  membrane  may  be  irritated  and  inflamed,  leading 
to  anorexia,  pain,  regurgitation,  and  vomiting,  and,  possibly,  spasm 
of  the  pylorus.  Gases  accumulate  and  produce  distension,  flatulency, 
and  belching.  Toxic  substances  are  formed,  which  may  be  absorbed 
and  lead  to  systemic  disorder.  The  condition  is  not  without  its  effect 
on  the  bowel  as  well,  for  diminished  acidity  in  the  stomach  usually 
leads  to  increased  putrefaction  in  the  intestine. 

Theoretically  speaking,  we  are  able  to  make  a  distinction  between 
increased  acidity  of  the  gastric  secretion  (hyperchlorhydria)  and  hyper- 
secretion.  In  the  former  there  is  a  relative  increase  in  the  amount  of 
acid  produced;  in  the  latter  there  is  an  absolute  increase  proportionate 
to  the  increase  of  the  gastric  secretion  as  a  whole.  In  view  of  the  gaps 
in  our  knowledge,  however,  as  to  the  normal  behavior  of  the  stomach 
in  digesting  varying  quantities  and  kinds  of  food,  it  is  hardly  possible 
to  make  this  distinction  in  practice.  As  a  result  of  his  experiments, 
above  referred  to,  Bickel,  indeed,  concludes  that  what  is  usually  designated 
by  clinicians  as  "  hyperacidity"  is  in  reality  hypersecretion.  The 
effect  of  hypersecretion  is  to  raise  the  percentage  of  hydrochloric  acid 
in  the  mixture  of  gastric  juice  and  food  ordinarily  submitted  to  examina- 
tion, and  the  result  may,  therefore,  be  wrongly  interpreted.  The  total 
amount  of  acid  in  the  stomach  contents  in  so-called  "hyperacidity," 
as  a  matter  of  fact,  does  not  exceed  that  present  in  normal  gastric 
juice.  Under  the  circumstances,  we  cannot,  perhaps,  do  better  than 
use  the  terms  hyperacidity  and  hypersecretion  in  the  ordinary  sense  in 
which  they  are  employed  by  certain  clinicians.  Hyperacidity  may,  then, 
be  taken  to  mean  an  increased  secretion  of  the  hydrochloric  acid  with 
the  gastric  juice,  occurring  during  digestion.  Hypersecretion  is  an 
excessive  secretion  of  the  gastric  fluid,  usually  hyperacid,  occurring 
not  only  during  digestion  but  in  the  intervals  also.  Both  conditions 
are  to  be  regarded  as  symptoms  rather  that  actual  disease  entities. 

The  diagnosis  of  hyperacidity  depends  on  the  detection  in  the  stomach 
contents  of  an  increased  amount  of  hydrochloric  acid.  The  chief  causes 
of  the  condition  are  dietetic  errors,  overwork,  worry,  the  various  neuroses, 
and  the  abuse  of  tobacco.  The  condition  is  found  also  in  cases  of  ulcer 
of  the  stomach.  Disease  elsewhere  in  the  body,  such  as  chlorosis,  chole- 
lithiasis, and  renal  calculus,  may  be  at  work  in  some  cases. 

Hypersecretion  may  be  a  transient  condition,  may  recur  periodically, 
or,  again,  may  be  continuous.  Transient  and  periodical  hypersecretion 
is  met  with  in  certain  nervous  affections,  such  as  locomotor  ataxia, 
hysteria,  and  neurasthenia,  or  it  may  occur  more  or  less  independently 
(gastroxynsis  of  Rossbach).  It  is  apparently  due  to  irritability  of  the 


362     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

gastric  mucous  membrane,  of  its  secretory  nerves,  or,  in  some  cases, 
to  stimulation  of  the  cerebral  centres. 

Continuous  hypersecretion  (Reichmann's  disease)  is  found  particularly 
in  young  neurotic  individiuals.  Dietetic  errors,  emotions,  and  motor 
insufficiency  are  the  chief  causes.  The  condition  is  met  with  also  in 
association  with  certain  forms  of  chronic  gastritis.  An  important  factor 
in  the  causation  is  dilatation  of  the  stomach,  with  its  concomitant  muscular 
insufficiency,  whether  this  be  due  to  a  primary  paresis  of  the  stomach 
wall  or  to  the  many  forms  of  obstruction  to  the  evacuation  of  the  gastric 
contents.  In  the  chronic  forms  the  hypersecretion  is  probably  to  be 
attributed  to  the  stimulation  of  the  mucous  membrane  by  the  retained 
food.  In  the  acute  forms  of  gastric  dilatation  a  relatively  enormous 
amount  of  fluid  may  be  secreted.  As  much  as  several  quarts  have  been 
removed  in  some  instances  with  the  stomach  tube.  Here,  as  in  the  earlier 
stages  vomiting  is  persistent  and  no  food  can  be  retained,  it  is  impossible 
that  the  stimulus  can  be  stagnated  food.  Unfortunately,  very  few  com- 
plete studies  of  the  gastric  secretion  have  been  made  in  these  cases,  so 
that  we  are  somewhat  in  the  dark.  Hydrochloric  acid  has  been  found 
present  in  some  cases,  but  not  invariably.  It  would  seem  reasonable, 
however  to  think  that  the  gastric  fluid  in  acute  dilatation  is  not  a  true 
secretion.  Other  factors  may  enter  into  the  question.  Owing  to  the 
stretching  of  the  stomach  wall,  the  vessels  are  elongated  and  thin-walled, 
and  lack  tone;  furthermore,  there  is  the  depreciating  effect  of  the  toxins 
present  within  the  stomach.  Consequently,  it  would  not  be  surprising 
if  the  bloodvessels  should  become  more  permeable  and  allow  a  con- 
siderable quantity  of  fluid  to  exude  and  enter  the  cavity.  Organic 
obstruction,  too,  promotes  the  condition  by  preventing  the  normal 
discharge  of  the  stomach  secretions  into  the  bowel.  In  some  cases,  also, 
in  which  obstruction  of  the  duodenum  below  the  bile  papilla  has  been 
demonstrated,  the  gastric  fluid  has  contained  bile  and  a  diastatic  ferment. 

We  pass  on  now  to  the  consideration  of  the  subject  of  intestinal  diges- 
tion. 

The  Intestinal  and  Related  Secretions. — The  strongly  acid  chyme, 
when  it  leaves  the  stomach,-  passes  into  the  duodenum,  where  it  meets 
with  an  alkaline  medium,  composed  of  pancreatic  secretion,  bile,  and 
succus  entericus.  The  hydrochloric  acid  is  neutralized  and  the  chyme 
is  thus  prepared  for  the  action  of  the  pancreatic  ferments,  which  can 
act  only  in  the  presence  of  alkalies. 

The  old  view  that  an  acid  reaction  of  the  chyme  persists  for  a  considera- 
able  distance  down  the  intestine,  and  that  the  acid  is  only  gradually 
neutralized,  is  almost  certainly  erroneous.  Mere  traces  of  hydrochloric 
acid  are  enough  to  destroy  the  activity  of  the  pancreatic  ferments,  which 
are  only  poured  out  in  the  duodenum.  Consequently,  it  is  impossible 
to  understand  how  pancreatic  digestion  could  be  carried  on  under  such 
circumstances.  As  a  matter  of  fact,  only  small  amounts  of  food  pass 
through  the  pylorus  at  any  one  time,  and  they  come  in  contact  with 
a  much  greater  bulk  of  alkaline  secretion  in  the  duodenum,  so  that 
the  acid  is  immediately  neutralized.  The  chief  digestive  agents  in  the 


THE  BILE  303 

intestines  are  the  pancreatic  secretion  and  the  succus  entericus.  Some 
authorities  describe  also,  another,  maltase,  which  converts  maltose  into 
dextrose. 

The  Succus  Entericus. — Little  is  known  about  the  succus  entericus.  It 
contains,  however,  a  ferment  which  converts  cane  sugar  and  maltose 
into  dextrose  and  levulose,  hence  called  invertin. 

The  Pancreatic  Secretion.— By  far  the  most  important  part  is  played 
by  the  pancreatic  secretion.  This  contains  three  ferments:  amylopsin, 
which  converts  the  carbohydrates;  trypsin,  which  peptonizes  proteins; 
and  steapsin,  which  hydrolyzes  fats.  Amylopsin  in  every  way  resembles 
ptyalin  in  its  action  except  that  it  is  more  powerful. 

Steapsin  is  the  only  ferment  in  the  body  that  has  a  specific  chemical 
action  on  fat.  It  is  true  that  some  observers  have  described  a  fat-splitting 
ferment  in  the  gastric  secretion,  but  their  conclusions  are  by  no  means 
as  yet  generally  accepted.  Steapsin  acts  by  breaking  down  neutral  fats 
into  fatty  acids  and  glycerin.  It  has  been  usually  held  that  only  a  small 
portion  of  the  fat  undergoes  this  decomposition,  and  that  the  fatty  acids 
produced  combine  with  the  alkalies  of  the  intestinal  contents  to  form 
soaps,  which  soaps  emulsify  the  unconverted  fat.  The  chemically 
unaltered  fat  is  then  absorbed  by  the  columnar  cells  of  the  intestinal 
villi.  There  is  considerable  evidence  for  thinking,  however,  in  view  of 
later  experimental  work,  that  this  view  is  incorrect,  and  that  practically 
all  of  the  fat  (84  per  cent.)  is  hydrolyzed  into  fatty  acids  and  glycerin 
and  in  this  soluble  form  absorbed. 

The  action  of  trypsin  is  similar  to  that  of  pepsin,  but  is  more  rapid 
and  more  complete.  The  proteins  are  first  converted  into  alkali- 
albumin,  and  this  into  deutero-albumoses.  Whether  primary  albumoses 
are  formed  is  questionable.  If  they  are,  they  appear  to  be  very  unstable 
and  undergo  rapid  conversion.  The  deutero-albumoses  are  transformed 
into  peptone  and  the  latter,  for  the  most  part,  into  amido-acids  and 
organic  nitrogenous  bases.  Only  about  half  of  the  peptone  present 
is  converted,  the  more  stable  moiety  remaining  being  known  as  anti- 
peptone.  Those  substances  that  are  partially  peptonized  in  the  stomach 
will,  of  course,  only  require  to  undergo  the  later  stages  of  tryptic  digestion. 

The  Bile. — The  bile  has  no  zymotic  action  on  foodstuffs,  but  is  valuable 
as  an  adjuvant  to  the  pancreatic  secretion,  in  that  it  renders  more  soluble 
substances  which  would  otherwise  be  quite  insoluble  in  water.  It  can, 
for  example,  dissolve  to  some  extent  lecithin  and  cholesterin,  and  thus 
aids  in  their  elimination  from  the  body.  It  also  aids  in  the  solution  of 
the  soaps  and  free  fatty  acids.  Its  antiseptic  properties,  referred  to  by 
some,  are  decided,  though  not  powerful. 

The  purpose  of  the  milk-coagulating  ferment,  said  by  certain  authori- 
ties to  be  present,  is  not  clear,  any  milk  in  the  food  being  acted  upon 
by  the  rennin  in  the  stomach. 

The  effects  of  a  deficient  secretion  of  the  pancreatic  ferments  cannot 
be  stated  precisely.  One  would  expect,  and  it  undoubtedly  is  a  fact, 
that  undigested  proteid  matter  and  unconverted  fats  are  to  be  found  in 
the  stools  in  increased  amounts  in  certain  cases  of  pancreatic  disease. 


364     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

Yet  clinical  studies  on  patients  having  extensive  degeneration  of  the 
pancreas,  and  the  experimental  extirpation  of  the  pancreas  in  dogs, 
have  given  contradictory  results  in  the  experience  of  different  observers. 
Some  have  found  that  the  absorption  of  proteins  and  fats  in  the  bowel 
were  considerably  diminished,  while  others  did  not.  In  the  same  way, 
some  have  noted  a  diminished  cleavage  of  fats  in  the  bowel,  while  others 
did  not.  It  is  probable  that  these  discrepancies  in  the  results  of  clinical 
studies  are  to  be  explained  in  this  way.  Unless  the  pancreas  were 
extensively  damaged  and  its  secretion  lessened  almost  to  the  vanishing 
point,  not  much  alteration  in  the  normal  processes  of  digestion  need 
result.  For  it  is  clear  that  the  pancreatic  secretion  is  normally  secreted 
in  an  amount  much  greater  than  is  absolutely  necessary  to  digest  any 
meal  that  is  ordinarily  taken.  The  contents  of  the  small  intestine 
at  a  point  just  above  the  ileocecal  valve  have  been  shown  to  be  exceedingly 
rich  in  amylolytic  and  proteolytic  ferments,  and  it  is  altogether  probable 
that  these  are  not  absorbed  but  destroyed  in  the  large  bowel.  An 
extreme  amount  of  degeneration  would  be  requisite,  then,  to  produce 
notable  changes  in  the  constitution  of  the  feces.  In  a  similar  way,  the 
pancreas  must  be  extensively  diseased  before  diabetes  will  result.  In 
any  case,  a  compensatory  mechanism  is  not  unlikely  to  be  at  work  in 
some  cases,  for  the  intestine  is  competent  to  some  extent  to  absorb 
unaltered  proteins  and  fats  and  would  be  still  more  likely  able  to  absorb 
those  partially  converted.  Hydrochloric  acid,  too,  can  invert  sugars. 
Given  sufficient  time,  a  very  fair  amount  of  intestinal  digestion  may 
take  place,  even  if  the  pancreatic  secretion  were  reduced  to  a  minimum. 
The  experiments  on  dogs  are,  moreover,  scarcely  comparable  to  the 
conditions  prevailing  in  man,  for  the  extirpation  of  the  pancreas  is 
attended  with  much  shock,  and  we  have  no  information  how  the  secre- 
tion of  bile  is  influenced  by  this  operation.  The  fact,  however,  that 
the  administration  of  pig's  pancreas  to  the  animals  deprived  of  their 
pancreas  increased  the  absorption  of  food  goes  to  prove  that  a  deficient 
pancreatic  secretion  may  inhibit  absorption. 

The  conditions  which  might  be  expected  to  seriously  interfere  with 
the  action  of  the  secretion  are  those  which  extensively  damage  the  struc- 
tural integrity  of  the  pancreas.  Such  are  atrophy,  fibrosis,  chronic 
inflammation,  and  carcinoma.  Obstruction  to  the  duct,  unless  due  to 
the  presence  of  a  new-growth  in  the  head  of  the  pancreas  or  a  calculus, 
is  not  likely  to  be  often  a  cause.  Opie1  has  shown  that  in  about  two- 
thirds  of  all  cases  there  is  a  patent  duct  of  Santorini  in  man.  It  would 
be  extremely  unlikely  for  both  the  duct  of  Wirsung  and  that  of  Santorini 
to  be  obstructed  at  one  time,  though  it  is  not  impossible.  The  most 
likely  site  for  obstruction  is  at  the  papilla  of  Vater,  and  here  the  common 
bile  duct  would  be  occluded  as  well. 

In  one  other  way  the  pancreas  can  suffer  severe  injury,  and  this  is 
from  the  entry  of  bile  into  the  duct  of  Wirsung.  This  occurs  most  often 
where  there  is  a  biliary  calculus  impacted  at  the  bile  papilla,  of  such 

1  Diseases  of  the  Pancreas,  Lippincott  &  Co.,  1903. 


THE  BILE  365 

a  size  and  shape  as  to  prevent  the  discharge  of  bile  into  the  intestine, 
while  permitting  a  regurgitation  into  the  pancreatic  duct.  Acute 
hemorrhagic  pancreatitis  may  result1  or,  in  less  extreme  cases,  chronic 
pancreatitis.  The  same  result  can  be  produced  experimentally  by  in- 
jecting bile,  hydrochloric  acid,  or  certain  other  substances  into  the  duct. 

The  modifications  in  the  composition  of  the  bile  that  occur  as  the 
result  of  disease  are  by  no  means  well  understood.  Nor  is  their  practical 
significance,  if  any,  at  all  times  evident.  Only  in  the  case  of  the  forma- 
tion of  gallstones  have  we  anything  like  an  adequate  conception  of  the 
processes  that  take  place  and  the  important  results  that  may  eventuate 
from  them. 

The  modm  operandi  in  cholelithiasis  has  been  sufficiently  considered 
elsewhere  in  this  work  (vol.  i,  p.  874),  so  that  we  need  not  occupy  space 
by  discussing  the  subject  again  here.  Suffice  it  to  say,  that  the  chief 
factors  at  work  are:  (1)  An  infectious  catarrh  of  the  biliary  passages; 
(2)  stagnation  of  the  bile;  (3)  the  secretion  of  cholesteryl  oleate  and  cal- 
cium salts  by  the  inflamed  mucous  membrane;  (4)  the  splitting  up  of 
cholesteryl  oleate,  under  the  influence  of  alkalies,  into  cholesterin  and 
oleic  acid,  with  the  precipitation  of  the  former  and  the  formation  of 
more  or  less  soluble  soaps;  (5)  the  combination  of  the  calcium  salts 
with  bile  pigment  to  form  bilirubin  calcium. 

The  stagnation  of  the  bile  and  the  injury  to  the  walls  of  the  bile  pas- 
sages, that  so  commonly  result  from  the  presence  of  calculi,  lead  often 
to  secondary  infection  and  other  widespread  results.  These  will  be 
dealt  with  later,  and  we  shall  simply  content  ourselves  here  with  con- 
sidering the  effects  of  a  deficiency  of  the  supply  of  bile  in  the  intestine 
upon  the  processes  of  digestion. 

Bile  may  be  prevented  from  entering  the  bowel  by  obstruction  of  the 
common  or  hepatic  ducts  by  calculi,  the  pressure  of  enlarged  glands  or 
inflammatory  adhesions,  by  catarrhal  inflammations  of  the  passages, 
and  by  new-growths. 

The  results  that  follow  depend  upon  the  position  of  the  obstruction. 
If  at  the  bile  papilla,  the  outflow  of  the  pancreatic  juice  may  be  more  or 
less  interfered  with  as  well  as  that  of  the  bile.  If  higher  up,  the  supply 
of  bile  alone  is  cut  off.  Complete  absence  of  the  bile  from  the  intestine 
is  evidenced  clinically  by  jaundice,  more  or  less  malaise  and  constitutional 
disturbance,  and  a  peculiar  coloration  of  the  stools.  The  discharges 
are  pale  gray  or  "  clay-colored,"  owing  to  an  excess  of  fat  and  an  absence 
of  the  bile  pigments.  It  has  been  found,  as  a  result  of  experimentation 
on  dogs,2  and  observations  on  man  (Fr.  Miiller),  that  the  process  of 
intestinal  digestion  is  considerably  interfered  with.  The  absorption 
of  carbohydrates  is  practically  normal,  that  of  proteins  only  slightly, 
if  at  all,  diminished,  but  about  60  to  80  per  cent,  of  the  fat  ingested  escapes 
absorption.  This  effect  is,  in  part,  explained  by  the  fact  that,  to  be 

1  Opie,  Johns  Hopkins  Hospital  Bulletin,  12:1901:182. 

2  Voight,    Beitriige   z.   Biologic,    Stuttgart,    1882;   Rohmann,   Pfliiger's  Archiv 
29:  509. 


366      THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

absorbed  the  fats  must  be  first  hydrolyzed  and  rendered  soluble,  and 
the  cholates  are  excellent  solvents  for  fatty  acids.  Should  the  bile  be 
deficient,  the  solution  is  rendered  correspondingly  difficult.  Hewlett,1 
also,  has  shown  that  bile  has  the  power  of  accelerating  the  fat-splitting 
action  of  the  pancreatic  secretion  eightfold  or  more.  Lack  of  bile, 
therefore,  will  greatly  diminish  the  activity  of  the  pancreatic  juice  in 
respect  to  fats. 

While  the  bile  has  no  great  antiseptic  properties,  so  that  an  absence  of 
bile  in  the  intestine  is  not  attended  by  any  notable  increase  in  its  bacterial 
flora,  yet  it  is  possible  that  in  the  condition  of  biliary  obstruction  the 
processes  of  fermentation  may  be  more  or  less  abnormal.  On  this 
point,  however,  we  have  little  accurate  information. 

The  practical  application  of  these  facts  is  that  in  cases  of  this  kind, 
the  diet  should  consist  mainly  of  proteins  and  carbohydrates.  By 
keeping  out  the  fats,  we  not  only  avoid  giving  a  food  that  cannot  be 
digested  and  absorbed,  but  lessen  the  possibility  of  abnormal  decomposi- 
tions and  consequent  irritation. 

DISORDERS  OF  ABSORPTION  AND  ASSIMILATION. 

By  the  term  absorption,  as  we  shall  use  it  in  the  present  discussion, 
we  mean  the  process  by  which  the  various  nutritive  substances  contained 
within  the  alimentary  tract  are  taken  up  into  the  circulation.  The  sub- 
stances to  be  absorbed  are  the  reduction  products  derived  from  the 
primitive  constituents  of  the  food,  proteins,  carbohydrates,  and  fats, 
as  a  result  of  the  action  of  the  various  ferments.  These  are,  in  the  main, 
peptones,  albumoses,  dextrin,  various  sugars,  glycerin,  and  fatty  acids. 
To  a  slight  extent  proteins  and  fats  are  absorbed  as  they  are  without 
preliminary  hydrolization.  Water  and  salts  also  have  to  be  considered. 
In  our  discussion  of  this  subject  we  are  concerned  with  the  process  as 
it  occurs  in  the  stomach  and  bowel.  Absorption  practically  does  not 
take  place  in  the  mouth  and  oesophagus.  These  structures,  for  one  thing, 
are  covered  with  a  dense,  comparatively  impermeable  membrane  of 
squamous  epithelial  cells,  which  is,  further,  during  mastication  and  deglu- 
tition, protected  by  a  coating  of  mucus.  It  is  true  that  under  certain 
circumstances  toxic  substances  may  gain  an  entrance  through  the  buccal 
mucous  membrane,  but  the  mastication  of  food  is  not  a  parallel  case, 
for  the  food  remains  much  too  short  a  time  in  the  mouth  for  such  a  result 
to  follow  and  the  different  elements  of  the  food  are  scarcely  in  a  soluble 
state.  The  chief  function  of  the  stomach,  as  we  have  seen,  is  to  be  a 
receptacle  for  the  ingested  food.  Its  powers  of  digestion  are  compara- 
tively limited  and  its  powers  of  absorption  are  also  limited.  The 
carbohydrates,  dextrin,  saccharose,  dextrose,  lactose,  and  maltose,  are 
absorbed  fairly  well,  the  better  the  more  concentrated  the  solution. 
Peptones  are  absorbed  with  difficulty,  and  water  hardly  at  all.  Alcoholic 
solutions  are  taken  up  with  fair  avidity  and  seem  to  increase  the  power 

1  Johns  Hopkins  Hospital  Bulletin,  January,  1905. 


DISORDERS  OF  ABSORPTION  AND  ASSIMILATION  387 

of  the  stomach  to  absorb  other  substances.  By  far  the  most  important 
agent  in  the  absorption  of  foodstuffs  is  the  intestine,  for,  unlike  the 
stomach,  it  is  provided  with  special  organs  for  the  purpose,  namely,  the 
villi  and  solitary  follicles.  Peptones,  sugars,  emulsified  fats,  glycerin, 
and  fatty  acids  air  absorbed  chiefly  in  the  small  and,  to  a  much  less 
extent,  in  the  large  bowel. 

To  gain  anything  like  an  adequate  apprehension  of  the  complicated 
processes  concerned  in  absorption,  we  must  acquaint  ourselves  with  the 
physical  and  chemical  conditions  present  during  digestion.  Let  us 
take  the  small  intestine  as  an  illustration. 

Here  the  organs  specially  differentiated  for  the  purpose  of  absorption 
arc  the  villi.  Each  villus  is  a  minute  elevation,  projecting  about  1  mm. 
above  the  general  level  of  the  mucosa.  It  is  composed  of  a  core  of 
delicate  areolar  and  adenoid  tissue,  covered  with  a  basement  membrane 
and  a  single  layer  of  columnar  cells  continuous  with  those  lining  the 
intestine  elsewhere.  In  the  centre  is  a  lacteal,  or  possibly  two  lacteals, 
connected  by  lateral  channels,  and  an  afferent  bloodvessel.  The  wall 
of  the  lacteals  is  composed  of  a  single  layer  of  endothelial  plates.  About 
the  lacteals  can  be  detected  a  few  fibers  of  unstriped  muscle,  whose 
function  is,  by  their  contraction,  to  empty  the  lacteals  of  their  contents. 
Surrounding  the  lacteals  is  a  freely  anastomosing  meshwork  of  venous 
capillaries,  lying  between  them  and  the  superficial  epithelium.  The 
total  number  of  the  villi  is  very  great  and  has  been  estimated  at  4,000,000. 
They  are  so  closely  packed,  as  a  matter  of  fact,  that  the  mucosa  presents 
a  velvety  appearance.  During  digestion  the  blood  capillaries  are  con- 
gested and  the  villi  assume  an  erectile  condition. 

In  regard  to  the  process  of  absorption,  the  position  of  affairs  may  be 
briefly  summarized  in  this  way.  In  the  villi  the  bloodvessels  are,  of 
course,  filled  with  blood,  and  the  other  structures  are  bathed  in  lymph. 
The  lumen  of  the  intestine  contains  foodstuffs  largely  in  solution. 
Between  the  two  sets  of  fluids  there  is  only  a  basement  membrane  and 
a  single  layer  of  cells.  The  diffusion  of  the  fluids  must  take  place  through 
this  barrier.  To  reach  the  circulation,  furthermore,  the  resulting  mixture 
must  pass  another  obstruction,  namely,  the  cells  forming  the  walls  of 
the  lacteals.  The  old  view  was  that  this  interchange  was  of  the  nature 
of  an  osmosis,  but  no  physiologist  of  repute  would  indorse  this  idea 
now.  One  of  the  fundamental  properties  of  primitive  cells,  the  amoeba 
for  example,  is  the  power  of  taking  up  from  the  surrounding  medium 
foodstuffs  and  other  materials,  assimilating  what  are  necessary  and 
useful,  and  rejecting  those  that  are  not  required  or  positively  harmful. 
There  is  no  reason  for  supposing  that  the  cells  of  the  animal  body,  even 
when  differentiated  for  special  purposes,  lose  necessarily  these  early 
peculiarities.  In  fact,  all  the  evidence  that  we  possess  points  unquestion- 
ably the  other  way.  Applying  this  to  the  subject  in  hand,  we  believe 
that  absorption  cannot  be  explained  on  a  mere  physical  basis  of  osmosis, 
but  are  forced  to  conclude  that  the  process  is  inseparably  bound  up  with 
the  vital  properties  of  the  cells  concerned.  Absorption  and,  for  that 
matter,  secretion  are  selective  processes. 


368     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

The  investigation  of  the  subject  of  absorption  is  one  attended  by  great 
difficulties,  and  the  finer  details  of  the  process  are  still  to  a  large  extent 
matters  for  conjecture.  So  far  as  can  be  gathered  from  recent  work, 
what  takes  place  is  briefly  this :  The  various  substances  resulting  from 
protein  decomposition  are  taken  up  by  the  columnar  cells  covering  the 
villi,  pass  through  the  cells  into  the  subepithelial  reticulum,  and  find 
their  way  into  the  blood  capillaries,  whence  they  eventually  reach  the 
portal  vein  and  the  liver.  The  farther  the  process  of  decomposition  has 
been  carried,  the  easier  and  more  rapid  is  the  absorption.  It  is  likely, 
moreover,  that  these  products  of  protein  metamorphosis  undergo  some 
further  modification  in  the  tissue  spaces  before  they  enter  the  blood.  In 
a  similar  manner,  the  greater  part  of  the  sugars  reach  the  blood-vascular 
system,  though  possibly  some  small  portion  may  be  taken  up  by  the 
lymph.  The  fate  of  the  fats  has  been  a  matter  of  great  discussion.  It 
is  a  fact  that  globules  of  fat  can  be  demonstrated  by  appropriate  methods 
within  the  columnar  cells.  On  the  older  theory,  the  cells  in  question 
took  up  the  fats,  which  are  in  a  finely  emulsified  state,  bodily,  by  a  sort 
of  amreboid  action.  We  know  now,  however,  that  the  most  of  the  fats 
ingested  are  hydrolyzed  in  the  intestine  into  fatty  acids  and  glycerin. 
These,  with  the  soaps,  are  soluble,  and  are  probably  taken  up  in  this 
form  by  the  columnar  cells  and  there  recombined  to  form  neutral  fats. 
For,  it  has  been  shown  experimentally,  that  if  we  feed  an  animal  with 
fatty  acids,  neutral  fats  can  be  demonstrated  in  the  epithelium.  Ap- 
parently, then,  the  columnar  cells  can  synthesize  the  products  of  fat 
decomposition,  and  even  supply  the  glycerin  for  the  purpose.  The  re- 
combined  fats  are  then  taken  up  by  the  lymph-radicles  and  pass  into  the 
central  lacteals,  and  eventually  into  the  thoracic  duct  and  general 
circulation.  As  in  the  case  of  the  proteins,  the  fats  undergo  transforma- 
tion in  their  passage  from  the  epithelium  to  the  lacteals,  for  a  large  part 
of  the  fat  in  the  chyle  is  in  a  state  of  fine  division  or  molecular  disintegra- 
tion. 

The  carbohydrates,  chiefly  in  the  form  of  sugars,  are  taken  up  by  the 
epithelium  and  passed  into  the  portal  blood.  To  some  extent,  also, 
dextrin  can  be  dealt  with  by  the  columnar  cells. 

The  water  is  taken  up  by  the  mucosa  of  the  small  intestine,  but  the 
amount  lost  in  this  way  is  replaced  by  water  excreted  by  the  cells,  for 
the  contents  of  the  bowel  at  the  ileocecal  valve  are  just  about  as  fluid  as 
those  at  the  duodenum.  The  chief  absorption  of  water  takes  place  in 
the  large  bowel,  so  that  the  feces,  when  they  reach  the  rectum,  are  firm 
and  comparatively  dry.  In  cases  of  constipation  almost  the  whole 
of  the  water  may  be  taken  up  and  the  stools  become  hard,  stony,  and 
scybalous. 

The  fate  of  the  unused  ferments  is  somewhat  doubtful.  Probably, 
they  are  not  resorbed,  but  are  destroyed  in  the  colon. 

We  must  not  leave  this  part  of  the  subject  of  absorption  without  a 
brief  reference  to  the  role  played  by  the  leukocytes  in  the  process. 
During  digestion  numerous  leukocytes  can  be  seen  in  the  subepithelial 
connective  tissue,  between  the  columnar  cells,  and  on  the  surface  of  the 


DISORDERS  OF  ABSORPTION  AND  ASSIMILATION  369 

mucous  membrane  of  the  intestine,  which  are  apparently  attracted  from 
the  vessels  and  tissue  spaces  by  positive  chemiotaxis.  This  fact  was 
long  ago  pointed  out  by  Heidenhain,  and  has  been  amply  demonstrated 
by  numerous  observers  since.  One  part,  at  least,  of  their  function  is 
connected  with  absorption,  for  fat  and  the  precursors  of  fat  can  be 
detected  in  their  substance  by  appropriate  methods.  A.  B.  Macallum1 
has  very  prettily  and  conclusively  shown  that  the  leukocytes  can  take 
up  foreign  substances,  notably  iron.  Experimenting  with  the  lake 
lizard  (necturus),  he  took  an  animal  that  had  fasted  for  thirty  months 
(to  insure  that  the  intestine  would  be  empty)  and  fed  it  on  albuminate 
and  peptonate  of  iron.  Killing  the  lizard  eight  hours  later,  he  found 
leukocytes  laden  with  iron  within  the  lumen  of  the  bowel  between  the 
columnar  cells  of  the  mucosa,  and  even  in  the  capillaries  of  the  liver 
and  spleen,  showing  that,  through  the  agency  of  leukocytes,  iron  could 
enter  the  portal  system  and  general  circulation.  Presumably  the  same 
would  hold  good  for  other  substances,  bacteria  and  foodstuffs.  We 
must,  however,  interpret  this  attraction  of  the  leukocytes  to  the  mucous 
surface  of  the  bowel  with  some  caution,  for  the  same  thing  occurs  as  a 
result  of  the  exhibition  of  a  saline  purge,  such  as  magnesium  sulphate, 
where  the  process  at  work  is  quite  the  reverse  of  absorption.  Still, 
this  probably  only  means  that  in  this  case,  also,  we  have  to  deal  with 
an  irritation  and  stimulation  of  the  secreting  cells,  which  determines  the 
attraction  of  the  leukocytes.  It  is  altogether  likely  that  the  leukocytes 
play  an  important  part  in  the  function  of  absorption,  perhaps  not  so 
much  in  the  case  of  the  neutral  fats,  but  more  especially  in  regard  to 
proteins  and  the  soluble  products  of  digestion. 

We  shall  be  helped  to  a  better  understanding  of  the  disorders  that  may 
attend  absorption  if  we  bear  in  mind  the  chief  factors  in  the  mechanism 
of  this  function.  It  is  evident,  from  what  has  been  said  above,  that  the 
structures  mainly  concerned  are  the  columnar  cells  lining  the  mucosa  of 
the  gastro-intestinal  tract,  the  bloodvessels,  and  the  lymphatic  system. 
Disorders  of  absorption  would  naturally,  then,  be  likely  to  attend  dis- 
turbance of  any  portion  of  this  mechanism.  But  there  is  another  way 
in  which  these  disorders  might  arise.  The  stomach  and  intestine  may 
contain  abnormal  substances  that  are  actually  deleterious  to  the  economy, 
or  secretions  that  are  quite  normal  may  be  resorbed  instead  of  passing 
away. 

We  must  admit  that  our  knowledge  of  the  pathology  of  absorption  is 
far  from  being  complete,  but  some  points  seem  fairly  clear.  The 
intestinal  mucosa  constitutes,  as  it  were,  a  first  line  of  defence.  So  long 
as  the  layer  of  columnar  cells  is  intact,  it  is  possible  for  secretory  and 
absorptive  processes  to  go  on  normally,  and  a  powerful  barrier  exists 
against  microbic  invasion.  Should  a  solution  of  continuity  occur,  or 
should  the  vitality  of  the  lining  cells  be  damaged,  then  infection  and 
intoxication  are  likely  to  be  induced.  Yet  this  is  not  absolutely  necessary, 
for  it  has  been  shown  conclusively  that  under  certain  circumstances 

1  Jour,  of  Phys.,  16: 1894: 268. 
24 


370     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

bacteria  may  penetrate  a  normal  mucosa.  Notably  is  this  the  case  with 
the  bacillus  of  tuberculosis.  Similarly,  soluble  toxins  may  pass  in.  Some 
of  these  may  be  ingested  with  the  food,  while  others  are  produced  in 
situ  by  abnormal  decompositions  and  bacterial  fermentations.  Thus, 
ptomaines,  like  neurin,  mydalein,  rnytilotoxin,  and  tyrotoxicon,  may 
be  present  in  putrefying  food.  Specific  bacteria,  such  as  the  typhoid 
bacillus,  the  cholera  vibrio,  the  bacillus  of  tuberculosis,  and  the  actino- 
myces  bovis,  may  lead  to  infections  of  alimentary  origin.  Or,  again, 
chemical  substances,  like  the  metallic  salts,  aromatic  compounds,  and 
the  fatty  acids,  may  cause  trouble.  In  the  vast  majority  of  cases,  how- 
ever, these  substances  are  distinctly  irritating  and  lead  to  injury  of  the 
intestinal  mucosa. 

Normally,  the  gastro-intestinal  tract  contains  bacteria  in  considerable 
numbers  and  some  variety.  The  excessive  growth  of  these  is  held  in 
check  by  the  acid  in  the  stomach,  and  by  the  biliary  acids,  the  fatty 
acids  resulting  from  the  decomposition  of  fats,  and  the  digestive  secretions, 
in  the  intestine.  Perhaps,  of  even  more  importance,  is  the  regular  and 
frequent  elimination  from  the  body  of  fecal  matter,  whereby  stasis  is 
prevented  and  little  time  is  allowed  for  development  to  occur.  In 
the  large  bowel,  where  the  refuse  products  are  retained  longest,  the 
greater  part  of  the  nutritive  material  has  been  absorbed  and  there  is  less 
for  the  bacteria  to  decompose.  Probably,  too,  those  microorganisms 
that  may  be  called  the  natural  inhabitants  of  the  alimentary  tract  perform 
a  useful  function  in  antagonizing  the  effects  of  foreign  invaders. 

Disorders  may  be  brought  about  by  excessive  multiplication  of  the 
normal  bacterial  inhabitants  of  the  tract  or  an  increase  in  their  virulence. 
Indigestible  food,  disturbances  of  secretion,  and  motility  are  competent 
to  cause  the  former  condition,  while  strangulation  of  the  bowel  may 
cause  the  latter.1  Local  inflammation  and  irritation  of  the  bowel  often 
then  result.  Pathogenic  bacteria  of  extraneous  origin,  when  ingested, 
do  not  always  produce  serious  results,  for  they  may  be  rendered  inert 
by  the  various  agencies  already  referred  to.  But  if  they  gain  entrance 
to  the  body  in  large  numbers,  or  in  small  doses  frequently  repeated,  or 
if  their  virulence  be  high,  infection  usually  occurs.  Their  pathogenic 
powers  will  be  aided  by  any  diminution  of  vigor  in  the  ordinary  bacterial 
flora,  or  by  diminished  resistance  on  the  part  of  the  mucosa.2 

Disturbances  affecting  the  vitality  of  the  lining  columnar  cells  of 
the  gastro-intestinal  mucous  membrane,  if  widespread,  seriously  inter- 
fere with  absorption.  Thus,  in  enteritis  of  a  moderate  grade,  the 
absorption  of  fats  is  diminished,  and  in  the  most  severe  forms  the  absorp- 
tion of  all  kinds  of  foodstuffs  is  difficult  or  impossible.  Where  only  small 
isolated  patches  of  the  mucosa  are  involved  this  result  does  not  follow. 
Thus,  in  most  cases  of  typhoid  fever  the  power  of  absorption  is  not 
notably  diminished. 

1  Macaigne,  Arch.  gen.  de  Med.,  December,  1896. 

2  The  reader  will  find  the  subject  of  intoxication  and  infection  originating  in  the 
digestive  tract  dealt  with  in  vol.  i,  pp.  279  and  349. 


JAUNDICE  371 

Passive  congestion  of  the  intestines  will  delay  absorption  and  especially 
interferes  with  the  absorption  of  fats.  Obstruction  of  the  lymphatics, 
such  as  occurs  in  tuberculosis  of  the  bowels  and  mesenteric  glands, 
notably  hampers  the  absorption  of  the  fats. 

Increased  peristalsis,  as  in  diarrhoea,  if  affecting  the  small  bowel,  will 
seriously  diminish  absorption  by  lessening  the  time  that  the  chyme  re- 
mains in  contact  with  the  mucosa.  When  due  to  disordered  conditions 
of  the  large  bowel,  the  absorption  of  water  is  lessened,  but  the  effect  on 
the  general  nutrition  is  not  so  great,  for  by  the  time  the  foodstuffs  have 
reached  the  colon  the  greater  part  of  the  nutritive  material  has  already 
been  extracted. 

A  number  of  serious  disturbances  may  arise  from  the  resorption  of  the 
secretions  normally  found  in  the  intestines.  A  certain  amount  of 
resorption  is,  indeed,  physiological.  For  example,  the  solidity  of  the  feces 
found  in  the  large  bowel  indicates  that  a  considerable  amount  of  water 
is  taken  again  into  the  circulation.  The  increase  in  the  viscosity  of  the 
bile  contained  within  the  gall-bladder,  as  compared  with  that  found  in 
the  ducts,  points  in  the  same  direction.  It  is  probable,  too,  that  a  con- 
siderable proportion  of  the  bile  salts  and  pigments  are  resorbed  from 
the  intestine.  We  have,  in  fact,  to  recognize  a  reversibility  of  cellular 
action.  According  to  the  direction  of  the  forces  exerted  upon  a  cell  or 
series  of  cells,  either  secretion  or  resorption  will  take  place.  Under 
ordinary  circumstances  resorption  is  followed  by  no  noticeable  effects, 
but  should  a  given  secretion  be  resorbed  in  excess,  or  should  it  be  taken 
up  by  cells  other  than  those  that  produced  it,  far-reaching  disorders  are 
certain  to  follow.  The  most  important  disorders  that  we  have  to  con- 
sider here  are  those  connected  with  the  bile  and  pancreatic  secretion. 

Bile  will  be  resorbed  into  the  circulation  if  from  any  cause  there  be 
•obstruction  to  its  free  discharge  into  the  intestine.  This  may  be  brought 
about  by  calculi  in  the  common  or  hepatic  ducts,  a  catarrh  of  the  finer 
bile  channels  (cholangitis),  tumors  of  the  common  bile  duct  or  the  head 
of  the  pancreas,  enlarged  glands  or  abscesses  at  the  hilus  of  the  liver, 
and  tumors  of  the  liver  itself.  The  condition  is  evidenced  clinically  by 
jaundice,  slowed  pulse,  mental  hebetude,  itchiness  of  the  skin,  and 
lessened  coagulability  of  the  blood.  According  to  the  degree  of  obstruc- 
tion, the  digestive  processes  within  the  bowel  are  interfered  with,  as 
described  above,  and  the  stools  are  more  or  less  devoid  of  their  normal 
brown  pigment.  Bile  can  be  detected  in  the  urine.  The  immediate 
mechanical  effect  is  dilatation  of  the  bile  passages,  particularly  the  capil- 
laries, with  compression  and,  possibly,  atrophy  of  the  parenchymatous 
cells  of  the  liver.  The  retained  bile  passes  into  the  lymph-channels  and 
the  venous  system  and  eventually  reaches  the  general  circulation.  The 
resorption  of  the  bile  is  dependent  not  only  on  the  degree  of  the  mechanical 
obstruction,  but  on  the  character  of  the  secretion  also.  A  thick,  viscid 
bile  may  be  resorbed  even  when  the  obstruction  is  comparatively  slight, 
as  might  result,  for  example,  from  a  catarrh  of  the  finer  bile  passages 
or  swollen  hepatic  cells.  The  icterus  found  in  phosphorus  and  toluylen- 
diamin  poisoning,  snake-bite,  and  certain  infections  and  intoxications 


372     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

is  of  this  type.  The  toxic  symptoms  are  referable  to  the  presence  of  the 
bile  salts  in  the  blood,  as  has  repeatedly  been  demonstrated  experiment- 
ally. The  cholates,  even  in  small  quantities,  stimulate  the  vagi,  and 
larger  amounts  act  upon  the  heart  itself  and  produce  convulsions.  The 
resorption  of  bile  from  the  intestines  in  such  amounts  as  to  produce 
jaundice,  in  the  absence  of  mechanical  obstruction  to  its  outpouring, 
is  hardly  likely,  though  Quincke  has  considered  this  probable  in  the  case 
of  icterus  neonatorum.  Jaundice  can  be  brought  about,  of  course,  in 
other  ways  besides  obstruction,  but  this  phase  of  the  subject  does  not 
concern  us  here.  For  fuller  information  on  this  part  of  the  subject,  the 
reader  is  referreo!  elsewhere  in  this  work  (vol.  i,  p.  885  et  seq.). 

Obstruction  to  the  free  outpouring  of  the  pancreatic  secretion  may  at 
times  be  followed  by  serious  results.  Dilatation  of  the  ducts  and  the  fine 
ramifications  of  the  ducts  within  the  acini  are  the  immediate  sequel, 
but  a  chronic  interstitial  inflammation  with  fibrosis  is  eventually  set  up. 
Obstruction  of  a  lateral  branch  leads  to  cystic  dilatation,  provided  that 
the  cells  of  the  affected  lobule  are  capable  of  secretion.  Some  of  these 
retention  cysts  attain  a  relatively  enormous  size.  Should  large  areas  of 
the  secreting  structure  be  destroyed  by  catarrhal  inflammation  and 
pressure,  the  internal  secretion  of  the  pancreas  may  be  interfered  with 
and  a  form  of  glycosuria  result.  Obstruction  to  the  outflow  of  secretion 
may  be  produced  by  new-growths  and  fibrosis  in  the  head  of  the  pancreas, 
or  a  calculus  impacted  in  the  ampulla  of  Vater.  Should  the  calculus 
be  of  a  certain  shape  and  size,  and  so  placed  as  to  allow  the  entry  of  bile 
into  the  pancreatic  duct,  acute  hemorrhagic  pancreatitis  is  apt  to  be  set 
up.  This  is  associated  with  a  curious  condition,  known  as  fat  necrosis, 
in  which  whitish  opaque  areas  are  to  be  found  in  the  fatty  tissues  sur- 
rounding the  pancreas,  the  omentum,  the  appendices  epiploicse,  the 
mesentery,  and  peritoneum.  This  has  been  shown  to  be  due  to  the 
action  of  the  fat-splitting  ferment  of  the  pancreatic  juice  upon  the  fat, 
whereby  glycerin,  fatty  acids,  and  calcium  soaps  are  formed  in  the  tissues. 
The  diffusion  out  of  the  secretion  appears  to  be  due  to  pressure  within 
the  ducts  or  to  actual  injury  to  the  cells.  The  condition  has  been  met 
with  in  other  forms  of  inflammation  of  the  pancreas,  but  is  rare  in  cases 
of  suppuration  (see  vol.  i,  p.  903). 

THE  RELATIONSHIP  OF  DISEASES  OF  THE  ALIMENTARY  TRACT 
TO  DISORDERS  OF  THE  GENERAL  SYSTEM. 

As  we  have  had  occasion  to  point  out  already,  the  alimentary  tract  is 
brought  into  close  connection  with  the  other  great  systems  of  the  body 
through  the  blood  and  lymph-circulation  and  by  means  of  the  nervous 
mechanism.  It  is  not  surprising,  therefore,  that  disorders  of  other  systems, 
or  of  the  body  as  a  whole,  will  often  have  a  profound  effect  upon  the 
digestive  organs,  and  vice  versa.  The  cells  of  the  alimentary  system  being 
both  absorptive  and  eliminative  in  their  functions,  we  are  prepared  to 
find  that  intoxication  and  infection  will  bulk  very  largely  in  any  con- 
sideration of  this  part  of  our  subject. 


RELATIONSHIP  OF  DISEASES  OF  THE  ALIMENTARY  TRACT     373 

It  is  a  well-recognized  principle  in  pathology  that  when  one  organ 
or  system  is  from  any  cause  inhibited  in  its  action,  others  will  attempt 
to  take  up  the  work  and  carry  it  on  as  perfectly  as  they  may.  This 
is  known  as  the  law  of  vicarious  function.  It  is  particularly  well  ex- 
emplified in  the  case  of  glandular  organs,  and  the  attempt  at  compensa- 
tion is  often  manifested  by  structural  change  as  well  as  increase  or 
perversion  of  function.  Instances  might  be  multiplied  and  are  not 
wanting  in  connection  with  the  alimentary  system.  As  a  corollary 
to  this,  it  can  be  laid  down  that  the  excessive  secretion  of  any  gland,  if 
it  result  in  a  great  loss  of  the  body  fluids,  will  be  accompanied  by  dimin- 
ished activity  of  other  secretory  organs.  A  familiar  example  of  the  first 
class  is  the  elimination  of  urea  and  other  substances  by  the  skin  and 
mucous  membranes  in  cases  of  chronic  Bright's  disease.  Here  it  is 
not  uncommon  to  get  stomatitis  and  diarrhoea  as  a  result.  Ulceration  of 
the  lower  bowel  is  a  not  infrequent  accompaniment.  The  exposure  of  the 
body  to  alternations  of  heat  and  cold  is  frequently  followed  by  diarrhoea 
in  some  people.  This  is,  in  part,  due  to  the  interference  with  perspira- 
tion, but,  possibly  also,  there  may  be  a  nervous  element  as  well.  Sup- 
pressed menstruation,  the  absorption  of  abscesses,  the  retrocession  of 
eruptions  in  smallpox,  measles,  and  scarlatina,  are  occasionally  followed 
by  diarrhoea.  Gout  in  many  cases  is  associated  with  a  looseness  of  the 
bowels;  in  fact,  diarrhoea  may  be  the  only  notable  manifestation  of  the 
uratic  diathesis  (diarrhoea  arthritica).  Cases  are  known  where  checking 
the  intestinal  evacuations  has  been  followed  by  the  arthritic  manifesta- 
tions. Again,  an  example  of  the  second  group,  the  constipation  of 
diabetes,  often  associated  with  dryness  of  the  skin  and  mouth,  is  the 
result  of  the  polyuria. 

The  diarrhoea  that  at  times  accompanies  the  infectious  diseases, 
notably  the  exanthemata  and  pneumonia,  is  probably  for  the  most  part 
due  to  attempts  at  elimination  and  perverted  excretion. 

Other  ways  in  which  the  stomach  and  bowels  are  affected  in  systemic 
diseases  are  through  the  circulatory  and  nervous  systems.  The  diarrhoea 
met  with  in  exophthalmic  goitre  and  Addison's  disease  are  apparently 
vasomotor  in  origin.  That  found  in  amyloid  disease  of  the  bowels 
is  possibly  due  to  interference  with  excretion  and  absorption,  whereby 
irritation  is  set  up.  The  vomiting,  which  is  so  often  an  early  symptom 
of  the  onset  of  acute  infectious  disease,  is  due  to  efferent  nervous  im- 
pulses which  cause  excessive  irritability.  Constipation  and  diarrhoea 
may  result  from  abnormal  mental  and  nervous  states,  as,  for  example, 
in  the  acute  infections,  notably  meningitis,  chronic  lead  poisoning,  neu- 
rasthenia, and  hysteria.  Involuntary  evacuations  and  diarrhoea  occur 
in  cases  of  coma,  mental  degradation,  and  hysteria. 

The  increasing  number  of  studies  that  have  been  made  in  regard  to 
the  normal  and  abnormal  digestive  processes,  especially  in  connection 
with  bacterial  activity  within  the  intestine,  serve  to  indicate  our  realiza- 
tion of  the  growing  importance  of  this  subject.  While  our  knowledge 
is  still  far  from  complete,  for  the  problems  involved  are  of  very  wide 
range,  sufficient  has  been  gathered  to  prove  that  the  absorption  of  dele- 


376     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

is  believed  to  be  a  physiological  process  and  is  of  no  special  importance. 
Jaundice  and  cholemia  never  seem  to  be  produced  in  this  way.  The 
gastric  and  pancreatic  ferments  that  are  not  utilized  may  also  possibly 
undergo  resorption,  but  observations  made  so  far  would  seem  to  show 
that  this  rarely  occurs  to  any  extent. 

Toxic  substances  are  produced  in  the  course  of  the  ordinary  physio- 
logical processes  of  digestion.  Among  these  may  be  mentioned: 

(a)  Those  derived  from  protein  disintegration:  Albumoses,  peptone, 
indol,  skatol,  phenol,  leucin,  tyrosin,  fatty  acids,  acetone,  ammonia, 
cystin,  carbon  dioxide,  hydrogen,  sulphuretted  and  carburetted  hydrogen, 
and  methyl  mercaptan. 

(6)  Those  derived  from  carbohydrates:  Formic,  acetic,  propionic, 
butyric,  valerianic,  lactic,  and  succinic  acids,  acetone,  and  various 
gases. 

(c)  Those  derived  from  fats :  Fatty  acids  and  glycerin. 

It  is  unlikely  that,  under  normal  circumstances,  any  of  these  sub- 
stances are  absorbed  in  sufficient  quantity  to  cause  disturbance,  for  the 
peristalsis  of  the  intestine  hurries  them  along,  and  they  are  either  grad- 
ally  neutralized  as  they  reach  the  lower  bowel  or  are  quickly  eliminated. 
In  cases  of  obstruction,  however,  they  might  be  expected  to  play  a  part 
by  causing  irritation  of  the  mucous  membrane,  and  with  the  multipli- 
cation of  the  bacterial  flora  of  the  intestine  which  inevitably  occurs  in 
such  cases,  they  join  their  forces  with  those  of  the  similar  substances 
resulting  from  putrefactive  fermentation. 

3.  The  activity  of  the  microorganisms  normally  found  within  the  gastro- 
intestinal tract  is  kept  within  bounds  by  the  acidity  of  the  gastric  secretion, 
the  presence  of  bile,  and  the  regular  evacuation  of  the  intestinal  contents. 
The  bacteria  that  are  present  under  these  circumstances  may  be  re- 
garded as  non-pathogenic,  yet,  on  occasion,  their  numbers  and  virulence 
may  be  so  increased  as  to  produce  pathological  effects.  This  is  par- 
ticularly the  case  when  there  is  constipation,  strangulation,  or  some 
inflammatory  affection  of  the  bowel.  The  bacteria  may  actually  pass 
through  the  wall  of  the  intestine,  if  its  vitality  be  lowered  in  this  way, 
even  at  times  without  any  solution  of  continuity,  and  may  set  up  a  gen- 
eral infection,  or,  in  many  instances,  a  peritonitis.  In  the  same  way,  per- 
foration or  rupture  of  the  bowel  is  followed  by  an  infective  inflammation 
of  the  peritoneum.  The  systemic  effects  of  constipation,  irritability,  men- 
tal dulness,  headache,  malaise,  and  earthiness  of  the  skin,  are,  no  doubt, 
attributable  to  the  slow  absorption  of  toxins,  in  large  part  of  bacterial 
origin,  from  the  lumen  of  the  bowel.  Where  actual  organic  obstruction 
exists,  the  symptoms  are  much  more  intense,  namely,  pain,  headache, 
vomiting,  intense  prostration,  lowered  temperature,  a  weak  circulation, 
coma,  and  perhaps  death.  The  higher  up  in  the  bowel  the  obstruction 
is,  the  more  severe  are  the  manifestations,  for  the  absorptive  powers  of 
the  mucous  membrane  are  much  more  active  in  the  small  bowel  than 
in  the  large.  In  conditions  of  health,  however,  what  might  be  termed 
the  natural  inhabitants  of  the  gastro-intestinal  tract  probably  exert  a 
beneficent  action  in  the  economy,  for  they  apparently  have  the  power  of 


CIRRHOSIS  OF  THE  LIVER  377 

inhibiting  the  activity  of  foreign  intruders  which  may  make  their  way 
into  the  bowel.1  They  may,  however,  be  overborne  in  their  resistance, 
should  alien  bacteria  be  introduced  into  the  alimentary  system  in  suffi- 
ciently large  numbers,  or  if  their  vigor  be  diminished  from  any  cause. 

The  bacteria  in  question  produce  their  effects  in  two  ways,  by  the 
excretion  of  the  products  of  their  own  metabolism  and  by  initiating  ab- 
normal processes  of  fermentation.  In  this  manner  are  formed  a  variety 
of  substances,  such  as  the  fatty  acids,  indol,  skatol,  phenol,  compounds 
of  the  aromatic  series,  pyridin  and  chinolin  bodies,  diamins,  toxalbumins, 
hydrogen,  carbonic  dioxide,  and  methane. 

The  effects  produced  may  be  local  in  the  tract  or  its  accessory  organs. 
If  acute,  we  may  find  degeneration,  inflammation,  or  ulceration ;  if  chronic, 
inflammation,  atrophy,  and  fibrosis.  At  other  times  the  resisting  barriers 
are  broken  down,  to  the  extent  that  toxins  make  their  way  into  the  circu- 
lation and  set  up  general  toxemia.  Some  of  the  substances  absorbed, 
notably  those  resulting  from  protein  decomposition,  are  rendered  non-toxic 
by  combination  with  sulphuric  acid,  glycocoll,  and  glycuronic  acid,  but, 
naturally,  to  this  transformation  there  is  a  limit.  We  may  gain  a  fair  idea 
of  the  amount  of  putrefactive  change  going  on  in  the  bowel  by  estimating 
the  amount  of  ethereal  or  aromatic  sulphates  in  the  urine,  as  they  are 
both  absolutely  and  relatively  increased  in  amount  in  this  condition.  It 
should  be  remarked  here,  in  passing,  that  it  is  possible  that  we  have 
hitherto  laid  too  great  emphasis  on  the  role  played  by  the  protein  deriva- 
tives, for  recent  experiments  would  tend  to  indicate  that  the  pernicious 
effects  attributed  to  the  products  of  protein  decomposition  are  rather  to 
be  laid  at  the  door  of  the  potassium  salts. 

The  interesting  and  very  important  attempts  that  have  been  made 
to  elucidate  the  nature  of  hepatic  cirrhosis,  to  take  a  familiar  condition, 
will  serve  to  illustrate  the  important  part  played  by  certain  substances, 
products  both  of  normal  and  abnormal  fermentation.  Hanot2  was 
of  the  opinion  that  cirrhosis  of  the  liver  is  due  to  the  irritation  produced 
by  the  absorption  of  certain  substances  resulting  from  disordered  diges- 
tion. Hanot  and  Boix,  in  support  of  this  theory,  showed  that  atrophic 
cirrhosis  of  the  liver  could  be  produced  in  rabbits  by  the  administration 
of  lactic,  butyric,  and  valerianic  acids.  The  deleterious  effects  of  potas- 
sium salts  is  well  shown  by  some  experiments  of  Lancereaux.3  He 
noticed  that  in  Paris  cirrhosis  of  the  liver  was  more  common  in  those 
drinking  wine  than  in  those  using  other  alcoholic  beverages.  This  he 
attributed  to  the  fact  that  many  of  the  wines  contained  sulphate  of  potas- 
sium to  render  them  "dry."  Some  of  these  'plastered"  wines  contained 
as  much  as  four  to  six  grams  per  liter  of  this  substance.  By  feeding 
rabbits,  guinea-pigs,  and  dogs  with  sulphate  of  potash  for  from  six  to 
eighteen  months  he  could  produce  typical  portal  cirrhosis. 

While  the  attention  of  these  observers  was  concentrated  on  the  deter- 

1  Bienstock,  Arch.  f.  Hyg.,  36:1900:335,  and  39:1900:390. 

2  Arch.  g<§n.  de  Med.,  1:1899:3. 

3  Bull,  de  1'Acad.  de  Med.,  38:1897:202. 


378     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

mination  of  one  particular  point,  it  may  be  remarked,  in  criticism  of  their 
conclusions,  that  one  cannot  exclude  the  influence  of  bacteria  in  contribu- 
ting to  the  result.  That  bacteria  and  their  toxins  play  an  important  role 
in  this  connection  cannot  be  doubted.  Krawkow1  has  noted  that  cirrhosis 
could  be  induced  by  giving  bacterial  toxins  by  the  mouth.  Ramon,2 
also,  tried  a  number  of  feeding  experiments  on  different  series  of  animals, 
giving  (1)  alcohol,  (2)  microbic  toxins,  (3)  living  cultures  of  bacteria, 
and  (4)  alternate  doses  of  alcohol  and  bacterial  toxins.  The  animals 
to  whom  the  cultures  were  administered  died  of  septicemia,  with  fatty 
degeneration  of  the  liver.  Those  fed  on  alcohol  alone  developed  fatty 
livers.  In  one  case,  in  which  alternating  doses  of  alcohol  and  toxins 
were  given,  the  animal,  after  surviving  ten  months,  exhibited  signs 
of  hepatic  cirrhosis.  The  influence  of  alcohol  in  the  pathogenesis  of 
cirrhosis  has  been  the  subject  of  much  debate.  Some  have  held  that 
it  sets  up  a  gastro-intestinal  catarrh,  and  that  then  the  toxins  elaborated 
are  carried  to  the  liver  and  set  up  the  disturbance.  Others  think  that 
the  alcohol,  being  quickly  absorbed,  is  carried  directly  to  the  liver 
and  leads  to  irritation  and  degeneration.  Still  others,  like  Ramon, 
take  an  intermediate  position,  holding  that  gastro-intestinal  catarrh 
alone  does  not  explain  the  condition,  but  that  alcohol  promotes  the 
absorption  of  toxins  from  the  bowel,  and,  moreover,  lessens  the  power 
of  the  liver  to  resist  them.  Further,  Bindo  de  Vecchi3  was  able  to  pro- 
duce proliferation  of  the  interstitial  connective  tissue  of  the  liver  by  intro- 
ducing certain  germs  into  the  intestine.  His  experiments  point  to  the 
pathogenicity  of  the  B.  coli.  Weaver4  obtained  similar  results  by  the 
subcutaneous  injection  of  a  germ  belonging  to  the  colon  group,  which 
he  isolated  from  guinea-pigs  dying  spontaneously,  and  Hektoen,5  with  a 
bacillus  of  the  pseudodiphtheria  type.  The  preponderance  of  evidence 
therefore,  goes  to  show  that  certain  products  of  fermentation  in  the  bowel 
are  competent  to  produce  cirrhosis.  Bacteria,  while  in  general  they  are 
likely  to  produce  acute  lesions  of  a  suppurative  type,  or  even  septicemia, 
can,  if  sufficiently  attenuated,  lead  to  a  slow  proliferative  change. 
Apparently,  however,  before  bacteria  can  act,  there  must  be  a  lowering 
of  the  vitality  of  the  liver  parenchyma.  This  can  be  produced  by  alcohol, 
bacterial  toxins,  and  certain  organic  fatty  acids. 

The  hemolytic  action  of  certain  microorganisms  and  their  toxins  has 
now,  for  some  years,  been  widely  recognized.  It  is  not  at  all  improbable, 
and  there  is  a  certain  amount  of  evidence  in  favor  of  it,  that  certain  forms 
of  severe  anemia  and  the  deposit  of  iron  pigment  in  various  parts  of  the 
body  are  due  to  gastro-intestinal  intoxication  and  infection.  Hunter,6 
for  example,  is  a  strong  upholder  of  the  view  that  pernicious  anemia  is 
infective  in  origin.  His  earlier  observations  tended  to  the  conclusion  that 

1  Arch,  de  M6d.  exp<§r.  et  d'Anat.  path.,  1896: 106  and  244. 

2  Presse  MSdicale,  April  21,  1897:178. 

3  Lo  Sperimentale,  An.  53 : 3. 

4  Trans.  Chicago  Path.  Soc.,  3: 1900:  228. 

5  Jour.  Path,  and  Bact.,  7:  1901 :  214. 

6  Lancet,  London,  i:  1900: 221,  296,  371. 


GASTRO-INTESTINAL  INFECTION  379 

this  disease  is  due  to  the  hemolytic  action  of  some  special  toxin  elaborated 
in  and  absorbed  from  some  part  of  the  alimentary  tract.  This  toxin  is 
not  simply  the  product  of  ordinary  fermentative  and  putrefactive  pro- 
cesses, but  is  of  a  special  infective  nature.  Later,  he  showed  that  the 
region  of  the  greatest  absorption  was  the  stomach,  but  to  some  extent 
the  buccal  and  intestinal  mucosse.  The  important  etiological  factors 
in  the  disease  are  carious  teeth,  stomatitis,  and  glossitis,  which  lead 
to  an  infective  gastritis.  Certain  local  conditions  in  the  stomach,  such 
as  malignant  disease,  gastritis,  and  atrophy  of  the  mucous  membrane, 
also  predispose.  The  nature  of  the  infecting  agent  is  not  clear,  but  it 
is  possibly  of  a  mixed  kind. 

One  other  phase  of  this  subject,  in  conclusion,  demands  a  few  words. 
There  is  now  abundant  evidence  to  show  that  microorganisms  are 
constantly  gaining  an  entrance  into  the  system  by  way  of  the  alimentary 
tract.  Ruffer,1  on  examining  sections  taken  from  the  small  intestine 
of  healthy  rabbits,  found  that  leukocytes  were  present  on  the  surface  of 
the  mucosa;  others,  again,  between  the  epithelial  cells,  that  had  engulfed 
bacteria.  The  Peyer's  patches  contained  immense  numbers  of  micro- 
organisms, apparently  within  the  lymphoid  cells.  Bizzozero2  and 
Ruffer  found  an  analogous  state  of  things  in  the  case  of  the  rabbit's 
tonsil.  Further,  the  leukocytes,  travelling  back  from  the  surface  to  the 
lymphoid  follicles,  were  taken  up  by  certain  large  cells  (macrophages) 
and  eventually  digested,  together  with  any  bacteria  or  foodstuffs  they 
might  contain.  One  of  us  (A.  G.  N.3),  also,  has  demonstrated  that 
microorganisms  can  be  found  in  various  stages  of  disintegration  within 
the  capillary  vessels  of  the  mesentery  in  rabbits,  cats,  dogs,  and  in  the 
human  subject  at  postmortem.  Others,  again,  may  be  detected  in  the 
meshes  of  the  tissues,  grouped  about  the  nuclei  of  what  are  presum- 
ably wandering  cells,  and  within  the  lining  endothelia  of  the  vessels. 
Carrying  the  thought  one  step  farther,  Bizzozero  and  Ribbert4  showed 
the  presence  of  bacteria  within  the  normal  mesenteric  gland.  Finally, 
Nicholls5  and  Ford6  have  proved  that  bacteria  can  be  recovered  from 
healthy  organs  by  cultivation.  Recently,  Wrosczek  has  brought  for- 
ward a  pretty  confirmation  of  these  studies.  Feeding  healthy  animals 
on  food  contaminated  with  non-pathogenic  pigmented  microorgan- 
isms, he  regained  these  by  cultures  from  the  internal  organs  without 
there  being  the  slightest  evidence  of  any  lesion  of  the  alimentary  tract. 
These  observations,  taken  together,  prove  beyond  question  that  micro- 
organisms are  constantly  passing  into  the  recesses  of  the  organism  from 
the  gastro-intestinal  mucous  membrane.  These  do  not,  as  a  rule,  lead 
to  infection,  for  they  are  quickly  rendered  inert  by  the  action  of  the 
leukocytes,  the  various  endothelia,  and,  probably,  the  body  juices.  Still, 

1  Brit.  Med.  Jour.,  2: 1890:  491. 

2  Centralbl.  f.  d.  med.  Wiss.,  23: 491. 

3  Jour.  Med.  Research,  11 : 1904:  2. 

4  Deutsche  med.  Woch.,  11 : 1885: 197. 

5  Canadian  Jour,  of  Med.  and  Surg.,  6 : 1899 : 405. 
8  Trans.  Assoc.  Amer.  Phys.,  15:  1900:  389. 


380     THE  DIGESTIVE  FUNCTIONS  AND  THEIR  DISTURBANCES 

a  certain  number  of  them  may  retain  a  limited  degree  of  vitality,  a  poten- 
tiality for  harm  that  on  occasion  may  be  called  into  activity.  This  latency 
of  germs,  termed  by  one  of  us  (J.  G.  A.1)  "subinfection,"  and  noted  also 
by  others,  particularly  Schnitzler,2  probably  explains  those  puzzling  cases 
of  terminal  and  "  cryptogenic"  infection  occasionally  met  with.  Less 
intense  than  this  action,  we  must  recognize,  we  think,  a  "condition 
in  which,  as  a  consequence  of  chronic  inflammatory  disturbances  in 
connection  with  the  gastro-intestinal  tract,  there  may,  for  long  periods, 
pass  in  through  the  walls  of  the  stomach  or  of  the  intestine  a  greater 
number  of  bacteria;  and  while  the  bacteria  undergo  the  normal  and 
inevitable  destruction  by  the  cells  of  the  lymph-glands,  the  liver,  the 
kidneys,  and  other  organs,  nevertheless  the  excessive  action  of  the  cells 
and  the  effect  on  them  of  the  bacterial  toxins  liberated  in  the  process 
of  destruction  may  eventually  lead  to  grave  changes  in  the  cells  and  in 
the  organs  of  which  they  are  part — changes  of  a  chronic  nature."  Prob- 
ably, in  this  way  we  should  explain  many  of  the  forms  of  chronic 
fibrosis  which  occur  so  insidiously  in  the  various  organs. 

1  Jour.  Amer.  Med.  Assoc.,  33:1899:1506  and  1572. 

2  Arch.  f.  klin.  Chir.,  59: 1899:866. 


CHAPTER    XVII. 

THE  MOUTH  AND  ITS  ACCESSORIES. 

THE  MOUTH. 
CONGENITAL  ANOMALIES. 

ABNORMALITIES  in  the  structure  of  the  oral  cavity  and  its  associated 
parts  are  not  infrequent.  Certain  of  them  are  of  great  practical  impor- 
tance, inasmuch  as  they  interfere  with  speech  or  the  proper  manipulation 
of  the  food. 

Astomia,  or  complete  absence  of  the  mouth,  is  very  rare.  It  is  usually 
associated  with  other  defects  of  development,  and  is,  of  course,  incom- 
patible with  life.  The  mouth  may  be  excessively  large  (macrostomia),  or 
exceptionally  small  (microstomia).  The  buccal  cavity  may  be  present 
but  the  external  orifice  wanting  (atresia  oris). 

Abnormal  shortness  of  the  frenum  of  the  tongue  (tongue-tie)  is  by  no 
means  uncommon.  All  newborn  infants  should  be  examined  for  this 
condition,  as  it  may  seriously  interfere  with  nursing.  The  tongue  may 
be  double,  or  cleft  at  the  tip  (snake-tongue). 

Among  the  commonest  and  most  important  anomalies  are  harelip 
and  cleft  palate.  Harelip  is  unilateral  or  bilateral,  the  fissure  or  fissures 
being  situated  to  one  side  of  the  median  line  at  the  lines  of  junction  be- 
tween the  intermaxillary  and  the  supramaxillary  bones.  Various  grades 
of  the  condition  are  met  with,  from  a  slight  notching  of  the  edge  of  the 
lip  to  a  deep  cleft  sometimes  reaching  into  the  nasal  cavity.  The 
defect  may  also  extend  into  the  hard  palate  or  even  into  the  soft  palate. 
In  the  latter  case  the  fissure  assumes  a  median  position. 

Anomalies  in  the  development  of  the  jaws,  such  as  agnathia,  brachy- 
gnathia,  ateloprosopia,  are  occasionally  met  with. 

Defects  in  the  teeth  are  common  but  relatively  unimportant.  Hutchin- 
son  has  described  certain  peculiarities  associated  with  congenital  syphilis. 
The  upper  central  incisors  are  the  teeth  affected.  They  are  peg- 
shaped,  short,  and  thin,  the  top  being  smaller  than  the  crown.  There 
is  a  small  concave  notch  in  the  cutting  edge.  The  affected  teeth  are 
often  yellow  in  color.  The  condition  is  not  absolutely  pathognomonic, 
being  found  in  other  conditions,  notably  rickets. 


CIRCULATORY  DISTURBANCES. 

The  structures  forming  the  buccal  cavity  are  among  the  most  vascular 
in  the  body.     Consequently,  alterations  in  the  quantity  or  the  quality 


382  THE  MOUTH  AND  ITS  ACCESSORIES 

of  the  blood  are  easily  recognized  and  afford  valuable  clinical  evidence 
of  disease. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  occurs  physio- 
logically with  the  act  of  mastication,  but  as  a  pathological  condition  is 
an  evidence  of  local  irritation  or  a  manifestation  of  certain  of  the  infective 
fevers. 

Koplik's  sign  in  measles  is  an  eruption  of  irregular  hyperemic  spots 
of  bright  red  color,  often  having  a  small,  bluish-white  centre,  which 
appears  on  the  buccal  or  labial  mucosa  some  hours  or  even  days  before 
the  appearance  of  the  skin  exanthem.  Occurring  as  it  does  so  early, 
sometimes  even  before  the  catarrhal  symptoms  have  developed,  it  is  a 
valuable  aid  in  diagnosis. 

Passive  Hyperemia. — Passive  hyperemia  is  met  with  especially  in 
obstructive  cardiac  and  pulmonary  affections.  The  lips  and  buccal 
mucosa  assume  a  dull  reddish-purple  color. 

Anemia. — Anemia  may  be  one  manifestation  of  severe  general  anemia. 
The  mucous  membrane  appears  pallid  or  even  quite  bloodless  in  such 
cases.  Anemia  of  the  soft  palate  is  frequently  associated  with  pul- 
monary and  laryngeal  tuberculosis. 


INFLAMMATIONS. 

Inflammation  of  the  lips  is  termed  cheilitis;  of  the  mouth,  stomatitis; 
of  the  tongue,  glossitis;  of  the  gums,  gingivitis. 

Stomatitis. — Catarrhal  Stomatitis,  Cheilitis,  and  Glossitis. — These 
affections  are  usually  brought  about  by  the  action  of  mechanical,  thermic, 
or  chemical  irritants.  Excessive  indulgence  in  alcohol  or  tobacco  are  of 
importance  in  this  connection.  Catarrhal  glossitis  is  a  constant  accom- 
paniment of  all  febrile  conditions,  and  catarrhal  inflammation  of  the  whole 
buccal  mucosa  is  often  observed  in  infectious  fevers.  The  mucous  mem- 
brane of  the  lips,  cheeks,  tongue,  and  alveolar  processes  is  reddened, 
swollen,  and  covered  with  secretion.  The  papillae  of  the  tongue  may 
swell,  giving  it  a  granular  appearance.  The  secretion  contains  leuko- 
cytes and  desquamated  epithelial  cells.  If  it  be  allowed  to  remain, 
the  exudate  collects  upon  the  tongue  and  about  the  roots  of  the  teeth  in 
the  form  of  a  dry,  dirty,  grayish-white,  or  brown  coating  (sordes).  The 
lips  and  tongue  often  become  dry,  fissured,  and  ulcerated. 

Aphthous  Stomatitis. — This  presents  all  the  features  of  the  catarrhal 
form,  but  is  further  characterized  by  the  formation  of  small  grayish  or 
yellowish-white  spots,  either  single  or  in  clusters,  usually  upon  the  lips 
and  tongue.  The  spots  in  question  have  a  dull,  opaque  appearance,  and 
are  bounded  by  a  bright  red  hyperemic  zone.  They  may  coalesce 
and  form  large  patches.  According  to  E.  Fraenkel,  the  process  is  essen- 
tially a  fibrinous  inflammation. 

The  affection  is  usually  found  in  children  who  are  badly  nourished 
and  whose  mouths  are  not  kept  properly  clean.  It  has  been  met  with 
in  women  at  the  menstrual  periods,  during  pregnancy,  and  in  the  puer- 


GANGRENOUS  STOMATITIS  383 

perium.     It  is  not  uncommon  also  in  those  who  have  been  on  protracted 
sprees. 

Ulcerative  Stomatitis. — Ulcerative  stomatitis  is  an  acute  affection, 
rarely  chronic,  which  begins  in  the  gums  near  the  roots  of  the  teeth. 
The  tissues  are  at  first  red,  swollen,  and  oedematous,  and  may  form 
warty  projections.  The  gums  tend  to  loosen  from  the  teeth.  Later, 
the  parts  become  pale,  spongy,  and  friable,  bleeding  at  the  slightest 
touch,  and  eventually  necrosis  sets  in.  The  ulceration  may  extend  to 
the  lips  and  cheeks,  and  may  penetrate  deeply,  leading  to  sequestration 
of  the  bone  of  the  jaw.  The  teeth  not  infrequently  fall  out.  Salivation 
is  a  marked  feature,  and  the  breath  possesses  a  peculiarly  offensive, 
penetrating  odor. 

The  disease  attacks  by  preference  those  who  are  badly  nourished  or 
who  are  weakened  by  long-standing  disease.  It  is  found  in  diabetics, 
in  persons  suffering  from  scurvy,  and  in  those  who  have  been  poisoned 
by  mercury,  phosphorus,  lead,  or  copper. 

Somewhat  similar  to  this  is  the  affection  known  as  pyorrhoea  alveolaris. 
This  disease  is,  however,  more  chronic  in  its  course,  and  is  apt  to  progress 
insidiously  with  occasional  acute  exacerbations.  The  gums  are  swollen, 
reddened,  and  spongy,  bleeding  readily,  and  show  a  tendency  to  retrac- 
tion. Gentle  pressure  will  cause  thin  pus  to  exude  from  about  the  roots 
of  the  teeth.  In  time  the  teeth  loosen  and  fall  out. 

Single  ulcers  under  the  tongue  are  not  uncommon  in  whooping-cough. 
A  local  ulcer,  with  irregular  borders,  situated  near  the  frenum  of  the 
tongue,  occurs  epidemically  and  endemically  in  certain  parts  of  Italy 
(Riga's  disease). 

Suppurative  Stomatitis  and  Glossitis. — Suppurative  processes,  like 
erysipelas,  may  extend  to  the  buccal  cavity  from  without.  Suppurative 
gingivitis  and  glossitis  may  also  result  from  direct  trauma  and  infection. 
They  may  be  met  with  as  complications  in  certain  infective  fevers  and 
in  Bright's  disease.  Suppurative  glossitis  is  a  rare  complication  of 
typhoid  fever.  One  of  us  (A.  G.  N.1)  has  recorded  a  case  of  hemiglossitis 
in  this  disease,  and  Thomas  McCrae  has  published  a  similar  instance 
and  collected  five  others  from  the  literature.2  The  whole  tongue  or 
any  portion  of  it  may  be  affected.  The  condition  may  be  diffuse  or 
phlegmonous,  or  multiple  small  abscesses  may  be  found. 

Gangrenous  Stomatitis. — Gangrenous  stomatitis  (noma;  cancrum  oris ; 
cancer  aquations;  Wasserkrebs)  is  a  peculiarly  rapid  and  fatal  form  of  gan- 
grene affecting  the  face,  almost  certainly  of  infective  nature.  With  few 
exceptions  the  disease  is  met  with  in  badly  fed  and  uncared-for  children, 
especially  when  debilitated  from  disease.  It  not  infrequently  complicates 
one  of  the  acute  infective  fevers.  Rarely,  it  originates  independently,  or 
supervenes  upon  acute  ulcerative  stomatitis.  Children  between  the  ages 
of  two  and  twelve  are  those  usually  attacked. 

affection  begins  with  the  formation  of  a  livid,  swollen  patch  in 

1  Nicholls,  Montreal  Med.  Jour.,  25: 1896: 104. 

2  McCrae,  Johns  Hopkins  Hosp.  Bull.,  9:  1898:  118. 


384 


THE  MOUTH  AND  ITS  ACCESSORIES 


the  buccal  mucous  membrane,  usually  near  the  angle  of  the  mouth,  but 
sometimes  in  the  gums.  Small  blisters  form  and  the  tissues  present 
a  grayish-yellow  inflammatory  infiltration  that  quickly  becomes  gangre- 
nous. The  structures  in  the  neighborhood  are  infiltrated  and  cedema- 
tous.  The  gangrenous  process  quickly  spreads  until  the  whole  thickness 
of  the  cheek  is  converted  into  a  reddish-black  necrotic  material.  The 
condition  is  usually  unilateral,  but  may  extend  to  the  opposite  side,  and 
may  even  penetrate  so  deeply  as  to  involve  the  bones  of  the  nose  and 

jaw.     Septic  infection  of  the  whole 

FIG.  82  system   usually   sets    in    and    death 

soon  results.  In  the  rare  event  of 
healing  taking  place,  the  necrotic 
tissue  separates  and  cicatricial  con- 
traction gradually  ensues,  often  lead- 
ing to  considerable  deformity. 

No  particular  germ  has  as  yet 
been  proved  to  be  the  specific  cause. 
Bishop  and  Ryan  and  Schimmel- 
busch  have  demonstrated  the  pres- 
ence of  a  bacillus  resembling  that  of 
diphtheria  in  some  cases.  It  is  not 
always  to  be  found,  however. 

Specific  Stomatitis.  —  Thrush.— 
Thrush  is  a  mycotic  stomatitis  due  to 
the  action  of  a  special  fungus,  the 
oi'dium  albicans.  It  is  found  usually 
in  infants  during  the  first  year  of  life, 
but  occasionally  also  in  debilitated 
adults.  The  use  of  milk  and  starchy 
(Case  foods,  with  imperfect  cleansing  of 
the  mouth,  favors  the  process.  The 
affection  begins  with  diffuse  redden- 
ing of  the  mucosa  and  the  formation  of  a  glistening,  slimy,  somewhat 
adhesive  exudate  of  grayish  appearance.  Small,  whitish  dots  next  appear, 
which  stand  out  prominently  against  the  hyperemic  background.  These 
patches  gradually  increase  and  may  coalesce  to  form  a  membrane. 
When  this  is  removed,  the  underlying  mucosa  is  greatly  reddened  and 
often  eroded.  The  membrane  quickly  reappears  upon  the  denuded 
surface.  The  disease  usually  begins  on  the  tongue  and  the  inner  sides 
of  the  cheeks,  but  in  bad  cases  may  spread  to  the  palate,  lips,  pharynx, 
oesophagus,  or  even  to  the  stomach  and  intestines.  The  growth  of  the 
fungus  begins  in  the  epithelial  layers  and  extends  to  the  deeper  structures. 
Exceptionally,  mycotic  emboli  may  find  their  way  to  the  internal  viscera. 
Gonorrhceal  stomatitis  has  occasionally  been  observed. 
Diphtheria. — Diphtheria  of  the  mouth  is  usually  secondary  to  the  ordi- 
nary pharyngeal  diphtheria. 

Tuberculosis. — Tuberculosis   of   the  buccal  cavity  may  be  primary, 
but  much  more  often  is  secondary  to  tuberculosis  of  the  lungs  or  larynx, 


Noma  of  the  face  or  cancrum  oris. 
of  Dr.  A   T.  Bazin.) 


SYPHILIS  385 

or  to  lupus  of  the  face.  In  the  secondary  form  the  condition  is  set  up  by 
the  passage  of  infective  sputum  over  the  mucous  membrane.  One  or 
more  small  isolated  nodules  of  grayish-yellow  color  are  found  on  the 
dorsum  of  the  tongue,  usually  near  the  tip,  which  eventually  break  down. 
A  typical  tuberculous  ulcer  of  the  tongue  is  round,  oval,  or  irregular, 
and  painful.  The  edges  are  slightly  indurated  and  raised  above  the 
general  level,  inverted  or  undermined.  The  base  is  uneven  and  nodular, 
and  covered  with  reddish-gray  granulations,  or  a  grayish  or  yellow 
shreddy  slough.  Smaller  tubercles  may  form  about  the  periphery  of 
the  main  ulcer,  which  break  down  and  coalesce  with  it.  Primary  tuber- 
culosis occurs  most  frequently  on  the  tongue,  palate,  and  tonsils. 

Syphilis. — The  primary  chancre  is  occasionally  found  upon  the 
tongue,  lip,  or  tonsil,  but  secondary  and  tertiary  manifestations  are  far 
more  common.  The  mildest  form  of  the  affection  in  the  secondary  stage 
is  a  simple  erythema  or  angina,  but  the  more  characteristic  appearance 
is  the  presence  of  small,  flattened  patches  of  grayish-yellow  color  situated 
on  the  gums  or  near  the  angles  of  the  mouth  (mucous  patches).  Not 
infrequently,  the  superficial  epithelium  assumes  a  peculiar  bluish-white, 
pearly  appearance,  somewhat  resembling  the  corrosion  produced  by 
nitrate  of  silver  (plaques  opalines').  These  are  found  ordinarily  on  the 
lips,  cheeks,  and  tongue,  but  at  times  also  on  the  gums,  tonsils,  and 
pharyngeal  wall.  Such  lesions  may  go  on  to  superficial  ulceration  and 
small  fissures  be  produced.  In  young  children  radiating  scars  at  the 
angles  of  the  mouth  (rhagades)  are  characteristic  of  syphilis.  Gummas 
are  situated  most  frequently  in  the  posterior  wall  of  the  pharynx,  the 
palate,  gums,  and  tongue. 

Gummas  of  the  tongue  generally  occur  about  the  centre  of  the  dorsum, 
and  give  rise  to  deep,  irregular  excavations,  having  thickened,  slightly 
concaved,  or  undermined  edges,  and  a  base  covered  with  yellowish 
slough.  They  are  generally  bounded  by  a  reddish  areola. 

The  ulcerative  lesions  of  tuberculosis,  syphilis,  and  epithelioma, 
affecting  the  tongue,  are  not  unlike  one  another,  and  an  error  of  diagnosis 
may  easily  be  made,  a  mistake  which  will,  of  course,  have  serious  con- 
sequences to  the  patient.  Tuberculous  ulcers  are  usually  situated  on  the 
dorsum  of  the  tongue  near  the  tip  or  toward  the  root;  syphilitic  gum- 
mas,  on  the  dorsum  near  the  middle;  epitheliomatous  ulcers,  usually 
on  the  edge  of  the  tongue,  opposite  the  molar  or  bicuspid  teeth.  Epi- 
thelioma is  more  common  in  men  than  in  women,  and  rarely  occurs 
under  the  age  of  forty.  The  epitheliomatous  ulcer  has  irregular, 
raised,  hard,  and  everted  edges  and  the  tissues  about  it  are  much  indu- 
rated. It  spreads  rapidly,  and  is  attended  with  neuralgic  pain  and  much 
salivation.  Tuberculous  and  tertiary  syphilitic  ulcers  are  not  indurated 
and  the  edges  are  not  everted.  Glandular  involvement  is  not  found, 
while  it  is  common  in  the  case  of  epithelioma.  The  history  often  affords 
a  clue  to  the  nature  of  the  lesion.  In  the  case  of  tuberculosis  of  the  tongue 
there  is  usually  evidence  of  tuberculosis  in  the  lungs  or  elsewhere;  in 
gummas  of  the  tongue,  we  have  a  history  of  specific  infection  and  of 
gummas  in  other  situations;  in  epithelioma,  we  have  the  irritating 
25 


386 


THE  MOUTH  AND  ITS  ACCESSORIES 


influence  of  a  pipe  or  tooth,  or  again,  the  new-growth  may  originate  from 
an  old  scar  or  from  leukoplakia. 

Actinomycosis. — This    disease  is  not   uncommon  in  cattle,    giving 
rise  to  the  condition  known  as  "lumpy  jaw."     The  jaw  becomes  hard 


FIG.  83 


Actinomycosis  ("lumpy  jaw")  of  the  lower  jaw  of  a  cow.      Note  the  overgrowth  of  bone  and 
the  inflammatory  osteoporosis.      (From  the  Pathological  Museum,  McGill  University.) 

FIG.  84 


Actinomycosis  fungus  in  pus.      Fresh,  unstained  preparation.      Magnified  about 
500  diameters.      (Abbott.) 

and  wooden,  or.  in  the  more  acute  forms,  may  be  riddled  with  sinuses. 
Rarefaction  and  hyperplasia  of  the  bone  may  take  place.  The  disease 
is  occasionally  seen  in  man,  and  is  perhaps  more  common  than  has 
been  suspected. 

The  disease  is  due  to  infection  of  the  tissues  with  a  ray  fungus,  the 
actinomyces  bovis.  The  usual  point  of  entrance  is  the  mucous  mem- 
brane of  the  mouth  and  pharynx,  especially  in  the  neighborhood  of 
a  carious  tooth.  Israel  and  Partsch  have  demonstrated  the  presence  of 
the  fungus  in  the  cavities  of  decayed  teeth.  The  actinomyces  is  believe^ 


PROGRESSIVE  METAMORPHOSES 


387 


to  be  present  on  the  stalks  of  certain  kinds  of  grass.  Infection  has  fol- 
lowed the  practice  of  picking  the  teeth  with  a  stalk  of  hay,  or  with  a 
needle.  A  few  instances  are  on  record  in  which  granulomatous  tumors 
have  been  formed  upon  the  tongue  (Claisse1).  When  the  jaw  is  attacked, 
the  appearances  produced  are  not  unlike  those  of  periosteal  sarcoma, 
but  when  the  looser  tissues  of  the  neck  are  reached  growth  is  very  rapid, 
following  the  line  of  the  fascia.  From  the  face  and  neck  the  process 
may  invade  the  meninges  and  the  brain  and  cord,  or  the  thoracic  organs. 
If  an  incision  be  made  into  the  mass,  before  suppuration  has  occurred, 
minute  yellowish  dots  are  to  be  seen,  which  are  the  actinomyces.  If 
there  be  a  discharge,  careful  search  should  be  made  for  the  fungi,  which 
appear  as  yellowish  masses  (sulphur  grains)  the  size  of  a  pin-head  or 
smaller.  These  may  be  picked  out  and  examined  with  a  low-power 
lens,  or  a  film  may  be  made  and  stained  by  Gram's  method.  In  human 
actinomycosis  the  filaments  are  apt  to  lack  the  characteristic  clubbed 
appearance. 

PROGRESSIVE  METAMORPHOSES. 

Occasionally,  the  squamous  epithelium  covering  the  tongue  becomes 
thickened  and  gives  rise  to  plaques  of  a  firm,  somewhat  glistening 
appearance  (leukoplakia  or  psoriasis 

linguae).    Some  attribute  this  condi-  FlQ-  85 

tion  to  chronic  inflammation,  not- 
ably syphilis.  The  affection  occa- 
sionally goes  on  to  the  formation  of 
epithelioma. 

The  lingual  papillae  may  be 
hypertrophied  so  that  the  tongue 
has  a  warty  appearance,  being  lined 
by  intersecting  furrows.  Exception- 
ally, the  filiform  papillae  become  so 
elongated  as  to  resemble  hair,  and 
may  give  the  upper  surface  of  the 
tongue  a  greenish  or  blackish  furry 
appearance  (so-called  hairy  tongue). 

Tumors. — Hemangioma  and  lym- 
phangioma  are  met  with  in  early 
life.  The  former  is  usually  found  on 
the  lips,  where  it  forms  bluish-red, 
somewhat  elevated,  blotches.  The 
so-called  macroglossia  and  macro- 
cheilia  are  examples  of  diffuse  lym- 

£       .  *  Macroglossia.       (Dr.  Shepherd  s  case,  Montreal 

phangiectasis  affecting  the  tongue  General  Hospital.) 

and  lips  respectively.    These  struc- 
tures are   greatly   enlarged,  owing  to   increase   in  all  the  component 
elements,  fibrosis,  or,  again,  actual  new-growth  of  lymph-vessels.     In 

1  Presse  Med.,  Paris,  1897:  789. 


388 


THE  MOUTH  AND  ITS  ACCESSORIES 


macroglossia  the  tongue  may  be  so  much  enlarged  that  it  projects 
beyond  the  lips.  It  often  becomes  dry,  fissured,  and  ulcerated,  owing 
to  exposure  to  the  air  and  the  pressure  of  the  teeth.  From  its  size  it 
may  interfere  with  feeding  and  respiration.  Microscopically,  the  organ 
shows  increase  in  fibrous  tissue  and  contains  numerous  small  cavities 
lined  with  endothelium — the  dilated  lymph-channels.  Actual  cysts  may 
be  formed.  Among  the  benign  new-growths  appearing  at  birth  or  soon 
after  may  be  mentioned  the  fibroma,  lipoma,  myxoma,  and  teratoma. 

Teratomas  usually  develop  from  the  palate  or  vault  of  the  pharynx. 
They  arise  either  from  embryonic  "cell  inclusions"  or  are  to  be  regarded 
as  examples  of  polar  hypergenesis  (vol.  i,  p.  218).  Tumors  having  the 
structure  of  thyroid  substance  occasionally  have  been  met  with  in  the 
base  of  the  tongue,  originating  from  "rests"  of  thyroid  cells  situated 
along  the  course  of  the  foetal  thyroglossal  duct. 


FIG.  86 


Giant-celled  sarcoma,  from  the  periosteum  of  the  jaw.     Winckel  No.  6,  without  ocular. 

The  malignant  tumors,  sarcoma  and  carcinoma,  are  more  common  in 
adult  life.  The  term  epulis  is  a  clinical  one  applied  to  a  tumor  situated 
on  the  jaw,  which  springs  usually  from  the  gums.  Some  of  them  have 
the  structure  of  a  fibroma  (fibrous  epulis);  others  are  sarcomatous 
(myeloid  epulis);  still  others  are  epitheliomatous  (epitheliomatous  epulis). 
Myeloid  or  giant-celled  sarcomas  originate  in  the  periosteum  or  bone 
marrow,  and  form  rounded,  nodular  growths  of  rather  firm  consistence. 
On  section,  they  often  have  a  brick-red  color,  owing  to  hemorrhage. 
Other  forms  of  sarcomas  are  not  infrequently  met  with. 


PROGRESSIVE  METAMORPHOSES 


389 


Epithelioma  is  found  upon  the  lip,  tongue,  or  gums.     It  begins  as  a 
small,  elevated  papule,  or  as  a  firm,  circumscribed,  whitish-gray  infiltra- 


FIG.  87 


Epithelioma,  starting  from  the  lip.     Winckel  obj.  No.  3,  without  ocular. 
(From  the  collection  of  Dr.  A.  G.  Nicholls). 

FIG.  88 


Epithelial  pearl  or  "cell-nest"  from  an  epithelioma  of  the  lip.     Winckel  No. 
without  ocular.     (From  Dr.  A.  G.  Nicholls'  collection.) 


388 


THE  MOUTH  AND  ITS  ACCESSORIES 


macroglossia  the  tongue  may  be  so  much  enlarged  that  it  projects 
beyond  the  lips.  It  often  becomes  dry,  fissured,  and  ulcerated,  owing 
to  exposure  to  the  air  and  the  pressure  of  the  teeth.  From  its  size  it 
may  interfere  with  feeding  and  respiration.  Microscopically,  the  organ 
shows  increase  in  fibrous  tissue  and  contains  numerous  small  cavities 
lined  with  endothelium — the  dilated  lymph-channels.  Actual  cysts  may 
be  formed.  Among  the  benign  new-growths  appearing  at  birth  or  soon 
after  may  be  mentioned  the  fibroma,  lipoma,  myxoma,  and  teratoma. 

Teratomas  usually  develop  from  the  palate  or  vault  of  the  pharynx. 
They  arise  either  from  embryonic  "cell  inclusions"  or  are  to  be  regarded 
as  examples  of  polar  hypergenesis  (vol.  i,  p.  218).  Tumors  having  the 
structure  of  thyroid  substance  occasionally  have  been  met  with  in  the 
base  of  the  tongue,  originating  from  "rests"  of  thyroid  cells  situated 
along  the  course  of  the  foetal  thyroglossal  duct. 


FIG.  86 


Giant-celled  sarcoma,  from  the  periosteum  of  the  jaw.     Winckel  No.  6,  without  ocular. 

The  malignant  tumors,  sarcoma  and  carcinoma,  are  more  common  in 
adult  life.  The  term  epulis  is  a  clinical  one  applied  to  a  tumor  situated 
on  the  jaw,  which  springs  usually  from  the  gums.  Some  of  them  have 
the  structure  of  a  fibroma  (fibrous  epulis);  others  are  sarcomatous 
(myeloid  epulis);  still  others  are  epitheliomatous  (epitheliomatous  epulis). 
Myeloid  or  giant-celled  sarcomas  originate  in  the  periosteum  or  bone 
marrow,  and  form  rounded,  nodular  growths  of  rather  firm  consistence. 
On  section,  they  often  have  a  brick-red  color,  owing  to  hemorrhage. 
Other  forms  of  sarcomas  are  not  infrequently  met  with. 


PROGRESSIVE  METAMORPHOSES 


389 


Epithelioma  is  found  upon  the  lip,  tongue,  or  gums.     It  begins  as  a 
small,  elevated  papule,  or  as  a  firm,  circumscribed,  whitish-gray  infiltra- 


FIG.  87 


Epithelioma,  starting  from  the  lip.     Winckel  obj.  No.  3,  without  ocular. 
(From  the  collection  of  Dr.  A.  G.  Nicholls). 

FIG.  88 


Epithelial  pearl  or  "cell-nest"  from  an  epithelioma  of  the  lip.     Winckel  No.  6, 
without  ocular.     (From  Dr.  A.  G.  Nicholls1  collection.) 


390  THE  MOUTH  AND  ITS  ACCESSORIES 

tion  of  the  tissues.  The  surface  ulcerates  and  the  ulcer  quickly  enlarges, 
invading  the  neighboring  structures  and  the  regional  lymph-nodes. 
Epithelioma  of  the  upper  jaw  has  a  special  tendency  to  involve  the 
antrum.  Histologically,  epitheliomas  are  of  the  squamous-celled  variety. 
Finger-like  down-growths  of  the  superficial  epithelium  are  to  be  seen, 
which  are  united  by  lateral  processes  in  such  a  way  as  to  form  a  sort 
of  meshwork.  Here  and  there,  epithelial  "pearls"  or  cell-nests  may  be 
seen.  Round-celled  infiltration  is  common. 

Cysts. — Cysts  are  not  uncommon  in  the  mouth.  They  are  usually 
of  the  nature  of  "retention"  cysts  and  are  caused  by  the  blocking  of  the 
duct  of  one  or  other  of  the  glands  discharging  into  the  buccal  cavity. 
Dermoid  cysts  are  also  met  wth. 

THE  TEETH. 

Caries. — Caries  is  the  most  important  affection.  This  begins  with 
the  formation  of  an  opaque,  white,  or,  more  often,  greenish  or  greenish- 
black  speck  upon  the  enamel,  the  result  of  disintegration  and  destruction 
of  the  enamel  prisms.  The  process,  if  not  interfered  with,  steadily 
advances  until  the  centre  of  the  tooth  is  excavated,  and  the  whole  tooth 
eventually  undergoes  decalcification.  It  thus  becomes  soft  and  is  apt 
to  break.  Very  commonly  caries  is  accompanied  by  inflammation  of  the 
pulp  (pulpitis),  or  of  the  alveolar  periosteum. 

FIG.  89 


Head  of  a  woodchuck:  showing  hypertrophy  of  the  incisor  teeth  from  lessened  wear  ; 
the  cause,  fracture  of  the  lower  jaw.     (Pathological  Museum,  McGill  University.) 

Hypertrophy. — Excessive  growth  of  the  teeth  occurs  as  a  result  of 
insufficient  attrition.  Loss  of  the  opposing  teeth  or  mal-apposition,  as 
from  fracture  of  the  jaw,  are  the  usual  conditions  at  work.  The  tusks 
of  the  wild  boar  are  a  familiar  example. 

Tumors. — Among  tumors  may  be  mentioned  the  odontoma,  odontin- 
oids,  fibroma,  myxoma,  and  sarcoma.  The  odontoma  is  formed  during 
the  period  of  growth  and  arises  from  the  pulp  or  forms  excrescences 
about  the  crown  or  root.  Odontinoids  develop  later  in  life  from  the 
dentin.  Sarcoma  and  the  other  connective-tissue  tumors  usually  arise 
from  the  periosteum  of  the  jaw  or  about  the  teeth.  Rarely,  they  start 


PALATE,  PHARYNX,  AND  TONSILS  391 

from  the  pulp,  (luring  the  period  of  development.  Falkson  has  called 
attention  to  a  multilocular  cyst  or  cystadenoma,  lined  with  cylindrical 
epithelium  and  containing  teeth,  which  develops  from  embryonal  tooth 
follicles. 

INFLAMMATION. 

Inflammation  may  occur  in  the  pulp  (pidpitis)  or  around  the  root 
of  the  tooth.  The  condition  is  due  to  infection.  According  to  Miller, 
acid  fermentation  of  the  particles  of  food  clinging  about  the  teeth  assist 
the  pathogenic  action  of  the  bacteria.  Pepsin  and  various  vegetable 
acids  are  said  to  be  responsible  for  the  destruction  of  the  dentin 
(Schlenker).  The  inflammation  may  be  acute  or  chronic,  suppurative, 
or  non-suppurative.  Acute  suppuration  may  occur  in  the  pulp  of  the 

FIG.  90 


Odontoma.     (Garretson.) 

tooth  or  deep  down  in  the  periosteum  surrounding  it.  It  leads  to 
inflammation  of  the  alveolar  process  and  the  formation  of  a  local  abscess. 
If  not  relieved,  fistulse,  necrosis  of  the  bone,  phlegmon  of  the  neck  and 
floor  of  the  mouth,  or  even  general  septicemia  may  result.  In  chronic, 
non-suppurative  inflammation  of  the  pulp  and  periosteum,  granulation 
tissue,  new  bone,  and  dentin  may  be  produced. 


PALATE,  PHARYNX,  AND  TONSILS. 

The  mucous  membrane  of  the  palate,  pharynx,  and  tonsils  is  not 
unlike  that  of  the  other  portions  of  the  buccal  cavity,  but  has  these 
peculiarities,  namely,  that  its  character  as  a  mucous  membrane  is  still 
more  pronounced  and  that  it  is  particularly  rich  in  lymphoid  elements, 
both  in  the  shape  of  follicles  and  the  larger  aggregations  known  as  the 
tonsils.  Consequently,  exudative  processes  are  of  more  importance 
than  are  desquamative  ones. 

The  tonsils  appear  to  have  an  important  function.  While  the  lym- 
phoid cells  are  themselves,  to  a  limited  degree,  phagocytic,  polymorpho- 
nuclear  leukocytes  in  considerable  numbers  make  their  way  from  the 
bloodvessels  to  the  surface  through  the  epithelial  covering.  These 
leukocytes  are  strongly  phagocytic  and  their  activity  suggests  that  the 


392  THE  MOUTH  AND  ITS  ACCESSORIES 

tonsils  form  one  of  the  barriers  against  the  invasion  of  the  system  by 
pathogenic  microorganisms. 


INFLAMMATIONS. 

Pharyngitis. — The  term  angina  or  pharyngitis  is  a  general  one  used 
to  designate  inflammation  of  the  posterior  part  of  the  buccal  cavity, 
pharynx,  tonsils,  and  palate.  The  condition  is  comparatively  common, 
and  is  usually  the  result  of  irritation  from  mechanical,  thermic,  or 
chemical  agents.  It  is  found  also  in  association  with  certain  of  the 
infectious  diseases,  such  as  scarlatina,  measles,  acute  rheumatism, 
diphtheria,  and  variola.  When  the  tonsils  are  chiefly  or  alone  involved 
the  affection  is  called  tonsillitis,  or  amygdalitis. 

The  inflammations  of  this  region  may,  according  to  their  severity 
and  duration,  be  divided  into  acute  and  chronic;  or,  according  to  their 
morbid  peculiarities,  into  catarrhal,  herpetic,  phlegmonous,  and  mem- 
branous. 

Acute  Catarrhal  Pharyngitis. — Acute  catarrhal  pharyngitis,  or  angina, 
is  characterized  by  redness  and  swelling  of  the  mucous  membrane,  which 
becomes  glazed  owing  to  the  inhibition  of  the  secretion.  Later,  there 
is  an  abundant  discharge  of  a  mucoid  or  mucopurulent  exudate,  some- 
times tinged  with  blood.  Microscopically,  the  secretion  contains 
mucus,  pus  corpuscles,  blood  cells,  and  desquamated  and  degenerated 
epithelium.  In  the  more  severe  cases,  small  abrasions  may  be  noted 
on  the  back  of  the  pharynx.  The  lymph-follicles  are  often  also  hyper- 
plastic  and  appear  as  small,  rounded  or  oval,  elevated,  and  reddish 
nodules,  projecting  through  the  membrane. 

Phlegmonous  Pharyngitis. — In  this  form  the  inflammatory  process  is 
less  marked  upon  the  surface  than  in  the  deeper  parts.  The  cases  are 
due  to  infection  with  pyogenic  microorganisms,  in  some  instances  assisted 
by  traumatism.  Not  infrequently,  they  arise  as  secondary  complications 
of  certain  of  the  infective  diseases,  such  as  scarlatina,  diphtheria,  ery- 
sipelas, and  syphilis.  The  mucosa  is  of  a  deep,  purplish-red  color, 
swollen,  tense,  and  shiny,  presenting  occasionally  superficial  vesicles. 
In  the  erysipelatous  form  the  infiltration  is  diffuse  and  the  exudate  sero- 
purulent  rather  than  purulent.  In  other  cases  the  process  is  localized 
and  quite  large  abscesses  may  form.  Retropharyngeal  abscess  sometimes 
results  from  tuberculous  caries  of  the  cervical  vertebrae.  Peritonsillar 
abscess  is  a  common  complication  of  acute  tonsillitis  and  may  occur  on 
one  or  both  sides.  Such  collections  of  pus  may  burst  into  the  pharynx, 
or  may  erode  the  internal  carotid  artery  or  one  of  its  branches,  and  cause 
fatal  hemorrhage,  or,  again,  if  deep  down  may  be  absorbed  with  the 
formation  of  a  scar.  If  the  discharge  of  the  abscess  occur  during  the 
night,  suffocation  may  result.  General  septicemia  may  also  occur. 
Gangrene  is  a  rare  terminal  event. 

Membranous  Pharyngitis. — Membranous  or  "croupous"  pharyngitis 
may  arise  directly  from  traumatism,  as,  for  example,  the  inhalation  of 


DIPHTHERIA  393 

irritating  gases  (steam,  ammonia),  but  much  more  commonly  it  is  due 
to  infection  with  pathogenic  microorganisms,  such  as  the  Streptococcus 
pyogenes,  Bacillus  diphtherise,  Pneumococcus,  and  Bacillus  coli.  Apart 
from  diphtheria,  membranous  pharyngitis  is,  as  a  rule,  met  with  as  a 
complication  of  certain  infective  fevers,  such  as  scarlatina,  measles, 
typhoid,  and  variola. 

Diphtheria. — True  diphtheria  may  be  taken  as  the  type  of  this  form 
of  pharyngitis.  Here,  the  inflammation  is  due  to  the  action  of  the 
Klebs-Loeffler  bacillus.  The  affection  begins  with  marked  congestion 
and  swelling  of  the  mucosa,  which  quickly  assumes  the  character  of  a 
membranous  inflammation.  In  the  earlier  stages  small,  grayish  or 
grayish-white  opalescent  spots  appear  on  the  tonsils,  uvula,  or  other  parts 
of  the  pharyngeal  wall.  These  gradually  become  distinctly  membranous 
and  of  a  dirty  yellow  or  yellowish-brown  color.  They  are  bounded  by 
a  hyperemic  zone,  and  may  be  slightly  separated  and  elevated  at  the 
edges  from  the  underlying  tissues.  If  the  membrane  be  removed  a 
superficial  erosion  is  left,  which  bleeds  readily.  On  this  area  the  mem- 
brane may  reform  with  great  rapidity.  In  the  severer  cases  the  patches 
become  confluent,  and  thick,  laminated  sheets  of  membrane  are  formed 
on  the  posterior  portion  of  the  pharynx,  which  may  extend  into  the  trachea, 
larynx,  and  bronchi,  into  the  nasal  passages,  or  even  to  the  skin.  In 
the  most  virulent  cases  extensive  necrosis  takes  place,  and  large  gangrenous 
excavations  may  be  found  about  the  pillars  of  the  fauces.  The  tonsils 
and  lymph-nodes  of  the  neck  are  usually  enlarged. 

By  examining  a  series  of  sections  taken  at  different  periods,  it  has  been 
made  out  that  the  mucous  membrane  is  at  first  congested  and  more 
or  less  infiltrated  with  inflammatory  products;  the  superficial  epithelium 
degenerates  and  is  cast  off  either  wholly  or  in  part;  the  cellular  exudate 
coagulates,  forming  a  fibrinous  deposit  both  in  the  superficial  layers  and 
on  the  exposed  surface  of  the  part  affected.  This  material,  composed  of 
leukocytes,  fibrin,  and  degenerating  tissue,  gradually  undergoes  a  form 
of  coagulation  necrosis  and  fuses  into  a  more  or  less  homogeneous  mass, 
the  diphtheritic  membrane.  In  the  deeper  parts  the  bloodvessels  and 
lymphatics  are  distended,  there  are  numerous  areas  of  cellular  and 
fibrinous  infiltration,  and  the  glands  are  blocked  with  exudate  and  desqua- 
mated cells.  In  cases  that  recover,  the  membrane  is  exfoliated  in  large 
shreds  or  en  masse,  especially  when  antitoxin  has  been  used,  or,  again, 
may  be  gradually  absorbed.  The  lost  epithelium  is  regenerated,  and 
healing  takes  place  without  scarring. 

The  specific  bacilli  are  usually  superficial  and,  as  a  rule,  do  not  enter 
the  circulation,  at  least  to  any  great  extent.  Severe  symptoms  are, 
however,  not  infrequently  present,  resulting  from  absorption  of  the 
toxin.  Death  may  occur  during  the  active  stages  of  the  disease  or  during 
convalescence,  from  vagus  paralysis.  A  commoner  form  of  paralysis 
is  that  affecting  the  muscles  of  the  throat,  especially  those  of  the 
palate.  Paralysis  of  accommodation,  monoplegia,  hemiplegia,  and 
paraplegia  may  occur.  In  the  more  intense  cases  of  intoxication  prac- 
tically all  the  muscles  of  the  body  may  be  involved  with  the  exception 


394  THE  MOUTH  AND  ITS  ACCESSORIES  . 

of  the  diaphragm.  Another  complication  of  diphtheria  is  abscess  forma- 
tion, the  result  of  secondary  infection  by  pyogenic  microorganisms. 

The  membranous  pharyngitis  due  to  streptococci  is  likely  to  be  more 
acute  than  diphtheria  in  regard  to  its  immediate  symptoms.  It  is  not 
followed  by  paralyses,  but  the  cocci  penetrate  deeply  and  are  apt  to  be 
carried  to  distant  parts,  setting  up  a  generalized  septicemia  or  multiple 
abscess  formation. 

Chronic  Pharyngitis. — Chronic  pharyngitis  may  arise  insidiously  or 
may  result  from  a  succession  of  acute  attacks.  The  abuse  of  tobacco 
and  alcohol  are  common  causes.  Many  cases,  too,  represent  the  exten- 
sion of  a  chronic  rhinitis.  The  mucous  membrane  is  more  or  less 
congested  and  presents  numerous  distended  venules.  Often,  it  has  a 
granular,  warty  appearance,  due  to  hyperplasia  of  the  lymphoid  follicles 
(chronic  granular  pharyngitis).  The  secretion  is  mucoid,  mucopurulent, 
or  purulent,  and  often  adheres  in  the  form  of  dry  scales  or  crusts,  which 
decompose  and  emit  an  offensive  odor.  In  other  cases,  secretion  is 
scanty,  and  the  mucous  membrane  is  of  a  reddish-brown  color,  thin, 
smooth,  and  shiny.  This  is  known  as  chronic  atrophic  pharyngitis 
(pharyngitis  sicca). 

Acute  Tonsillitis. — In  acute  tonsillitis  the  inflammation  may  be  a 
superficial  one  or,  again,  may  involve  the  parenchyma  of  the  glands, 
causing  considerable  swelling  of  the  parts.  The  tonsils  are  swollen,  red, 
and  hot,  and  covered  with  an  abundant  secretion  of  creamy  mucopus. 
When  the  tonsillar  crypts  are  distended  with  secretion,  yellowish-white, 
rounded  spots  can  be  seen  on  the  surface  of  the  tonsils,  and  the  condition 
is  then  commonly  known  as  follicular  or  lacunar  tonsillitis.  In  severe 
cases,  the  inflammatory  process  may  go  on  to  suppuration,  either  in  the 
tonsil  or  in  the  cellular  tissue  about  it  (quinsy,  peritonsillar  abscess). 

Histologically,  in  acute  tonsillitis,  we  find  marked  congestion  of  the 
glands,  hyperplasia  of  the  lymphoid  elements,  exudation  on  the  surface, 
and  desquamation  of  the  epithelium. . 

Occasionally,  in  addition  to  the  ordinary  manifestations  of  catarrhal 
inflammation,  minute  vesicles  are  formed  upon  the  mucous  membrane, 
which  rupture,  leaving  painful  ulcers — herpetic  tonsillitis  or  pharyngitis. 

Chronic  Tonsillitis. — In  chronic  tonsillitis  the  organs  are  permanently 
enlarged  and,  except  for  their  size,  often  appear  to  be  normal.  Some- 
times there  is  a  dusky  redness  of  the  palate  and  fauces.  The  crypts  are 
small  and  the  surface  smooth.  The  enlargement  may  be  so  great  as  to 
interfere  with  swallowing,  respiration,  and  speaking,  and  is  important 
also  in  that  it  tends  to  perpetuate  the  inflammation,  owing  to  the  in- 
creased susceptibility  of  such  tonsils  to  irritation.  Children  with  a 
rheumatic  taint  seem  to  be  specially  liable  to  this  disorder.  Chronic 
tonsillitis  and  pharyngitis  predispose  to  bronchitis  and  other  lung  affec- 
tions. 

In  another  form  of  chronic  tonsillitis  the  tonsils  are  not  enlarged, 
but  the  crypts  are  more  roomy  than  normal  and  are  filled  with  secretion, 
desquamated  cells,  food  particles,  and  microorganisms  of  various  kinds. 
This  material  becomes  inspissated  and  may  be  extruded  spontaneously 


PAROTITIS  395 

or  by  pressure  in  the  form  of  cheesy,  whitish-yellow  plugs,  which  have 
a  peculiar,  characteristic,  offensive  odor.  Sometimes  the  masses  are 
retained  and  become  infiltrated  with  lime  salts,  forming  concretions. 

Hyperplasia  of  the  tonsils  and  of  the  pharyngeal  or  Luschka's  tonsil  is 
often  met  with  in  children,  and  seems  to  be  in  many  cases  the  result  of 
a  chronic  inflammatory  process.  Adenoid  vegetations  are  papillomatous 
or  polypoid  growths  occurring  in  the  vault  of  the  pharynx  and  posterior, 
nares.  When  of  any  extent,  breathing  may  be  interfered  with,  and 
children  so  affected  complain  of  headache  and  earache,  breathe  through 
the  mouth,  and  are  apathetic  and  backward  at  school.  The  condition 
is  often  associated  with  enlargement  of  the  pharyngeal  tonsil  and  sore 
throat.  Deafness  and  inflammation  of  the  middle  ear  may  result  from 
interference  with  the  Eustachian  tubes  through  pressure  or  catarrh.  In 
long-standing  cases  the  chest  shows  a  characteristic  deformity. 

Histologically,  the  condition  is  due  to  hyperplasia  either  of  the  lymphoid 
elements  or  of  the  connective  tissue.  In  the  former  case  the  adenoids 
are  soft  and  friable;  in  the  latter,  firm  and  tough. 

Tuberculosis. — Tuberculosis  of  the  tonsils  and  pharynx  can  occur 
as  a  primary  infection,  but  is  almost  always  secondary  to  pulmonary 
or  laryngeal  tuberculosis.  Owing  to  their  exposed  position  the  tonsils 
are  liable  to  infection  of  all  kinds,  especially  from  the  food,  and  it  has  been 
possible  to  produce  experimentally  tuberculosis  in  these  glands  by  feeding 
animals  on  infected  material.  In  tuberculosis  of  the  pharynx  the  mucosa 
is  injected  and  contains  numerous  small  tubercles,  that  ultimately  break 
down  and  form  shallow  ulcers.  These  may  in  time  coalesce  and  lead 
to  extensive  loss  of  substance. 

Syphilis. — Syphilitic  sore  throat  usually  takes  the  form  of  a  catarrhal 
inflammation  that  is  difficult  to  distinguish  from  the  simple  variety, 
or  of  mucous  or  opaline  plaques.  Gummas  are  not  uncommon.  The 
primary  chancre  has  been  found  upon  the  tonsil. 

Typhoid. — Superficial  ulceration  of  the  posterior  pharyngeal  wall 
is  not  very  uncommon  in  typhoid  fever. 

Actinomycosis. — Cervical  and  prevertebral  actinomycosis  may 
originate  in  the  tonsils  and  the  pharyngeal  mucous  membrane. 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — Connective-tissue  tumors,  carcinoma,  sarcoma,  and  teratoma 
occur  in  this  region.     Sarcoma  of  the  tonsil  is  occasionally  met  with. 


THE  SALIVARY  GLANDS. 

These  are  glands  of  acinous  structure,  and  in  the  human  subject  are 
of  serous  or  mixed  serous  and  mucous  type.  Their  ducts  discharge  into 
the  buccal  cavity. 

Parotitis. — Acute  Parotitis. — Acute  parotitis  and  analogous  inflam- 
mations of  the  other  salivary  glands  are  usually  due  to  infection  with 


396  THE  MOUTH  AND  ITS  ACCESSORIES 

microorganisms  from  the  mouth.  They  may  also  complicate  many  of 
the  infective  fevers,  such  as  typhoid,  diphtheria,  pyemia,  cholera,  and 
syphilis.  Parotitis  has  been  observed  as  a  complication  of  certain 
abdominal  conditions  and  after  operations  on  the  abdominal  viscera. 
When  pyogenic  organisms  are  at  work  suppuration  of  the  glands  may 
follow,  or  even  gangrene.  In  such  cases  salivary  fistula  sometimes 
result.  Milder  inflammations  may  lead  to  increased  secretion  and  later 
to  fibrous  induration  of  the  gland  with,  possibly,  stenosis  of  the  duct. 
In  such  cases  concretions  composed  of  phosphate  or  carbonate  of  lime 
are  not  uncommonly  found  within  the  duct  (sialoliths),  sometimes 
causing  or  associated  with  cystic  dilatation  of  the  ducts  and  acini. 

Epidemic  Parotitis. — Epidemic  parotitis  (mumps)  is  an  infectious 
disease,  characterized  by  great  swelling  of  the  parotid  gland  and  to  some 
extent  of  the  submaxillary  and  sublingual  glands,  associated  with  slight 
febrile  disturbance.  The  infecting  agent,  which  has  not  as  yet  been 
absolutely  determined,  presumably  enters  through  the  excretory  duct. 
In  the  course  of  two  or  three  days  the  inflammation  subsides  and  the 
gland  gradually  resumes  its  normal  state.  The  inflammatory  exudate  is 
mainly  serous.  Suppuration  rarely  occurs.  The  affection  is  occasionally 
complicated  by  orchitis  or  oophoritis. 

Angina  Ludovici. — Angina  Ludovici  is  a  somewhat  rare  and  peculiar 
form  of  phlegmon  or  septic  cellulitis,  occurring  in  the  floor  of  the  mouth 
and  sides  of  the  neck.  The  affected  parts  are  swollen,  dusky  red  in 
color,  and  present  a  brawny  induration.  Abscess  formation  and  gangrene 
may  supervene.  Cases  are  not  infrequently  fatal  from  general  septi- 
cemia.  Angina  of  this  type  may  originate  in  inflammation  of  the  sub- 
maxillary  gland,  but  is  more  common  as  a  result  of  trauma  or  infection 
from  carious  teeth.  Cases  occasionally  are  met  with  in  scarlatina. 

Parasites. — Parasites  are  rare.     Echinococcus  has  been  recorded. 

Tumors. — Among  tumors  of  the  parotid  may  be  mentioned  fibroma, 
myxoma,  chondroma,  adenoma,  rhabdo myoma,  sarcoma,  endothelioma, 
and  carcinoma.  The  most  common  tumor  of  the  parotid  gland  is  mixed 
in  character,  consisting  of  chondroma  together  with  fibrous  and  myxo- 
matous  elements,  and  is  probably  to  be  attributed  to  the  overgrowth  of 
misplaced  embryonic  tissue.  It  has  a  distinct  tendency  to  undergo 
sarcomatous  transformation.  Carcinoma  is  rare. 


CHAPTEE    XVIII. 

THE  (ESOPHAGUS. 

AFFECTIONS  of  the  oesophagus  are  relatively  uncommon.  Some  of 
them  are  of  great  clinical  importance,  however,  inasmuch  as  they  may 
interfere  with  the  passage  of  food  into  the  stomach  and  thus  lead  to 
malnutrition.  Owing  to  the  special  function  of  the  oesophagus  it  is 
peculiarly  liable  to  suffer  from  the  effects  of  mechanical,  chemical, 
and  thermal  irritation.  It  may  also  be  affected  by  disease  of  neighbor- 
ing structures — larynx,  trachea,  mediastinal  and  peribronchial  glands, 
and  vertebrae. 

CONGENITAL  ANOMALIES. 

Malformations  may  occur  alone  or  associated  with  other  defects. 
The  most  common  event  is  for  the  upper  third  of  the  tube  to  end  in  a 
blind  cul-de-sac,  often  dilated,  while  the  lower  portion  forms  a  fistulous 
communication  with  the  trachea  or  a  bronchus.  Any  part  of  the  tube 
may  be  absent  either  completely  or  the  defective  portion  may  be  repre- 
sented by  a  fibrous  cord.  Local  areas  of  stenosis  have  been  met  with. 
Rarely,  the  lumen  is  partially  occluded  by  a  diaphragm-like  fold  of 
mucous  membrane. 

A  localized  dilatation  of  the  oesophagus,  the  so-called  "fore-stomach" 
or  "antrum  cardiacum"  has  been  observed.  It  is  decidedly  rare.  In 
acardiac  monsters  the  oesophagus  may  be  completely  wanting.  Partial 
or  complete  reduplication  is  found  in  double  monsters. 


ALTERATIONS  OF  THE  LUMEN  AND  SOLUTIONS  OF  CONTINUITY. 

Simple  contracture  of  the  lumen  of  the  oesophagus  from  spasm  is 
occasionally  observed  in  hysterical  persons  and  hypochondriacs,  and 
also  in  chorea,  epilepsy,  and  hydrophobia.  Stenosis  of  the  oesophagus 
may  be  developmental  or  acquired.  In  the  latter  form  the  lumen  may 
be  narrowed  from  extrinsic  or  intrinsic  causes.  Thus,  pressure  from 
enlarged  mediastinal  glands,  aneurisms,  tumors  of  the  lung  and  pleura 
may  bring  it  about.  Local  inflammatory  swellings,  phlegmon,  growths 
of  the  thrush  fungus,  tumors,  cicatricial  contraction  of  the  wall  from 
trauma,  corrosive  poisoning,  syphilis,  and  diphtheria  are  among  the 
intrinsic  causes.  Foreign  bodies  may  also  lodge  in  the  tube  and  obstruct 
its  lumen. 


398  THE  (ESOPHAGUS 

Dilatation. — Dilatation  may  be  developmental  or  acquired.  The 
first-mentioned  variety  is  rare.  The  condition  is  general  throughout 
the  tube. 

Acquired  dilatation  is  usually  secondary  to  stenosis  and  may  be 
general  or  local.  Generalized  dilatation  is  either  cylindrical  or  fusi- 
form and  results  from  stricture  or  chronic  oesophagitis.  The  oesophagus 
may  be  enormously  dilated,  the  lumen  measuring  30  cm.  or  more  in 
circumference.  The  muscular  coats  usually  hypertrophy  in  these  cases. 
Rarely,  "  idiopathic"  dilatation,  without  stenosis,  is  observed.  It  has  been 
attributed  to  spa'sm  of  the  muscle  at  the  cardiac  end,  or  to  relaxation 
of  the  muscular  fibers  of  the  wall.  A  similar  condition  can  be  pro- 
duced experimentally  in  the  dog  by  cutting  both  vagi  in  the  neck. 
Kraus1  has  described  a  case  of  this  kind  in  the  human  subject,  where, 
at  autopsy,  the  vagi  were  found  to  be  diseased. 

Local  dilatations  or  di vertical  a  are  of  two  kinds,  pressure  diverticula 
and  traction  diverticula.  The  first  form  is  due  to  pressure  from  within 
the  lumen;  the  second,  to  external  force  pulling  out  the  wall. 

Pressure  diverticula  are  rare.  They  generally  are  situated  on  the 
posterior  wall  at  the  junction  of  the  pharynx  and  oesophagus,  at  the 
point  where  the  muscular  wall  is  normally  weakest.  Local  bulging  is 
first  brought  about  by  trauma,  the  swallowing  of  large  boluses  of  food, 
which  gradually  increases  until  a  sac  is  formed  extending  downward 
between  the  oesophagus  and  the  vertebrae.  Microscopic  examination 
shows  that,  as  a  rule,  the  muscular  fibers  are  lacking  in  the  wall  of  the 
sac,  so  that  the  condition  might  be  regarded  as  a  hernial  protrusion  of 
the  mucosa  and  submucosa  through  the  muscularis.  In  some  few  cases 
the  muscular  fibers  have  been  found  to  be  continuous  in  the  wall.  In 
the  latter  case  the  condition  is  usually  regarded  as  an  ectasia  due  to  a 
disturbance  of  the  closure  of  the  fetal  cleft  at  this  point.  Food  tends 
to  enter  the  sac  and  become  lodged  there,  where  it  decomposes,  thus 
giving  rise  to  maceration  of  the  epithelium,  ulceration,  inflammation  of 
the  oesophagus  and  neighboring  structures.  Pressure  symptoms  on  the 
rest  of  the  oesophagus  are  common. 

Traction  diverticula  are  not  uncommon.  They  are  usually  to  be  found 
on  the  anterior  wall  about  the  level  of  the  bifurcation  of  the  trachea.  They 
result  from  inflammation,  usually  tuberculous  in  nature,  of  the  neighbor- 
ing lymph-nodes,  which  become  adherent  to  the  oesophageal  wall  and, 
from  subsequent  cicatricial  contraction,  exert  traction  upon  it.  The  sac 
is  comparatively  small  and  funnel-shaped.  The  remains  of  the  diseased 
gland  can  be  detected  at  the  apex.  Such  diverticula  may  be  single  or 
multiple.  Perforation  of  the  sac  may  take  place,  leading  to  suppurative 
perioesophagitis  and  extension  to  the  pleurae,  pericardium,  and  lungs. 
The  wall  of  the  sac  may  consist  of  all  the  elements  of  the  oesophageal 
wall,  or  the  muscularis  may  be  partly  or  completely  defective. 

Rupture. — Rupture  of  the  oesophagus  occurs,  but  is  rare.  It  may 
result  from  trauma  or  increased  internal  pressure.  It  is  said  to  have 

1  Leyden  Festschrift. 


(ESOPHAGITIS  399 

occurred  from  severe  vomiting  after  a  full  meal  and  when  in  a  state  of 
intoxication.  Probably,  in  such  cases  some  pathological  condition  in 
the  oesophagus  has  existed  previously  to  render  this  accident  possible. 
Perforation. — Perforation  of  the  oesophageal  wall  may  result  from 
traumatism,  the  rupture  of  simple,  syphilitic,  and  cancerous  ulcers,  the 
pressure  of  foreign  bodies  within  the  lumen,  from  suppuration,  or  tuber- 
culous caseation  in  the  neighboring  parts,  or,  again,  from  aneurism. 

CIRCULATORY  DISTURBANCES. 

Hyperemia. — Owing  to  the  comparative  scarcity  of  bloodvessels 
in  the  oesophagus,  hyperemia  is  seldom  a  striking  condition. 

Active  Hyperemia. — Active  hyperemia  may  be  due  to  the  irritating 
properties  of  certain  articles  of  food,  alcohol,  and  so  on.  It  occurs  also 
in  newborn  children,  in  the  early  stages  of  inflammation,  and  in  various 
infective  diseases. 

Passive  Hyperemia. — Passive  hyperemia  occurs  in  all  cases  of  general 
congestion,  in  obstructive  disturbances  of  the  circulation  in  the  heart, 
lungs,  and  liver.  In  portal  cirrhosis  of  the  liver,  the  veins  of  the  lower 
portion  of  the  oesophagus  often  become  enormously  distended  (oesopha- 
geal varices),  and  may  form  nodular  or  papillary  masses  projecting  into 
the  lumen.  The  appearance  produced  is  very  similar  to  that  in  rectal 
hemorrhoids.  The  veins  are  dilated  and  tortuous,  sometimes  reaching 
the  size  of  a  lead  pencil.  Rupture  of  these  varices  may  lead  to  serious 
and  even  fatal  hemorrhage. 

Hemorrhages. — (Esophageal  hemorrhages  may  be  caused  also  by 
traumatism,  ulceration,  or  malignant  new-growths.  The  effused  blood 
may  be  vomited  up  or,  again,  digested  and  passed  on  into  the  intestine. 
An  aortic  aneurism  may  erode  into  the  oesophagus  and  rupture  there. 

INFLAMMATIONS. 

(Esophagitis. — Catarrhal  (Esophagitis. — Catarrhal  oesophagitis  is  the 
commonest  form  of  inflammation.  It  is  due  to  the  action  of  thermal  or 
chemical  irritants  in  the  ingesta,  to  extension  of  inflammation,  from  the 
pharynx  or  stomach,  or  arises  as  a  complication  of  certain  of  the  infective 
fevers,  measles,  scarlatina,  typhoid,  and  variola.  It  is  characterized  by 
congestion  of  the  mucosa  and  exfoliation  of  the  superficial  epithelium, 
together  with  increase  in  secretion,  which,  however,  is  usually  scanty  on 
account  of  the  paucity  of  mucous  glands.  Shallow  erosions  commonly 
result,  situated  for  the  most  part  on  the  top  of  the  longitudinal  folds, 
which,  when  healing,  leave  small  scars. 

If  the  cause  persist  or  be  frequently  brought  into  play,  chronic  catarrh 
will  result.  This  condition  is  found  also  in  the  oesophagus  above  a 
stenosis,  and  as  a  result  of  prolonged  passive  congestion.  The  mucosa 
is  of  a  livid  red  color,  the  epithelium  is  thickened,  resulting  in  the  forma- 
tion of  papillomatous  or  polypoid  outgrowths  or  plaque-like  patches  of 


400  THE  (ESOPHAGUS 

leukoplakia.  The  surface  is  covered  with  tenacious  mucus  or  mucopus. 
The  muscular  wall  is  thickened,  both  from  hypertrophy  and  from  pro- 
ductive fibrosis.  The  lumen  is  usually  dilated,  but  may  be  narrowed. 
Superficial  ulceration  is  common. 

Follicular  (Esophagitis. — In  follicular  cesophagitis  the  mucous  glands 
are  involved.  The  lumina  are  obstructed  and  there  is  an  excessive 
production  of  mucus,  which  leads  to  the  dilatation  of  the  glands  and  ducts 
into  small  cysts.  Round  about  the  glands  there  is  a  small-celled  infiltra- 
tion. This  may  result  in  abscess  formation  and,  from  rupture  of  the 
abscess,  ulceration. 

Phlegmonous  (Esophagitis. — Phlegmonous,  or diffuse  suppurative  cesopha- 
gitis,  may  be  primarily  due  to  the  action  of  foreign  bodies  or  corrosive 
substances,  but  most  commonly  arises  by  extension  of  suppurative  inflam- 
mation from  neighboring  structures,  such  as  the  pharynx,  stomach, 
perioasophageal  lymph-nodes,  the  vertebral  column,  and  the  cricoid 
cartilage.  It  occurs  also  in  advanced  pulmonary  tuberculosis  without 
any  obvious  cause,  and  occasionally  supervenes  upon  the  follicular 
form.  The  affection  begins  as  a  purulent  infiltration  of  the  submucosa, 
leading  to  the  formation  of  localized  or  diffuse  collections  of  pus.  The 
mucosa  is  reddened  and  undermined,  and  fistulous  openings  may 
be  formed  in  its  substance,  which  give  vent  to  the  pent-up  exudation. 
The  tissues  about  the  oesophagus  are  sometimes  involved  and  the 
abscesses  may  discharge  into  the  larynx  or  trachea,  or,  more  rarely, 
into  the  mediastinum  or  pleura. 

Pustular  (Esophagitis. — Pustular  cesophagitis  is  the  name  given  to 
an  eruption  of  papules  in  the  mucosa  of  the  oesophagus  occurring  in 
smallpox.  As  in  the  skin,  the  papules  become  pustules,  and  when  they 
rupture  form  small  ulcers. 

Membranous  (Esophagitis. — Membranous  cesophagitis  accompanied  by 
superficial  necrosis  is  not  very  uncommon  in  variola,  measles,  scarlatina, 
typhoid,  typhus,  pyemia,  cholera,  chronic  Bright's  disease,  pneumonia, 
tuberculosis,  and  the  gastro-intestinal  catarrh  of  infants.  The  fibrinous 
deposit  is  rarely  generalized,  but  usually  confined  to  the  tops  of  the  folds. 
Ulceration  may  occur  with  stenosis  of  the  lumen  of  the  oesophagus 
from  cicatricial  contraction.  True  diphtheria  of  the  oesophagus  is  rare. 

Exfoliative  (Esophagitis. — Exfoliative  cesophagitis  (cesophagitis  dissi- 
cans  superficial^)  is  characterized  by  the  desquamation  of  the  lining 
epithelium  in  large  flakes  or  even  as  a  complete  cylinder.  The  etiology 
is  not  clear.  Some  cases  may  be  due  to  the  action  of  corrosives.  The 
disease  usually  occurs  in  neurotic  individuals. 

Corrosive  (Esophagitis. — Corrosive  cesophagitis  is  that  form  due  to 
the  action  of  corrosive  poisons,  chiefly  acids  or  alkalies.  Concentrated 
lye,  carbolic  and  sulphuric  acids  are  the  agents  commonly  at  work. 
The  lesion  produced  is  a  necrotising  inflammation.  The  epithelium 
is  swollen,  shreddy,  and  desquamating  in  patches,  or  in  the  more  severe 
cases  is  converted  into  a  yellowish,  grayish-white,  or  blackish  eschar. 
The  tissues  are  congested,  extravasations  of  blood  are  to  be  found  in 
the  submucosa,  and  a  line  of  demarcation  is  formed  between  the  living 


FOREIGN  BODIES  AND  PARASITES 


401 


FIG.  91 


and  the  dead  tissue.     The  necrotic  portion  is  eventually  cast  off,  if  the 
patient  live.     Suppuration  occurs,  and,  as  healing  takes  place,  serious 
contracture    of    the    lumen   results. 
The  inflammation  may  extend  to  the 
neighboring  tissues. 

Tuberculosis.  —  This  is  always 
secondary  to  advanced  tuberculosis 
in  other  parts.  Most  commonly  it 
is  due  to  extension  of  the  disease 
from  the  peritracheal  lymph-nodes, 
from  the  larynx  or  pharynx.  Less 
often,  it  results  from  swallowing  in- 
fected sputum.  Miliary  tuberculosis 
of  the  oesophagus  also  occurs,  but 
is  rare.  Tuberculous  ulceration  is 
usually  superficial,  but  perforation 
may  take  place.  The  borders  of  the 
ulcers  are  thickened,  and  small  tuber- 
cles can  be  made  out  in  the  sub- 
stance. The  bases  are  smooth  or 
irregular.1 

Syphilis. — Ulceration  of  the  oeso- 
phagus may  occur  both  in  the  sec- 
ondary and  tertiary  stages.  Gumma- 
tous  infiltration  is  the  most  frequent 
lesion.  It  leads  to  ulceration,  per- 
foration, and  cicatricial  contraction 
of  the  lumen. 

Actinomycosis.  —  This  affection 
is  rare.  Primary  actinomycosis  of  the 
oesophagus  has  been  described  in  a 
few  cases.  Extension  to  the  thoracic 
viscera  is  the  rule.2 

Parasites. — Thrush. — This  is  most 
commonly  met  with  in  poorly  nour- 
ished children  and  in  those  suffering 
from  prolonged  disease  and  cachexia. 
As  a  rule,  the  infection  extends  from 
the  mouth  or  pharynx,  but  some- 
times arises  independently.  It  may 

be   associated   with  tuberculosis  of 

the  OeSOphagUS.       Trichinosis  has  also      Pathological  Museum  of  McGill  University.) 

been  observed. 

Foreign  Bodies. — The  chief  foreign  bodies  that  are  at  times  found 
more  or  less  completely  obstructing  the  oesophagus  are:  bones,  fish, 

1  Claribel  Cone,  Johns  Hopkins  Hosp.  Bull,  80: 1897: 229. 

2  A.  Poncet,  Province  M&licale,  Lyon,  9: 1895: 205;  Bull,  de  1'Acad.  de  M£d.3  15: 
1896. 

26 


Stricture   of   the   oesophagus  in   a  child, 


402  THE  (ESOPHAGUS 

needles,  leeches,  and  false  teeth.  Phlegmonous  oesophagitis  commonly 
results.  Hard,  angular,  or  sharp  substances  of  course  do  more  damage 
to  the  wall  than  do  others,  and  may  lead  to  ulceration,  abscess  form- 
ation, or  even  gangrene,  sometimes  followed  by  perforation.  Septic 
matter,  at  times  mixed  with  food,  may  thus  be  discharged  into  the 
mediastinum,  pleura,  pericardium,  or  into  a  bronchus  or  a  large  vessel, 
such  as  the  aorta. 


RETROGRESSIVE   METAMORPHOSES. 

Atrophy. — Atrophy  of  the  wall  may  occur  in  general  marasmus  and, 
locally,  as  a  result  of  pressure.  Various  forms  of  degeneration  are  met 
with  also.  They  are  rare  and  have  been  but  little  studied.  The  most 
important  are  necroses.  Pressure  from  foreign  bodies  impacted  in  the 
lumen,  or  from  aneurisms  or  tumors  from  without,  lead  to  ischemia, 
atrophy,  and  eventually  loss  of  substance.  What  may  be  termed  a 
"bedsore"  of  the  oesophagus  is  occasionally  met  with  in  cachexia  and 
prolonged  fevers  as  a  result  of  pressure  of  the  larynx  upon  the  organ. 

A  round  or  peptic  ulcer,  analogous  in  all  respects  to  the  peptic  ulcer 
of  the  stomach,  has  been  described.  It  is  rare 

(Esophagomalacia. — (Esophagomalacia  has  been  oberved  as  an 
agonal  manifestation  in  cases  of  cerebral  disease.  It  is,  however,  not 
uncommon  as  a  postmortem  phenomenon  as  a  result  of  the  digestion 
of  the  tissues  by  the  gastric  juice  which  has  regurgitated  through  the 
cardia.  The  epithelium  is  macerated,  desquamated,  and  the  muscular 
coat  may  be  discolored,  softened,  or  liquefied.  Perforation  of  the  wall 
sometimes  occurs  with  escape  of  the  stomach  contents  into  the  pleura. 

Gangrene. — Gangrene  of  the  oesophagus  may  be  associated  with  noma 
of  the  cheek  or  pharynx,  gangrenous  tonsillitis,  or  gangrene  of  the  lung. 
It  may  also  result  from  the  action  of  corrosive  substances  and  severe 
inflammation. 

PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Hypertrophy  of  the  muscular  wall  is  produced  by  any 
cause  that  tends  to  obstruct  the  free  passage  of  food  into  the  stomach. 
Muscular  spasm,  carcinoma,  and  strictures  are  the  most  important 
conditions  to  be  mentioned  in  this  connection. 

Leukoplakia. —  Leukoplakia,  similar  to  that  occurring  upon  the 
tongue,  is  common.  Multiple,  small,  rounded,  or  oval,  plaque-like 
elevations  of  a  pearly  white  color  are  observed  upon  the  mucosa.  Micro- 
scopically, they  consist  in  a  simple  local  hyperplasia  of  the  squamous 
lining  epithelium.  They  are  found  in  cases  of  passive  congestion  and 
in  alcoholics. 

Tumors. — New-growths  are,  on  the  whole,  not  common.  They 
originate  in  the  oesophagus  itself  or  in  the  neighboring  parts,  and  are 
rarely,  if  ever,  metastatic.  The  benign  growths  are  usually  small  and 


DERMOID  CYSTS  403 

of  but  little  practical  importance.  The  most  common  are  the  polypoid 
or  papillary  fibromas,  and  the  intramural  fibromas.  Lipomas,  myxomas, 
myomas,  and  polypoid  adenoma  (one  case)  are  recorded. 

Sarcoma. — Primary  sarcoma  of  the  oesophagus  is  quite  rare.  Spindle- 
celled,  round-celled,  and  alveolar  forms  are  described,  and  also  lympho- 
sarcoma.  As  a  rule,  sarcoma  invades  the  oesophagus  by  extension  from 
some  of  the  neighboring  structures. 

Carcinoma. — The  most  important  growth  is  carcinoma,  which  may 
be  primary,  or  secondary  to  carcinoma  of  the  cardia  of  the  stomach, 
pharynx,  or  thyroid  gland. 

Primary  carcinoma  of  the  oesophagus  is  usually  of  the  squamous- 
celled  type,  adenocarcinoma  being  distinctly  rare.  The  latter  form  may 
originate  from  the  mucous  glands  or  from  developmental  cysts  lined 
with  columnar  cells,  which  are  sometimes  present  in  the  wall.  Gland- 
ular carcinoma  may  be  simple,  medullary,  or  scirrhous  in  type.  We 
have  met  with  one  instance  of  a  glandular  cancer,  approximating  the 
scirrhous  form,  which  formed  a  small,  isolated  growth  and  led  to 
complete  obstruction.  The  favorite  sites  for  carcinomatous  growths 
are  at  the  narrowest  parts  of  the  lumen  and  where  there  is  transitional 
epithelium.  They,  therefore,  occur  most  often  at  the  level  of  the  cricoid 
cartilage,  opposite  the  bifurcation  of  the  trachea,  and  at  the  entrance 
into  the  stomach.  The  tumor  tends  to  encircle  the  lumen  and  thus 
produces  stenosis,  a  condition  {hat  is  not  entirely  relieved  even  when 
ulceration  takes  place.  In  some  parts  there  may  be  sufficient  pro- 
liferation of  connective  tissue  to  form  a  scirrhous-like  growth,  but  in 
others  the  mass  is  softer  and  more  fungating.  The  mucous  mem- 
brane in  the  neighborhood  is  usually  inflamed  and  the  cesophageal 
wall  above  the  stricture  is  hypertrophied  and  the  lumen  dilated.  The 
new-growth  may  extend  through  the  wall  of  the  oesophagus  to  the 
trachea,  bronchi,  lung,  mediastinum,  pleura,  pericardium,  or  large 
bloodvessels.  Metastases  occur  in  the  regional  lymph-nodes,  the  liver, 
lungs,  and  bones. 

Carcinoma  occurs  most  often  in  males,  between  the  ages  of  fifty  and 
sixty  years,  particularly  often,  it  is  said,  in  smokers  and  drinkers. 

Dermoid  Cysts. — Dermoid  cysts  are  very  rare.  They  occur  at  a  point 
near  the  junction  of  the  oesophagus  and  pharynx.  The  congenital 
cysts  lined  with  columnar  epithelium  sometimes  found  in  the  cesophageal 
wall  are  of  teratoid  nature.  They  represent  the  remains  of  the  original 
communication  between  the  oesophagus  and  trachea. 


CHAPTER    XIX 

THE  STOMACH 
ANOMALIES. 

Congenital  Anomalies. — Complete  absence  of  the  stomach  has  been 
reported  in  connection  with  other  grave  anomalies,  such  as  acephalus. 
It  is  rare.  The  organ  may  be  abnormally  small,  even  in  otherwise 
well-developed  individuals.  The  pyloric  opening  may  be  completely 
or,  more  often,  partially  occluded,  and  attached  to  the  duodenum  by 

FIG.  92 


Hour-glass  stomach.     (From  the  Pathological  Museum  of  McGill  University.) 

a  fibrous  cord.  Diverticula  are  rare.  The  stomach  may  be  divided  into 
several  chambers  by  intersecting  septa.  It  may  also  be  composed  of  two 
chambers,  owing  to  a  constriction  about  the  middle  (hour-glass  deformity). 
In  complete  transposition  of  the  viscera  the  stomach  may  be  reversed, 
the  pylorus  being  situated  to  the  left.  Occasionally,  the  stomach  assumes 
a  vertical  position,  possibly  a  persistence  of  the  embryonic  condition. 

Another  type  of  congenital  displacement  is  that  found  in  connection 
with  defect  of  the  diaphragm.  There  may  be  absolute  defect,  with  free 
communication  between  the  thoracic  and  abdominal  cavities  (congenital 
false  hernia);  the  diaphragm  may  be  congenitally  weak  at  some  point, 
so  that  violence  or  muscular  strain  subsequently  applied  may  cause  it 
to  give  way,  with  protrusion  of  the  abdominal  viscera  into  the  thoracic 


ANOMALIES 


405 


cavity  (acquired  false  hernia);  or  there  may  be  thinning  of  the  diaphragm 
so  that  the  abdominal  organs  are  dislocated  into  the  thoracic  cavity, 


FIG.  93 


Congenital  diaphragmatic  hernia  in  man,  aged  fifty-seven  years;  partly  false,  there  being  at 
A  and  B  direct  communications  between  the  peritoneal  and  pleural  cavities,  from  the  edges  of 
which  fatty  omental  folds  of  tissue  passed  into  the  thorax;  partly  true,  the  serous  membrane 
forming  a  covering  over  fthe  stomach  and  left  kidney  as  they  lay  in  the  pleural  cavity.  The 
muscle  of  the  diaphragm  is  shaded  dark. 


FIG.  94 


Hypertrophic  stenosis  of  the  pylorus.     (From  the  Pathological  Museum  of  McGill  University.) 


406  THE  STOMACH 

but  are  contained  in  a  sac  of  diaphragm  or  peritoneum  (true  diaphrag- 
matic hernia).  The  second  variety  mentioned  is  the  most  common,  224 
of  Leichtenstern's  252  cases  being  of  this  type.  True  diaphragmatic 
hernia  is  a  rare  form,  occurring  in  only  11  per  cent,  of  all  cases.1 

Congenital  Hypertrophic  Stenosis  of  the  Pylorus. — Congenital  hyper- 
trophic  stenosis  of  the  pylorus  is  somewhat  rare,  although  undoubt- 
edly more  frequent  than  has  been  thought.  There  may  be  a  pre- 
existing congenital  stenosis  of  the  orifice  with  subsequent  hypertrophy 
of  the  muscles.  The  microscopic  examination  of  the  pylorus  shows 
merely  hyperplasia  of  the  muscle  with  some  fibrosis.  There  may  also 
be  slight  catarrh  of  the  mucosa.  The  affection  is  important,  as  it 
occurs  in  infants  usually  under  four  months  old,  and  may  lead  to  death 
from  starvation  if  unrelieved.2 


ACQUIRED  DISPLACEMENTS. 

Displacement  of  the  stomach  may  be  upward  (vide  supra),  down- 
ward (gastroptosis),  or  to  the  side.  In  all  cases  the  cardia  is  a  fixed 
point,  the  pylorus  or  some  other  portion  moving  upon  this  as  on  a  hinge. 
A  common  form  is  that  in  which  the  middle  portion  of  the  organ 
sags  downward,  the  pylorus  and  the  cardia  remaining  in  their  normal 
position. 

Dislocation  is  sometimes  due  to  causes  inherent  in  the  stomach  itself, 
such  as  dilatation  or  contraction,  but  is  often,  also,  the  result  of  extrinsic 
factors.  A  loaded  colon,  the  weight  of  tumors,  the  traction  of  adhesions, 
will,  on  occasion,  tend  to  drag  the  organ  down.  The  pressure  of  corsets 
or  an  enlarged  liver  may  force  it  downward. 

Gastroptosis  is  nearly  always  part  and  parcel  of  a  general  prolapse  of 
the  abdominal  viscera  (splanchnoptosis).  The  term  Glenard's  disease 
originally  referred  to  prolapse  of  the  intestines  alone  (enteroptosis), 
but  has  been  used  more  comprehensively  to  include  cases  of  enteroptosis 
combined  with  prolapse  of  other  organs  as  well.  Besides  the  stomach  and 
intestines,  the  liver  and  the  right  kidney  may  be  displaced  (hepatoptosis, 
nephroptosis). 

In  Glenard's  disease  there  is  a  peculiar  symptom  complex,  nervous 
dyspepsia,  and  atony  of  the  stomach,  constipation,  or  constipation 
alternating  with  diarrhea,  dragging  sensations  in  the  back,  anemia, 
and  in  the  later  stages,  neurasthenia.  Anatomically,  there  is  relaxation 
of  the  hepatic  and  hepatopyloric  ligaments.  Glenard,  who  originally 
described  the  condition,  thought  he  had  discovered  the  anatomical  basis 
of  neurasthenia,  but  this  view  is  not  generally  accepted.  Bouveret  and 
Charcot  considered  that  the  prolapse  was  rather  the  result  than  the  cause 

1  F.  M.  Fry,  Diaphragmatic  Hernia,  Mont.  Med.  Jour.,  24: 1895-6: 101. 

2Rolleston  and  Crofton-Atkins,  Congenital  Hypertrophy  with  Stenosis,  Brit. 
Med.  Jour.,  2:1900:1768.  See,  also,  Scudder,  Boston  Med.  and  Surg.  Journ., 
1905:494. 


DILATATION  407 

of  the  neurasthenia,  due  to  the  loss  of  muscular  and  nervous  tone.  The 
causes  of  splanchnoptosis  are,  pressure  upon  the  abdominal  contents 
from  above,  as  from  corsets;  the  cramping  effect  of  a  long,  narrow,  or 
phthisical  chest;  an  enlarged  liver;  relaxation  of  the  supporting  ligaments; 
weakening  of  the  abdominal  walls  from  pregnancy;  the  pressure  of 
tumors  or  cysts;  nervous  depression;  and  conditions  which  tend  to  drag 
the  viscera  down. 


ALTERATIONS  IN  THE  SIZE  AND  SHAPE  OF  THE  LUMEN. 

Contraction  of  the  stomach  is  found  in  certain  forms  of  chronic  gastritis 
and  in  diffuse  scirrhus  carcinoma.  The  cavity  of  the  stomach  in  the 
latter  case  may  not  be  large  enough  to  contain  more  than  a  cupful  of 
fluid. 

Hour-glass. — Hour-glass  contraction,  when  acquired,  may  be  due  to 
the  cicatrization  of  an  ulcer,  torsion  of  the  stomach,  tumors,  hernia, 
or  adhesion  to  displaced  viscera. 

Dilatation. — Dilatation  of  the  stomach  is  a  not  uncommon  condition. 
The  two  main  factors  in  its  causation  are  a  deficiency  of  muscular  tone 
and  difficulty  in  evacuation  of  the  organ.  The  affection  often  comes 
on  slowly  and  insidiously,  and  it  may  be  long  before  clinical  symptoms 
manifest  themselves.  Overeating  and  drinking  are  potent  causes  and 
act  by  bringing  about  a  gradual  distension  of  the  organ,  with  sometimes 
descent.  In  not  a  few  cases  the  stomach  is  considerably  enlarged, 
but  is  able  to  digest  and  discharge  its  contents  in  the  regular  space  of 
time.  Such  a  condition  is  termed  megalogastria,  and  probably  should 
be  differentiated  from  true  dilatation,  in  which  the  muscle  is  overstretched 
and  functionally  weak.  Among  the  causes  that  act  by  obstructing 
the  onflow  of  the  gastric  contents  may  be  mentioned  carcinoma  of  the 
pylorus,  stenosis  and  hypertrophic  stenosis  of  the  pylorus,  pyloric  spasm, 
external  pressure  of  new-growths  or  enlarged  glands,  the  traction  of 
inflammatory  adhesions,  gastroptosis,  and  tight  lacing. 

A  considerable  number  of  cases  of  acute  dilatation  of  the  stomach  are 
now  on  record,  and  the  condition  is  probably  much  more  common  than 
has  generally  been  realized.  Acute  dilatation  comes  on  with  great  sud- 
denness, and  unless  promptly  relieved  by  appropriate  measures  usually 
runs  a  rapidly  fatal  course.  The  chief  etiological  factors  of  a  predispos- 
ing nature  are:  (1)  operations  under  general  anesthesia;  (2)  severe  and 
prolonged  disease;  (3)  indiscretions  in  diet;  (4)  disease  or  deformity  of 
the  spine;  and  (5)  traumatism.  A  few  cases  have  come  on  without 
obvious  cause.  Now  and  then  one  will  supervene  upon  chronic  dilata- 
tion. The  direct  causes  appear  to  be  primary  paresis  of  the  gastric 
musculature,  and  obstruction  to  the  onflow  of  the  gastric  contents.  In 
about  half  the  recorded  cases  definite  mechanical  obstruction  has  been 
found,  occasionally  at  the  pylorus,  but  much  more  frequently  in  the 
duodenum,  just  below  the  bile  papilla.  Kinks,  the  traction  of  adhesions, 
and  the  pressure  of  misplaced  organs  may  bring  this  about.  The  most 


408  THE  STOMACH 

constant  anatomical  lesion,  however,  is  compression  of  the  duodenum 
by  the  root  of  the  mesentery.1 

Volvulus  of  the  stomach  has  been  observed.2 


CIRCULATORY  DISTURBANCES. 

The  amount  of  blood  in  the  stomach  varies  widely  within  physiological 
limits,  depending  upon  the  degree  of  functional  activity  for  the  time 
being.  Consequently,  postmortem  appearances  must  be  interpreted 
with  some  degree  of  caution. 

(Edema. — (Edema  is  rare,  even  in  inflammatory  processes. 

Anemia. — Anemia  occurs  as  a  manifestation  of  general  systemic 
anemia,  and  in  association  with  atrophy  of  the  mucosa.  In  the  latter 
case  it  is  difficult  to  determine  whether  the  atrophy  is  the  result  of  the 
anemia  or  vice  versa. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  occurs  physio- 
logically during  digestion  and  imparts  a  delicate  rose-pink  color  to  the 
mucosa.  It  also  results  from  the  action  of  irritating  or  corrosive  sub- 
stances. In  such  cases  the  congestion  is  very  intense  and  is  present  in 
irregular  patches,  localized  especially  to  the  tops  of  the  rugse. 

Passive  Hyperemia. — Passive  hyperemia  is  found  in  connection  with 
portal  obstruction,  particularly  that  occurring  in  cirrhosis  of  the  liver, 
and,  more  remotely,  with  vascular  obstruction  in  the  heart  and  lungs. 
The  pyloric  portion  is  the  part  chiefly  affected.  The  mucosa  is  of  a  dull, 
purple  red  color,  and  the  veins  are  often  distended.  Extravasations  of 
blood  occur,  so  that  patches  and  spots  of  a  yellowish  or  brownish  color 
are  not  infrequent.  In  long-standing  cases  the  mucous  membrane 
becomes  markedly  pigmented. 

Hemorrhages. — Hemorrhages  into  the  stomach  wall  are  quite 
common.  According  to  Birch-Hirschfeld,  they  are  found  in  about  50 
per  cent,  of  cadavers.  He  regards  them  as  due  to  severe  and  pro- 
tracted vomiting  just  before  death.  In  general,  the  extravasations  are 
due  to  increased  vascular  tension  or  disease  of  the  vessel  walls.  They  are 
found  in  cirrhosis  of  the  liver,  leukemia,  pernicious  anemia,  the  hemor- 
rhagic  diatheses,  scurvy,  acute  yellow  atrophy  of  the  liver,  and  in  some 
acute  infections;  also,  in  poisoning  from  phosphorus,  strychnine,  and 
morphine;  and  in  severe  vomiting. 

Hemorrhage  into  the  cavity  of  the  stomach  may  be  due  to  the 
erosion  of  a  vessel  from  a  simple  or  cancerous  ulcer,  traumatism,  or. 
rarely,  leukemia,  hemophilia,  or  vicarious  menstruation.  When  the 
bleeding  is  at  all  extensive  the  blood  is  usually  vomited  (hematemesis), 
or,  again,  may  be  passed  by  the  bowel  (melena).  When  recently 
effused  the  blood  is  acid,  bright  red,  pure  or  mixed  with  food;  when 

1  Nicholls,   Acute  Dilatation  of  the  Stomach,   Internat.  Clinics,   Lippincott,  4 : 
1908:80. 
2Pendl,  Wien.  klin.  Woch.,  1904:476. 


GASTRITIS  409 

the  hemorrhage  is  of  some  standing,  however,  the  blood  assumes  a 
turbid  brown  appearance  like  coffee-grounds,  owing  to  the  action  of 
the  gastric  juice.  It  is  worth  while  pointing  out  that  all  blood  vomited 
does  not  necessarily  result  from  gastric  hemorrhage.  It  may  be  due, 
for  example,  to  bleeding  from  the  nose,  mouth,  or  resophagus,  the  blood 
being  subsequently  swallowed,  or  to  the  oozing  from  an  aneurism.  In 
young  infants  pus  and  blood  from  a  suppurating  breast  may  be  swallowed 
and  later  on  regurgitated,  an  occurrence  that  sometimes  gives  rise  to  an 
erroneous  diagnosis. 

Hemorrhages  into  the  mucosa  lead  to  weakening  of  the  local  resisting 
power  of  the  stomach,  and  areas  so  affected  are  not  infrequently  converted 
into  shallow  ulcers,  owing  to  the  eroding  action  of  the  gastric  secretion 
(hemorrhagic  erosion). 

Thrombosis. — Thrombosis  of  certain  of  the  gastric  vessels  is  met 
with  occasionally.  It  is  thought  by  some  to  be  the  primary  condition 
leading  to  the  formation  of  the  peptic  ulcer.  It  occurs  occasionally 
in  connection  with  severe  burns  of  the  skin. 

Embolism. — Embolism  is  said  to  be  not  uncommon. 


INFLAMMATIONS. 

Gastritis. — Inflammation  of  the  stomach  in  the  vast  majority  of 
instances  is  due  to  the  irritating  action  of  substances  that  have  been 
ingested;  some  few  cases  are  attributable  to  the  agency  of  the  toxic 
substances  circulating  in  the  blood;  a  few,  also,  to  the  extension  of 
inflammatory  processes  from  neighboring  parts. 

Ingested  substances  act  as  irritants  largely  owing  to  their  physical  or 
chemical  properties.  Foodstuffs  may  on  occasion  act  as  the  excitants  of 
gastritis  (dyspeptic  gastritis).  An  excessive  amount  of  food  is  a  common 
cause,  owing  to  the  inability  of  the  stomach  to  digest  and  pass  it  along 
quickly  enough.  This  is  particularly  apt  to  occur  when  the  function 
of  the  stomach  is  impaired,  as  from  muscular  atony,  pyloric  stenosis, 
carcinoma,  or  fibrous  induration.  Should  food  from  any  of  these  causes 
be  retained,  it  undergoes  abnormal  fermentation.  Putrid  gases,  irri- 
tating fatty  acids,  and  bacterial  toxins  are  produced  in  notable  quan- 
tities and  lead  to  severe  disorders.  Pus,  infected  sputum,  and  foul 
material  from  the  buccal  cavity  may  also  induce  gastritis. 

The  most  severe  cases  of  gastritis  are  brought  about  by  corrosive 
poisons,  the  conditions  resulting  varying  according  to  the  character 
of  the  destructive  substance  and  the  length  of  time  that  has  elapsed 
since  its  ingestion.  Strong  corrosives  lead  to  extensive  necrosis  of  the 
mucous  membrane.  The  slough  produced  by  sulphuric  acid  is  hard,  dry, 
brittle,  and  of  a  grayish  color.  Nitric  and  hydrochloric  acids  give  a 
yellowish  tint.  Caustic  alkalies  produce  a  transparent,  pulpy,  digested 
appearance.  The  morbid  appearances  resulting  depend  considerably 
upon  the  amount  of  food  in  the  stomach  at  the  time,  being,  of  course, 
more  intensely  marked  in  the  fasting  organ.  The  effects  of  caustics 


410  THE  STOMACH 

are  usually  more  pronounced  at  the  fundus  and  posterior  wall,  and 
especially  on  the  tops  of  the  rugse.  The  remainder  of  the  mucosa  may 
be  free  from  necrosis,  but  may  show  signs  of  a  more  or  less  intense  inflam- 
mation, often  hemorrhagic  in  character. 

Hematogenous  Gastritis. — Hematogenous  gastritis  arises  more  especially 
in  the  course  of  infections  and  intoxications,  as  in  septicemia,  typhoid 
fever,  pulmonary  tuberculosis,  and  variola.  Metastatic  abscess  forma- 
tion is,  however,  rare. 

Gastritis  per  Extensionem. — Gastritis  per  extensionem  may  result  from 
any  localized  inflammatory  condition  in  the  neighborhood  of  the  stomach. 
Simple  adhesion  to  adjacent  structures  may  occur  or  perforation  of  the 
stomach  wall,  with  discharge  of  inflammatory  products  into  the  cavity. 
Fistulous  communications  with  other  viscera  may  thus  arise.  Chole- 
lithiasis, empyema  of  the  gall-bladder,  suppurative  pancreatitis,  may  be 
mentioned  in  this  connection.  A  perinephritic  abscess  has  been  known 
to  rupture  into  the  stomach.  One  of  us  (A.  G.  N.1)  has  recorded  an 
apparently  unique  case  of  this  kind  in  which  the  patient  vomited  pus 
and  blood  for  three  days  before  death. 

Acute  Catarrhal  Gastritis. — Acute  catarrhal  gastritis  is  characterized 
by  swelling  and  hyperemia  of  the  mucous  membrane,  together  with  the 
production  of  a  viscid  adhesive  exudate,  consisting  of  mucus,  desqua- 
mated epithelium,  and  leukocytes.  Here  and  there,  small  hemorrhages 
may  be  seen  and  also  superficial  erosions. 

Histologically,  the  secretory  cells  of  the  glands  are  cloudy  and  are 
desquamating,  while  goblet  cells  are  very  numerous.  The  epithelium 
of  the  peptic  glands  is  more  granular  than  usual  and  is  often  detached 
from  the  basement  membrane.  On  the  surface  can  be  seen  a  stringy 
deposit  of  mucus,  entangled  in  which  are  clumps  of  gastric  epithelium, 
red  blood-cells,  and  leukocytes.  There  is  a  round-celled  infiltration 
between  the  tubules  and  in  the  submucosa,  and  the  interstitial  bloodves- 
sels are  congested.  The  endothelium  of  the  lymphatics  also  may  show 
signs  of  proliferation.  These  changes  are  usually  confined  to  the  region 
of  the  pylorus,  but  may  be  generalized. 

Membranous  Gastritis. — A  more  intense,  though  somewhat  rare,  con- 
dition is  membranous  gastritis.  In  its  typical  form  it  occurs  most  fre- 
quently in  children.  The  membrane  lies  in  small  patches  on  the  tops 
of  the  rugae,  or,  more  rarely,  covers  the  whole  stomach,  forming,  as  it 
were,  a  cast  of  the  interior.  The  membrane  is  loosely  attached  to  the 
mucosa  and  is  of  a  grayish  color,  or,  again,  is  brownish  from  hemor- 
rhage. 

Membranous  gastritis  occurs  most  commonly  in  newborn  children, 
in  cases  of  septic  infection  of  the  umbilical  cord,  and  in  those  suffering 
from  scarlatina,  measles,  diphtheria,  and  variola,  rarely  in  other  infective 
fevers.  True  diphtheria  of  the  stomach  occurs,  and,  curiously  enough, 
the  infection  may  pass  from  the  throat  to  the  stomach  without  attacking 
the  oesophagus.  Necrosis  and  suppuration  may  cause  a  more  or  less 

1  Nicholls,  Mont.  Med.  Jour.,  27: 1898 : 119. 


CHRONIC  CATARRHAL  GASTRITIS 


411 


extensive  loss  of  substance  in  the  mucosa,  and  deeply  eroding  ulcers 
may  be  formed. 

Phlegmonous  Gastritis. — Phlegmonous  gastritis  is  rare.  It  occurs 
idiopathically,  particularly  in  drunkards,  and  occasionally  in  general 
pyemia.  More  or  less  localized  pus  collections  are  formed  in  the  stomach 
wall  which  may  reach  a  considerable  size  and  rupture  into  the  cavity  of 
the  organ. 

Follicular  Gastritis. — Follicular  gastritis  is  also  rare.  It  is  due  to  inflam- 
mation of  the  lymph-follicles,  which  are  present  in  small  numbers  in  the 
stomach.  Suppuration  may  occur,  giving  rise  to  small,  rounded  ulcers. 

Chronic  Catarrhal  Gastritis. — Chronic  catarrhal  gastritis  may  result 
from  repeated  acute  attacks,  but  not  infrequently  arises  independently. 
It  is  most  common  in  those  addicted  to  excess  in  alcohol  or  tobacco,  in 
cases  of  long-standing  passive  congestion  of  the  stomach,  and  as  an 
accompaniment  of  gastric  ulcer,  carcinoma,  and  dilatation.  It  may 
also  complicate  certain  constitutional  affections,  such  as  anemia,  Bright's 
disease,  tuberculosis,  and  gout. 

FIG.  95 


Multiple  adenomatous  polyps  (adenitis  polyposa)  of  stomach:   D,  duodenum;  P,  pyloric  ring. 

(Orth.) 

Several  different  forms  have  been  described,  but  they  are  all  probably 
to  be  regarded  as  stages  in  one  and  the  same  process.  The  mucous 
membrane  is  of  a  brown  or  grayish-brown  color,  owing  to  the  deposit  of 
an  iron-containing  pigment  derived  from  the  blood  in  the  secreting  cells 
and  interglandular  tissues.  Congestion  is  not  necessarily  present,  and 
may,  in  the  later  stages  at  least,  be  completely  lacking,  except  in  those 
cases  originally  due  to  passive  hyperemia  of  systemic  origin.  The 
mucosa  is  covered  with  a  thick,  adherent  layer  of  mucus,  mixed  with 
leukocytes  and  altered  desquamated  epithelial  cells.  In  not  a  few  cases 
numerous  flattened,  elevated  plaques  are  produced  (etat  mamelonne),  and 
the  proliferation  of  the  interglandular  fibrous  tissue  may  be  so  great  that 
warty  outgrowths  result  (gastritis  polyposa).  In  still  another  class  of 
cases  the  productive  change  is  most  marked  in  the  glandular  elements, 
so  that  outgrowths  resembling  adenomas  are  produced.  The  last  two 
forms  are  often  grouped  together  under  the  term  hypertrophic  gastritis. 


412  THE  STOMACH 

Again,  the  overgrowth  of  the  interstitial  connective  tissue  may  lead 
to  sclerosis  and  contraction  of  the  organ  (atrophic  gastritis).  Here, 
the  mucosa  is  thin,  hard,  and  of  a  grayish  color.  The  other  coats  may 
also  show  fibrous  hyperplasia,  so  that  the  thickness  of  the  stomach  wall 
is  greatly  increased  while,  at  the  same  time,  its  capacity  is  diminished. 

Microscopically,  the  most  striking  feature  is  a  cellular  infiltration  in 
the  interstitial  areolar  tissue,  more  especially  in  the  outer  layers  of  the 
mucosa.  This  leads  to  more  or  less  separation  of  the  gland-tubules. 
The  ducts  are  catarrhal,  often  tortuous,  dilated,  or  even  cystic,  the 
secreting  cells  being  increased  both  in  size  and  number.  These  changes 
lead  to  thickening  of  the  mucous  membrane  giving  it  an  irregular 
appearance. 

Specific  Forms  of  Gastritis. — Thrush  is  occasionally  present  in  small, 
isolated,  whitish  patches  on  the  mucosa,  in  cases  where  the  fauces  are 
extensively  involved. 

Tuberculosis. — Tuberculous  infection  of  the  stomach  is  rare,  prob- 
ably owing  to  the  fact  that  the  acid  character  of  the  gastric  secretion  is 
inimical  to  the  growth  of  the  tubercle  bacillus,  as  of  other  germs.  Tuber- 
culosis of  the  stomach  in  human  beings  is  almost  invariably  associated 
with  advanced  tuberculosis  elsewhere,  usually  in  the  lungs.  Van  Wart1 
has  recorded  one  case,  however,  which  is  an  exception  to  the  rule. 
Orth,  by  feeding  rabbits  on  tuberculous  material,  was  able  to  produce 
typical  lesions  in  the  intestines  in  seven  cases  and  in  the  stomach  in  one. 
Alice  Hamilton2  has  recorded  three  cases  of  tuberculous  ulceration  of 
the  stomach  and  has  collected  16  more  from  the  literature.  Tuberculous 
ulcers  are  generally  multiple  and  situated  near  the  pylorus.  Some  are 
little  more  than  superficial  erosions  of  the  mucosa,  the  discovery  of  the 
specific  bacillus  alone  deciding  the  nature  of  the  case,  while  others  have 
a  more  typical  appearance,  with  infiltrated  edges  and  an  irregular  base 
containing  caseous  tubercles.  It  is  remarkable  that  in  many  cases  the 
intestines  escape  the  infection. 

Syphilis. — This  disease  is  also  rare.  Ecchymoses,  hemorrhagic 
erosions,  and  chronic  gastritis  have  been  described  among  the  lesions 
occurring.  The  most  characteristic  manifestation  is  the  gumma,  which 
may  give  rise  to  ulcers  that  may  perforate.  Cicatricial  contractures  of 
the  affected  part  may  also  result. 

Typhoid. — Typhoid  ulcers  are  reported,  but  are  still  rarer  than 
tuberculosis. 

Actinomycosis. — Actinomycosis  is  also  excessively  rare. 

Inflammation  of  the  stomach,  more  particularly  the  ulcerative  and 
phlegmonous  forms,  may  extend  through  all  the  coats  and  involve 
adjacent  structures  (perigastritis).  If  the  infection  be  a  mild  one, 
simple  fibrinous  adhesion  takes  place  between  the  contiguous  surfaces, 
as,  for  example,  those  of  the  stomach  and  liver  or  anterior  abdominal 
wall,  with  subsequent  organization.  Or,  if  the  condition  be  more 
severe  and  of  a  septic  nature,  local  abscesses  may  be  formed  about  the 

1  Johns  Hopkins  Hosp.  Bull.,  14 : 1903 :  235.  2  Ibid.,  8 :  1897 :  75. 


PARASITES  AND  ABNORMAL  CONTENTS  413 

stomach.  Perforation  of  the  stomach  wall  and  adhesion  to  a  hollow 
viscus,  such  as  the  colon,  may  lead  to  the  formation  of  a  fistulous 
track.  Communication  may  thus  be  opened  up  between  the  stomach 
and  the  pleura. 

Parasites  and  Abnormal  Contents.— Any  of  the  ordinary  intestinal 
parasites  may  find  a  lodgement  in  the  stomach.  The  Pentastomum 
denticulatum  and  the  Echinococcus  are  met  with  in  the  stomach  wall, 
but  are  rare. 

It  is  not  uncommon  to  find  foreign  bodies  in  the  stomach  which  have 
got  in  with  the  food  or  have  been  swallowed  by  accident  or  design. 
Buttons,  needles,  spoons,  scissors,  hairpins,  nails,  false  teeth,  knives, 
forks,  coins,  stomach  tubes,  and  many  other  articles  are  at  times  swal- 

FIG.  96 


Hair  ball  in  the  stomach.      The  hair  forms  a  complete  cast  of  the  stomach  and  duodenum. 
(Case  of  Dr.  James  Bell,  Royal  Victoria  Hospital,  Montreal.) 

lowed  by  insane  and  hysterical  patients  or  by  circus  performers,  and  may 
give  rise  to  serious  trouble.  Hair-balls  (phytobezoar)  are  frequently 
found  in  cattle  and,  rarely,  in  human  beings,  Schopf1  has  collected 
16  cases,  and  1  has  been  reported  in  Montreal  by  James  Bell.2  The 
hair-ball  is  composed  of  the  patient's  own  hair,  or,  as  in  one  case  reported, 
a  mixture  of  that  of  the  patient  and  that  of  a  pet  dog,  felted  into  a  com- 
pact mass  by  the  muscular  action  of  the  stomach.  The  individual 
hairs  composing  it  may  be  quite  long  and  vary  somewhat  in  color.  As 
a  rule,  they  are  somewhat  darker  than  the  patient's  hair,  probably  owing 

1  Wien.  klin.  Woch.,  November  16,  1899. 

2  Montreal  Med.  Jour.,  32 : 1903 :  94. 


414  THE  STOMACH 

to  chemical  action  in  the  stomach  cavity.  The  hair  may,  in  time,  accu- 
mulate to  such  an  extent  as  to  form  a  complete  cast  of  the  interior  of 
the  stomach  and  duodenum.  Inflammation  of  these  viscera  not  in- 
frequently results,  which  may  lead  to  perforation. 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Simple  atrophy  is  met  with  in  cachexia  from  any  cause, 
marasmus,  pernicious  anemia,  and  as  a  senile  change.  The  stomach 
is  small,  the  wall  thin,  and  the  mucous  membrane  thin,  pale,  smooth, 
and  shiny.  The  glands  are  granular  and  diminished  in  size.  Diffuse 
or  patchy  fatty  degeneration  is  a  not  uncommon  associated  condition. 

Degeneration. — Fatty  Degeneration. — Fatty  degeneration  occurs  in 
typhoid  fever,  septicemia,  variola,  pernicious  anemia,  leukemia,  and  in 
poisoning  with  phosphorus,  arsenic,  and  lead. 

Hyaline,  Colloid,  and  Amyloid. — Hyaline,  colloid,  and  amyloid  trans- 
formation are  not  uncommon. 

Calcification. — Calcification  of  the  mucosa  has  been  observed  in 
cases  of  rapid  absorption  of  the  lime  salts  from  the  bones  in  corrosive 
sublimate  poisoning. 

Postmortem  Gastromalacia. — A  brief  reference  should  be  made 
to  the  condition  known  as  postmortem  gastromalacia.  The  condition 
is  not  common  in  our  experience  in  bodies  sectioned  within  twenty- 
four  hours  of  death.  In  other  cases,  local  areas  of  softening,  usually 
situated  on  the  posterior  wall  of  the  fundus  or  at  the  cardia,  may  be  found, 
which,  from  their  appearance  and  special  characters  are  to  be  attributed 
to  the  action  of  the  gastric  secretion,  assisted  probably  by  processes  of 
decomposition.  These  areas  are  gelatinous,  of  a  dirty,  reddish-brown 
or  green  color,  and  often  give  rise  to  perforation  and  escape  of  the  stomach 
contents.  Much  difference  of  opinion  has  been  expressed  over  the 
question  whether  or  not  this  softening  is  exclusively  a  postmortem 
appearance.  The  determining  factors  appear  to  be  the  condition  of  the 
secretory  function  of  the  stomach  at  the  time  of  death  and  the  length  of 
time  that  has  elapsed  since  death.  It  is  quite  possible,  however,  that 
in  certain  diseases  where  the  resisting  powers  of  the  mucosa  is  diminished 
the  phenomenon  may  be  an  agonal  one. 

Peptic  Ulcer. — Of  more  practical  importance  is  the  so-called  peptic 
ulcer,  found  both  in  the  stomach  and  duodenum.  When  in  the  duo- 
denum it  is  never  situated  below  the  bile  papilla,  being  confined  to  that 
portion  acted  on  by  the  gastric  juice. 

The  typical  peptic  ulcer  is  round  or  oval,  extending  more  or  less 
deeply  into  the  wall  of  the  viscus.  It  has  a  characteristic  funnel 
shape,  the  edges  being  terraced,  more  or  less  sharply  cut,  and  gradually 
narrowing  as  the  base  is  approached.  In  chronic  cases,  however, 
the  edges  may  be  rounded  and  the  whole  wall  thickened.  The  ulcer 
is  occasionally  deeply  pigmented,  owing  to  the  action  of  the  gastric 
secretion  upon  the  blood.  Sometimes  a  number  of  superficial  erosions 


PEPTIC  ULCER  415 

tending  to  coalesce  are  found,  a  circumstance  that  throws  some  light 
on  the  etiology  of  the  condition.  Microscopically,  a  recent  ulcer  shows 
but  little.  Except  for  the  loss  of  substance  and  the  terraced  condition 
of  the  edges  there  may  be  no  further  change,  or  at  most  only  a  fine  granu- 
lation of  the  cells.  Only  exceptionally  is  there  a  marked  inflammatory 
reaction.  Even  in  ulcers  of  long-standing  the  reactive  phenomena  are 

FIG.  97 


Peptic  ulcer.     (From  the  Pathological  Museum  of  McGill  University.) 

by  no  means  pronounced.  There  is  merely  slight  diapedesis  of  leuko- 
cytes, the  muscularis  shows  fatty  changes,  and  the  vessels  exhibit  pro- 
liferating endarteritis. 

Peptic  ulcers  are  single  or  multiple,  and  when  in  the  stomach  are 
usually  situated  at  the  pylorus,  preferably  on  the  lesser  curvature. 
They  may  be  mere  superficial  erosions,  round  or  oval  excavated  ulcers, 
or  may  be  so  large  as  to  girdle  the  stomach.  The  causes  are  many, 
but  seem  to  depend  mainly  on  defective  circulation  in  the  part,  the  result 


416  THE  STOMACH 

of  thrombosis,  embolism,  or  disease  of  the  vessel  walls.  Small  areas  of 
hemorrhage  or  anemia  predispose  to  ulcer  formation.  The  not  infre- 
quent association  of  gastric  and  duodenal  ulcers  with  severe  burns  of 
the  skin  is  well  recognized.  Here,  the  condition  appears  to  be  analogous 
to  the  necrotic  changes  that  occur  in  the  lymph-nodes  from  the  action  of 
toxic  substances  derived  from  the  injured  region.  Once  the  protecting 
mucous  membrane  is  damaged  or  destroyed,  the  digestive  action  of  the 
gastric  secretion  comes  into  play  and  leads  to  further  loss  of  substance. 

Peptic  ulcers  are  essentially  chronic  and  give  rise  to  a  variety  of  symp- 
toms. Repeated,  small  hemorrhages  will  produce  systemic  anemia; 
larger  ones  may  cause  death.  Pain  after  food  and  dyspeptic  symptoms 
are  quite  common,  but  the  condition  not  infrequently  is  entirely  latent 
and  unsuspected.  A  serious  event  is  perforation  of  the  ulcer  resulting 
in  general  peritonitis.  An  ulcer  on  the  posterior  surface  may  perforate 
into  the  lesser  peritoneal  sac,  a  relatively  favorable  event.  This  may  lead 
to  fibrous  perigastritis.  Rarer  sequels  are  perforation  into  the  colon, 
spleen,  gall-bladder,  through  the  abdominal  wall,  into  the  left  pleural 
cavity,  into  the  left  lung,  and  into  the  pericardium.  In  chronic  ulcera- 
tion,  fibrous  adhesion  between  the  affected  part  and  neighboring  organs, 
such  as  the  pancreas,  liver,  and  adjacent  lymph-nodes,  is  a  common 
event.  Finally,  ulcers  may  heal,  with  the  production  of  minute  fibrous 
scars  or  larger  stellate,  contracted  cicatrices.  Stenosis  of  the  pylorus 
and  hour-glass  constriction  of  the  stomach  are  some  of  the  results  of 
this  occurrence.  Carcinoma  may  develop  at  the  site  of  an  old  ulcer. 

Simple  Erosion. — There  is  one  form  of  ulceration  of  the  stomach, 
found  not  infrequently  at  autopsies,  about  which  a  word  or  two  should 
be  said,  especially  as  the  condition  is  one  that  has  not  as  yet  attracted 
much  attention.  The  ulcers  are  very  numerous  and  take  the  form  of 
small,  irregular,  shallow  pits,  giving  the  mucosa  a  somewhat  "moth- 
eaten"  appearance.  There  is  no  infiltration  and  no  surrounding  con- 
gestion. The  lesions  resemble  simple  erosions.  Little  is  known  about 
the  etiology.  Perhaps  they  are  produced  after  death  by  the  self-diges- 
tion of  the  stomach,  but  there  is  some  evidence  for  thinking  that  they 
may  be  an  agonal  manifestation. 

PROGRESSIVE    METAMORPHOSES. 

Hypertrophy. — Hypertrophy  of  the  muscular  wall  of  the  stomach 
is  met  with  in  association  with  stenosis  of  the  pylorus,  chronic  gastritis, 
and  tumors.  The  whole  wall  may  be  affected  or  merely  the  portion  near 
the  pylorus.  The  congenital  hypertrophy  of  the  pylorus  has  already 
been  referred  to.  Most  cases  are  probably  to  be  classed  as  hypertrophies 
from  overwork.  In  chronic  gastritis,  there  may  be  an  overgrowth  of 
the  mucosa  in  the  form  of  flattened  sessile  nodules  (etat  mamelonne) 
or  polypoid  or  papillary  excrescences  (gastritis  polyposa).  A  somewhat 
similar  condition  is  found  occasionally  in  the  neighborhood  of  chronic 
ulcers.  The  proliferation  in  these  cases  may  begin  in  the  interstitial 
fibrous  tissue  or  in  the  glands. 


CARCINOMA 


417 


Tumors. — Connective-tissue  new-growths  are  rare  and  comparatively 
unimportant.  Fibromas,  lipomas,  and  myomas  have  been  met  with. 
We  have  seen  one  case  of  a  pedunculated  fibromyoma  springing  from 
the  serous  surface  at  the  fundus.1  Lymphangioma  beneath  the  serous 
covering  of  the  stomach  has  been  recorded. 

Sarcoma  is  rare.  Corner  and  Fairbank2  have  collected  58  cases. 
Gastric  sarcomas  may  be  subserous  and  pedunculated.  Some  of  them 
contain  unstriped  muscle  fibers  and  are  called  by  some  observers  malig- 
nant myomas.  In  one  such  case  Korinski  found  fibers  of  unstriped  muscle 
in  the  metastatic  growths  in  the  liver. 

Secondary  sarcomas  are  occasionally  met  with. 

Of  the  epithelial  new-growths,  adenoma  is  said  to  occur,  but  carcinoma 
is  by  far  the  most  frequent  and  important. 

FIG.  98 


Secondary  melanotic  sarcoma  of  the  stomach.     (From  the  Pathological  Museum  of 
McGill  University.) 

Carcinoma. — Carcinoma  of  the  stomach  usually  arises  after  middle 
life,  and  is  somewhat  more  common  in  men  than  in  women,  the  relative 
proportion  being  52  to  48  per  cent.  The  growth  may  originate  from  an 
apparently  healthy  mucosa  or,  occasionally,  in  an  old  peptic  ulcer. 
With  regard  to  position,  more  than  one-half  the  cases  are  situated  at  or 
near  the  pylorus.  Next  in  frequency  is  the  lesser  curvature,  and  next, 
the  cardia.  Gastric  carcinomas  vary  considerably  in  external  appear- 
ance, some  being  soft  and  fungating,  some  papillomatous,  some  ulcer- 
ating, and  others  scirrhous  or  gelatinous. 


27 


1  Nicholls,  Mont.  Med.  Jour.,  32:1903:326. 

2  Practitioner,  72:1904:  810. 


418 


THE  STOMACH 


Gastric  carcinomas  fall  into  five  classes — medullary,  adenocarcinoma, 
scirrhous,  colloid,  and  squamous     In  1348  cases  of  gastric  carcinomas 


Fio.  99 


Diffuse  scirrhous  carcinoma  of  the  stomach.     Note  the  great  thickening  of  the  wall. 
(From  the  Pathological  Museum  of  McGill  University.) 

FIG.  100 


^ir 


Scirrhous  carcinoma  of  the  pyloric  portion  of  the  stomach.     (From  the  Pathological 
Museum  of  McGill  University.) 

collected  by  Fen  wick  and  Fenwick,  863  were  medullary,  447  scirrhous, 
and  38  colloid.1 


1  Cancers  and  Tumors  of  the  Stomach,  1903. 


CARCINOMA  419 

The  medullary  form  is  characterized  macroscopically  by  the  formation 
of  a  soft,  spongy  mass,  with  smooth  surface,  but  divided  into  flattened 
nodes.  It  is  usually  situated  near  the  pylorus.  When  of  any  size,  it 
may  ulcerate,  owing  to  lack  of  nutrition.  Histologically,  the  new- 
growth  consists  chiefly  of  masses  of  cylindrical  or  isodiametric  cells,  with 
relatively  little  connective  tissue.  Fibrous  induration  of  the  stomach 
wall  is  a  frequent  accompaniment. 

The  adenocarcinoma  forms  large,  soft,  fungous  masses,  having  a 
shreddy  almost  papillomatous  appearance.  Microscopically,  the  epi- 
thelial cells  are  arranged  in  atypical  glandular  masses,  preserving  a 


FIG.  101 


•  •      ,  & 
• 

%'* 


Carcinoma  of  the  stomach.    Section  is  taken  through  the  muscular  coat.    Reichert  obj.  7a,  without 
ocular.     (From  the  collection  of  the  Pathological  Department  of  McGill  University.) 

more  or  less  complete  resemblance  to  the  gland-tubules  from  which  they 
spring.  There  is  a  small  amount  of  stroma,  often  infiltrated  in  places 
with  round  cells. 

Scirrhous  carcinoma  may  involve  the  whole  wall  of  the  stomach  or 
merely  the  pyloric  region.  The  affected  portion  is  much  thickened 
and  fibrous.  The  mucous  membrane  may  be  intact  over  the  growth, 
but  is  not  infrequently  absent.  In  the  diffuse  form  the  stomach  may 
be  so  contracted  that  its  cavity  can  contain  only  about  a  cupful.  Scirrhus 
at  the  pylorus  may  lead  to  cicatricial  stenosis,  with  dilatation  of  the  organ. 
Histologically,  the  main  portion  of  the  growth  is  formed  of  dense  con- 
nective tissue,  the  epithelial  elements  being  scanty.  Fibrous  induration 
of  the  muscular  coat  is  a  marked  feature. 


420 


THE  STOMACH 


Colloid  carcinoma  is  found  in  nodular  masses  or  as  a  diffuse  infiltra- 
tion of  the  stomach  wall.  When  incised,  the  tumor  is  composed  almost 
entirely  of  gelatinous  material,  sometimes  collected  into  cysts.  Micro- 
scopically, it  is  a  cylindrical-celled  carcinoma,  as  a  rule,  but  the  marked 
feature  is  the  tendency  to  colloid  change  exhibited  by  the  cells,  not  only 
the  epithelial  cells,  but  those  of  the  stroma  as  well. 

Squamous  epithelioma  is  rather  rare.  It  occurs  at  the  cardia,  and  prob- 
ably originates  in  the  mucosa  of  the  lower  end  of  the  oesophagus,  whence 
it  invades  the  stomach. 

Carcinoma  of  the  stomach  may  infiltrate  all  the  coats  of  the  organ  and 
even  bring  about  adhesion  to  neighboring  structures,  pancreas,  colon, 
liver,  omentum,  which  may,  in  turn,  be  invaded.  It  is  not  uncommon 

FIG.  102 


Colloid  carcinoma  of  the  stomach.  Winckel  No.  3,  without  ocular.  The  carcinoma  cells  are 
greatly  degenerated  and  have  been  replaced  by  colloid,  which  can  be  recognized  as  long,  stringy 
fibrils.  (From  Dr.  A.  G.  Nicholls'  collection.) 

to  find  dilated  lymphatics  on  the  serous  surface  of  the  stomach  filled  with 
carcinoma  cells.  The  lymph-nodes  about  the  lesser  curvature,  the  retro- 
peritoneal,  inguinal,  thoracic,  and  supraclavicular  nodes  are  in  time 
involved.  In  a  large  proportion  of  cases  metastasis  takes  place  through 
the  blood-vascular  system,  somewhat  in  contravention  of  the  general 
rule.  Secondary  growths  are  common  in  the  liver,  invasion  taking  place 
through  the  radicles  of  the  portal  vein. 

Medullary  and  especially  colloid  cancers  tend  to  invade  the  peritoneum 
and  give  rise  to  numerous  and  widely  distributed  secondary  growths 
resembling  in  type  the  parent  growth.  The  omentum  is  often  infiltrated 
and  distorted,  and  may  sometimes  be  recognized  as  a  transverse  band 


CARCINOMA 


421 


crossing  the  upper  part  of  the  abdomen.  Peritonitis  may  also  supervene. 
One  of  us  (A.  G.  N.1)  has  reported  a  somewhat  unusual  case  of  diffuse 
scirrhous  carcinoma  of  the  peritoneum  and  omentum,  with  contraction 
of  the  mesentery  and  chronic  peritonitis,  originating  in  a  scirrhous  car- 
cinoma of  the  stomach. 


FIG.  103 


Scirrhous  carcinoma  of  the  stomach,  omentum,  and  peritoneum.  In  this  case  the  new-growth 
was  diffused  throughout  the  stomach  wall  and  lead  to  a  peculiar  condition  of  carcinosis  of  the 
peritoneum  with  serofibrinous  peritonitis.  The  great  omentum  and  mesentery  were  curiously 
puckered  and  contracted.  The  nodules  show  very  well  in  the  figure.  (Nicholls.  Case  cited  in 
the  text.) 

When  situated  near  the  pylorus,  gastrectasis,  with  all  that  that  implies 
— retention  of  food,  fermentation,  gastritis,  excessive  vomiting — super- 
venes. Ulceration  may  lead  to  erosion  of  a  vessel  and  fatal  hemorrhage, 
or  to  perforation  and  peritonitis. 


1  Nicholls,  Jour.  Amer.  Med.  Assoc.,  40: 1903:  696. 


CHAPTER    XX. 

THE  INTESTINES. 
CONGENITAL  ANOMALIES. 

TOTAL  absence  of  the  intestines  and  similar  gross  defects  are  found 
only  in  connection  with  other  serious  malformations.  Partial  or  com- 
plete defect  of  the  appendix  has  been  observed.  Inverted  positions  of 
the  various  portions  are  recorded,  both  with  and  without  inversion  of 
the  abdominal  or  thoracic  viscera.  An  abnormal  course  of  the  colon  is 
common.  It  may  pass  diagonally  across  the  abdomen,  without  forming 
a  hepatic  flexure,  or  there  may  be  accessory  loops.  Perhaps  the  most 
frequent  form  is  a  downward  loop  of  V-shape,  which  is  found  in  9.5  per 
cent,  of  autopsies.  Hernial  protrusions  may  take  place  through  a  fissure 
of  the  abdominal  wall,  a  patent  inguinal  canal,  or  a  defect  in  the  dia- 
phragm. 

Congenital  narrowing  (stenosis)  or  imperforate  areas  (atresia)  occur. 
Sometimes  portions  of  the  bowel  are  defective.  Thus,  the  anus  with 
more  or  less  of  the  rectum  may  be  absent;  or  the  anus  may  open  into 
a  sac  which  does  not  communicate  with  the  colon  above.  Membranous 
septa,  transverse  or  longitudinal,  may  be  present.  Defects  of  the  lower 
bowel  and  urogenital  sinus  are  rather  common.  Thus  there  may  be 
a  large  cloaca  into  which  the  bladder  and  rectum  discharge.  The 
bladder  may  be  divided  and  the  large  intestine  absent,  so  that  the  ileum 
empties  into  the  bladder.  In  less  extreme  cases  there  may  be  merely 
incomplete  closure  of  the  septum  that  exists  between  the  rectum  and 
urogenital  sinus.  The  rectum,  when  defective,  may  end  blindly  or  dis- 
charge into  the  bladder,  urethra,  vagina,  or  perineum. 

A  common  anomaly  is  the  Meckel's  diverticulum,  found  in  our  experi- 
ence in  about  2.7  per  cent,  of  autopsies.  It  is  found  usually  in  the  ileum, 
within  a  meter  of  the  ileocecal  valve.  The  diverticulum  is  cylindrical, 
funnel-shaped,  or  larger  at  the  distal  end.  The  apex  is  sometimes 
rounded,  bifid,  or  lobulated.  The  average  length  is  about  7  cm.  The 
diverticulum  is  situated  away  from  the  mesenteric  attachment,  and 
represents  the  remains  of  the  omphalomesenteric  duct.  All  degrees  of 
patency  of  this  duct  may  exist.  Thus,  it  may  remain  open  for  its  whole 
length,  giving  rise  to  what  is  known  as  an  omphalomesenteric  fistula; 
it  may  be  open  at  the  umbilical  end — omphalic  fistula ;  it  may  be  closed  at 
both  ends,  forming  a  cyst,  when  situated  in  front  of  the  peritoneum 
called  a  preperitoneal  cyst,  when  attached  to  the  intestine  called  an 
enterocystoma.  Not  infrequently  the  Meckel's  pouch  is  attached  to  the 
umbilicus  by  a  fibrous  cord,  the  remains  of  the  involuted  duct. 


ACQUIRED  DIVERTICULA  423 

Structurally,  the  diverticulum  consists  of  all  the  layers  of  the  intestine, 
although  the  muscular  coat  may  be  somewhat  thinned  at  the  apex.  It 
may  have  free  communication  with  the  bowel,  or  may  be  partially 
shut  off  by  a  valve. 

The  importance  of  the  condition  lies  in  the  fact  that  the  diverticulum 
may  become  strangulated  and  inflamed,  or,  again,  may  perforate. 
\Yhere  the  fibrous  attachment  persists,  coils  of  intestine  may  be  caught 
and  strangulated. 

Congenital  Hypertrophy  and  Dilatation. — Congenital  hypertrophy  and 
dilatation  of  the  colon  (megacolon,  Hirschsprung's  disease)  is  a  peculiar 
condition  affecting  mainly  the  ascending  colon,  but  sometimes  also  to 
a  lesser  degree  the  transverse.  The  cause  is  unknown,  though  many 
cases  are  undoubtedly  developmental  peculiarities.  In  one  reported 
by  Formad,  the  colon  contained  22  kilos,  of  feces.  The  irritation  of 
the  retained  feces  leads  to  inflammation  and  ulceration  of  the  bowel, 
and  sooner  or  later  to  a  fatal  termination.  The  condition  has  been 
found  at  birth,  but  frequently  develops  during  early  childhood. 


ACQUIRED   ANOMALIES  OF  THE   LUMEN.     - 

Acute  Dilatation. — Acute  dilatation  of  the  intestines  (enteroplegia),  or 
acute  paralytic  distension,  is  strictly  comparable  in  regard  to  causes  and 
results  with  acute  dilatation  of  the  stomach.  Many  cases  arise  from 
acute  obstruction,  as  from  foreign  bodies,  adhesions,  volvulus,  or  hernias. 
An  important  etiological  factor  is  infection,  either  local  or  systemic.  The 
influence  of  local  infection  is  seen  in  the  cases  that  occur  in  connection 
with  peritonitis,  appendicitis,  cholecystitis,  or  other  inflammatory  condi- 
tions of  the  abdominal  viscera.  The  gastro-intestinal  paralysis  accom- 
panying pneumonia,  scarlatina,  and  meningitis  is  probably  to  be  attrib- 
uted to  the  influence  of  toxins  circulating  in  the  blood.  Blows  on  the 
abdomen  and  falls  have  been  followed  by  this  condition.  Some  cases, 
again,  have  followed  upon  anesthesia.  Some  few  have  come  on  apparently 
without  cause,  and  have  been  attributed  to  nervous  influences.  A  not 
inconsiderable  number  follow  surgical  operations  on  the  abdominal 
viscera,  no  doubt,  as  experimental  work  has  shown,  the  result  of  pro- 
longed handling  and  the  exposure  of  the  intestinal  coils  to  the  air. 

Chronic  Dilatation. — Chronic  dilatation  begins  insidiously  and  may 
take  months  to  develop.  Perhaps  the  most  common  cause  is  partial 
obstruction  of  the  lumen  of  the  intestine.  The  intestinal  wall  is  usually 
hypertrophied  as  well  as  the  cavity  dilated. 

Acquired  Diverticula. — Acquired  diverticula  of  the  intestine  are  not 
uncommon.  They  differ  from  the  true  diverticula,  of  which  the  Meckel's 
is  the  type,  in  that  they  are  often  multiple,  situated  between  the  layers 
of  the  mesentery  or  near  to  the  mesenteric  attachment,  and  are  usually 
composed  of  one  or  two  coats  of  the  bowel,  the  muscular  layer  being 
often  wanting.  In  many  cases  the  condition  is  really  a  hernia  of  the 
mucosa  into  the  serosa,  or,  again,  the  serosa  may  be  the  only  constit- 


424  THE  INTESTINES 

uent.  Most  cases  are  associated  with  chronic  pulmonary  affections  in 
old  people.  The  strain  of  coughing  is  apparently  the  exciting  cause, 
the  bowel  wall  giving  way  at  its  weakest  point,  namely,  where  the 
mesenteric  veins  leave  it  at  the  mesenteric  attachment.  After  middle 
life  there  seems  to  be  a  physiological  tendency  to  weakening  at  these 
points.  Other  cases  are  associated  with  ulceration  of  the  mucosa. 
Hansemann1  has  recorded  one  in  which  there  were  about  400  diver- 
ticula,  varying  in  size  from  that  of  a  hemp-seed  to  that  of  a  pigeon's  egg. 

Stenosis. — Stenosis  of  the  lumen  may  be  brought  about  by  cicatrizing 
processes  in  the  wall  of  the  intestine,  as  in  tuberculosis,  syphilis,  dysen- 
tery, or  uremic  ulceration;  new-growths;  compression  of  or  traction  of 
the  bowel  from  without,  as  from  tumors,  healing  peritonitis,  or  a  coil 
of  intestine  loaded  with  feces.  These  are  common  causes  of  chronic 
obstruction. 

Volvulus. — Volvulus  occurs  generally  between  the  ages  of  thirty 
and  forty  years.  In  this  condition  the  bowel  is  more  or  less  com- 
pletely obstructed  owing  to  a  twist  and  kink  about  its  long  axis.  In 
other  cases,  a  loop  of  intestine  is  twisted  upon  itself  much  as  in  the 
case  of  a  handkerchief  used  as  a  tourniquet,  or,  again,  one  coil  of  bowel 
may  be  twisted  about  another.  Half  the  cases  occur  in  the  sigmoid 
flexure.  The  next  most  common  site  is  in  the  small  intestine.  McFar- 
land  notes  having  met  with  one  case  involving  a  Meckel's  diverticulum. 
Volvulus  occurs  in  the  movable  portion  of  the  intestines,  and  is  thought 
to  be  brought  about  by  excessive  peristalsis  caused  by  unequal  filling 
of  the  coils  of  the  intestines  or  by  contusions.  An  abnormally  long 
mesentery  would  no  doubt  predispose. 

Intussusception  or  Invagination. — Intussusception  or  invagination  is 
the  condition  in  which  one  portion  of  the  bowel  slips  into  another,  much 
as  one  might  invert  the  finger  of  a  glove.  It  is  comparatively  frequent 
and  is  usually  met  with  in  the  young,  34  per  cent,  of  cases  occurring 
in  children  under  the  age  of  one  year.  Irregular  peristalsis  and  muscular 
spasm  are  believed  to  be  the  direct  causes.  Active  purgation,  diarrhoea, 
intestinal  irritation,  intestinal  ulcers,  and  polypi  are  believed  to  pre- 
dispose. Intussusception  may  be  acute  or  chronic,  single  or  multiple. 
Multiple  intussusceptions  of  the  bowels  are  quite  common  at  autopsies, 
and  are  probably  an  agonal  manifestation.  They  are  readily  distinguish- 
able from  pathological  intussusception  from  the  fact  that  they  are  readily 
reduced  and  there  is  no  sign  of  severe  constriction  of  the  bowel  or  of 
inflammation.  They  are  frequently  ascending,  while  the  true  intussus- 
ceptions are  almost  invariably  descending,  that  is  to  say,  the  upper  portion 
of  the  bowel  is  invaginated  into  the  lower.  Various  grades  of  invagina- 
tion occur,  and  if  unrelieved  the  condition  tends  to  become  aggravated. 

The  following  varieties  are  recognized:  The  ileocecal,  in  which  a  part 
of  the  ileum  and  the  ileocecal  valve  enters  the  colon;  the  ileocolic, 
in  which  a  portion  of  the  ileum  passes  through  the  valve;  the  Heal,  in 
which  the  ileum  is  alone  involved;  the  colic,  involving  the  colon  alone; 

1  Virch.  Archiv,  144: 1896:  400.     See  also  Fischer,  Jour.  Exp.  Med.,  5:  1901 :  333. 


ACQUIRED  ANOMALIES  OF  POSITION  425 

and  the  colicorectal,  involving  the  colon  and  rectum.  The  portion  of  the 
intestine  involved  forms  a  sausage-like  tumor  with  a  curved  outline. 
On  further  examination  of  the  mass  three  layers  of  bowel  are  recogniz- 
able, an  outermost  or  receiving  layer — the  intussuscipiens;  an  inner- 
most or  entering  layer,  and  a  middle  or  returning  layer,  both  together 
constituting  the  intussusceptum.  At  the  point  where  the  intussusceptum 
enters  the  intussuscipiens  there  is  generally  a  constriction,  termed  the 
neck.  The  part  of  the  intussusceptum  farthest  away  from  the  neck 
is  called  the  apex.  Intussusception  may  also  be  double,  the  originally 
invaginated  portion  being  carried  bodily  into  the  intestine  below.  As 
the  condition  progresses  the  inner  and  the  middle  layers  increase  at  the 
expense  of  the  outer.  As  a  result  of  the  constriction  the  circulation  is 
interfered  with  and  often  entirely  arrested.  In  the  early  stages  there 
is  slight  reddening  of  the  affected  parts,  with  possibly  the  exudation  of 
a  Jittle  plastic  lymph.  Later,  the  parts  are  greatly  swollen  and  con- 
gested, and  the  various  layers  are  glued  together  with  inflammatory 
exudate.  Finally,  necrosis  and  gangrene  result,  and  the  patient  often 
dies  from  obstruction,  peritonitis,  or  shock.  If  operative  measures  be 
undertaken,  the  invagination  can  usually  be  reduced  in  the  large  majority 
of  cases  (94  per  cent.)  during  the  first  twenty-four  hours,  but  the  longer 
operation  is  delayed  the  more  difficult  does  this  procedure  become. 
By  the  fourth  day  only  about  one-third  can  be  relieved.  The  amount 
of  intestine  invaginated  is  often  of  great  extent.  The  ileum  has  been 
known  to  pass  through  the  ileocecal  valve  and  appear  at  the  anus. 
An  uncommon  event  is  separation  of  the  necrotic  portion  and  spon- 
taneous healing.  One  instance  is  on  record  (Hermes)  in  which  60  cm. 
of  the  bowel  came  away  and  the  patient  recovered.  In  the  Pathological 
Museum  of  McGill  University  are  seventeen  inches  of  small  intestine 
passed  per  anwn  by  a  boy  who  had  all  the  symptoms  of  strangulation  of 
the  bowel  and  recovered.  In  such  cases  inflammatory  lymph  forms  a 
union  at  the  point  of  constriction,  which  subsequently  becomes  organized. 


ACQUIRED    ANOMALIES    OF    POSITION. 

The  term  hernia  or  rupture  is  applied  generally  to  denote  the  dislo- 
cation of  one  or  more  viscera  with  partial  or  complete  passage  through 
the  limiting  body  wall.  In  a  more  restricted  sense  it  is  applied  to  the 
intestines  and  associated  structures. 

Hernias  of  the  intestines  may  be  external  or  internal.  In  the  former, 
the  intestines  enter  the  inguinal  or  femoral  canals,  the  umbilicus,  the 
abdominal  wall,  or  the  obturator  foramen,  and  eventually  present 
externally  beneath  the  skin  and  subcutaneous  tissues.  In  the  latter, 
which  are  rarer,  the  intestine  passes  through  openings  in  the  omentum, 
mesentery,  the  foramen  of  Winslow,  or  into  pockets  of  the  peritoneal 
sac. 

The  chief  causes  operating  to  bring  about  hernia  are  weakness  of  the 
peritoneal  sac  at  some  particular  point,  either  acquired  or  congenital, 


426  THE  INTESTINES 

as  in  patency  of  certain  canals  that  are  usually  closed;  and  internal 
pressure,  brought  about  by  strain  or  the  weight  of  the  contained  viscera. 
In  some  few  cases  a  tumor  or  local  adhesion  may  exercise  traction  upon 
the  peritoneum  and  lead  to  the  production  of  a  sac. 

The  structures  entering  into  the  hernia  are  various.  As  a  rule,  they 
are  the  small  intestine,  mesentery,  and  omentum;  less  often,  the  cecum, 
large  intestine,  or  other  viscera,  such  as  the  stomach,  spleen,  liver,  ovary, 
uterus,  and  urinary  bladder.  As  the  intestine  or  other  organ  prolapses 
or  descends,  it  carries  before  it  a  prolongation  of  the  peritoneal  membrane. 
This  covering  is  absent  only  in  cases  in  which  the  membrane  is  torn,  or 
when  portions  of  the  intestine  that,  like  the  cecum,  are  extraperitoneally 
situated  are  prolapsed  through  an  opening  in  the  fascia  and  muscles  of  the 
abdomen.  In  the  early  stages  the  sac  is  represented  merely  by  a  shallow 
concavity,  but  later,  owing  to  pressure,  is  converted  into  a  globular  or 
pear-shaped  receptacle  communicating  with  the  abdominal  cavity  by 
a  narrow  neck.  Occasionally,  only  a  small  portion  of  the  intestinal  wall 
is  caught  in  the  sac,  the  main  portion  of  the  loop  being  free  (Littre's 
hernia).  In  such  cases  there  may  be  the  same  evidences  of  obstruction 
as  in  the  ordinary  type. 

External  Hernias. — Inguinal  hernia  is  the  most  common  form  of 
hernia,  and  is  more  frequent  in  men  than  in  women.  The  etiological  fac- 
tors are,  congenital  weakness  or  patency  of  the  inguinal  canal,  or  descent 
of  the  intestines  or  other  viscera  with  the  peritoneal  membrane  through 
the  canal.  If  the  organs  in  question  pass  through  the  internal  ring  and 
enter  the  canal,  but  without  escaping  from  the  external  ring,  the  condition 
is  termed  an  incomplete  inguinal  hernia.  If  it  passes  outside  of  the  external 
ring  it  is  called  a  complete  inguinal  hernia.  If  the  descending  organs 
enter  the  scrotum  it  is  a  scrotal  or  inguinoscrotal  hernia.  In  women  the 
hernial  protrusion  may  occur  into  the  labium  majus,  forming  an  in- 
guinolabial  hernia. 

Several  subvarieties  are  recognized.  Thus,  if  the  organs  present  at 
the  external  abdominal  ring  without  having  traversed  the  canal,  it  is 
called  a  direct  inguinal  hernia.  If  they  pass  in  the  ordinary  way  through 
the  canal,  the  hernia  is  termed  an  indirect  or  oblique  inguinal  hernia. 
Hernias  are  further  divided  into  external  and  internal  inguinal  hernias, 
according  as  the  prolapsed  organs  pass  to  the  outer  or  inner  side  of  the 
deep  epigastric  artery. 

Femoral  hernia  is  also  comparatively  frequent,  and  is  more  common  in 
women  than  in  men.  In  this  form  the  intestines  are  prolapsed  along  the 
course  of  the  femoral  vessels  and  make  their  appearance  on  the  inner 
side  of  the  thigh  just  below  Poupart's  ligament. 

Obturator  hernia  is  not  common.  It  occurs  at  the  obturator  foramen, 
along  the  course  of  the  obturator  artery  and  nerve. 

Umbilical  hernia  may  be  congenital,  but  perhaps  is  more  frequent  in 
women  who  have  borne  children.  Prolapse  takes  place  through  the 
umbilical  ring  or  into  the  cord.  The  hernia  is  often  large  and  usually 
contains  intestine  with  omentum.  Rarely,  the  liver  may  be  extruded  as 
well. 


INTERNAL  HERNIAS 


427 


FIG.  104 


Abdominal  hernia  is  a  term  applied  to  prolapse  of  the  viscera  through 
the  abdominal  wall  in  regions  other  than  at  the  usual  foramina.  The 
hernia  often  takes  place  between 
the  recti  muscles  in  an  abdomen 
rendered  lax  by  childbearing,  or  into 
the  scar  of  an  operation  wound. 

Ischiadic  hernia  occurs  at  the  great 
sacrosciatic  foramen. 

Perineal  hernia  is  rare.  It  takes 
place  between  the  bundles  of  the 
levator  ani  muscle. 

Lumbar  hernia  consists  in  a  pro- 
trusion of  the  intestine  into  the 
space  bounded  by  the  crest  of  the 
ilium,  the  external  oblique,  and  the 
latissimus  dorsi  muscles. 

Vaginal  hernia  is  very  rare,  and 
consists  in  a  descent  of  the  bowel 
between  the  rectum  and  uterus. 

Internal  Hernias. — The  most  fre- 
quent form  is  said  to  be  that  in 
which  the  intestine  passes  through 
a  hole  in  the  mesentery.  Hernias 
may  also  occur  through  the  foramen 
of  Winslow,  through  openings  in  the 
omentum,  into  the  fossa  duodeno- 
jejunalis,  the  subcecal  fossa,  and 
the  fossa  intersigmoidea.  Diaphrag- 
matic hernia  may  occur  as  a  result  of 
congenital  defects  in  the  diaphragm 
or  as  a  result  of  traumatism  (see 
p.  405). 

According  to  the  structures  enter- 
ing into  the  hernia,  various  types  are  recognized.     An  enterocele  contains 
some  part  of  the  intestine;  an  epiplocele,  a  portion  of  the  omentum;  a 
cystocele,  a  portion  of  the  bladder;  a  cecocele,  a  part  of  the  cecum; 
a  rectocele,  a  portion  of  the  rectum;  a  hysterocele,  the  uterus. 

Hernias  probably  never  are  cured  spontaneously.  When  the  pro- 
lapsed organs  can  be  replaced  in  their  normal  relations  the  hernia  is 
spoken  of  as  reducible;  when  the  hernial  contents  cannot  be  returned, 
the  hernia  is  irreducible.  Hernias  may  be  temporarily  irreducible, 
owing  to  the  presence  of  gas,  fluid,  or  feces,  or  permanently  irreducible 
from  the  formation  of  inflammatory  adhesions.  If  a  hernia  has  lasted 
any  length  of  time  secondary  changes  set  in,  chiefly  of  a  mechanical  or 
inflammatory  nature.  The  hernial  sac  becomes  thickened  and  its  con- 
stituent elements  fuse  together  so  that  it  may  no  longer  be  possible  to 
distinguish  the  original  layers.  The  inner  surface  is  smooth  and  pearly, 
often  traversed  by  elevated  ridges.  The  coils  of  intestine  and  omentum 


Hernia  through  the  umbilical  region  of  the 
liver,  appendix,  and  part  of  the  colon;  child 
aged  eight  months.  (Dr.  A.  E.  Vipond's  case.) 


428  THE  INTESTINES 

in  acute  conditions  may  be  adherent  to  each  other  or  to  the  wall  of  the  sac 
with  fibrinous  lymph  or,  in  old  cases,  by  firm  fibrous  bands.  The 
mesentery  is  often  shortened  and  deformed  from  old  inflammatory 
thickening.  Acute  infection  with  inflammatory  exudation  may  occur 
in  an  incarcerated  or  strangulated  hernia,  or  in  any  long-standing 
case.  The  mobility  of  the  intestines  gradually  becomes  so  much  im- 
paired that  the  intestinal  contents  cannot  be  passed  on  and  severe 
derangement  of  the  circulation  sets  in  (incarceration).  Pressure  may  be 
exerted  through  the  elasticity  of  the  tissues  forming  the  neck  of  the  sac 
or  from  the  weight  of  feces  within  the  coils.  If  the  pressure  be  so  great 
as  to  hinder  the  venous  return,  the  intestine  becomes  greatly  congested. 
The  coils  assume  a  purplish-red  color,  and  are  swollen  and  cedematous, 
though  they  may  for  a  time  preserve  their  lustre.  If  the  condition  be 
not  relieved  the  hernial  contents  become  greatly  inflamed  and  finally 
gangrenous.  The  bowel  then  presents  an  intense  blackish  or  bluish-red 
appearance,  with  possibly  suppurative  foci  here  and  there  which  lead 
to  perforation.  At  the  point  of  constriction,  which  is  usually  the  neck, 
the  tissues  are  of  a  pale  grayish-white  color  (strangulation}. 

Intestinal  Obstruction. — Intestinal  obstruction*  results  from  any 
condition  which  impedes  or  prevents  the  passage  of  the  bowel  contents. 
The  chief  etiological  factors  are  hernia,  strangulation,  intussusception, 
volvulus,  paralysis  of  the  bowels,  stenosis  and  atresia,  tumors,  and  ab- 
normal contents.  Hernia  and  strangulation  are  the  most  frequent 
causes.  In  an  analysis  of  1000  operative  cases,  Gibson1  found  35  per 
cent,  were  due  to  hernia,  19  per  cent,  to  constricting  bands,  19  per  cent, 
to  intussusception,  and  12  per  cent,  to  volvulus.  In  Leichtenstern's 
series  of  1134  cases,  35  per  cent,  were  due  to  strangulation.  The  fibrous 
adhesions  resulting  from  peritonitis  may  cut  off  pockets  from  the  general 
abdominal  cavity  or  form  bands  and  loops  through  which  coils  of  the 
intestine  may  become  prolapsed.  Slits  in  the  mesentery  or  great  omen- 
turn,  and  an  attached  Meckel's  diverticulum,  sometimes  act  in  the  same 
way.  Owing  to  peristaltic  action  or  to  distension,  the  prolapsed  bowel 
may  become  kinked  at  some  point  and  obstruction  ensue.  The  intestine 
may  also  be  obstructed  by  the  pressure  of  tumors,  cysts,  wandering 
organs,  or  the  traction  of  cicatricial  bands.  Polypi,  cysts,  tumors,  and 
healing  ulcers  may  obstruct  the  bowel  from  within. 

The  lumen  of  the  bowel  may  be  occluded  by  the  accumulation  of  feces 
which  have  become  inspissated.  Large  gallstones  have  been  known 
to  enter  the  bowel  and  fill  up  the  lumen.  Intestinal  worms  seldom 
cause  trouble  but  tangled  clusters  of  ascarides  have  sometimes  led  to 
obstruction.  Foreign  bodies  occasionally  are  introduced  through  the 
rectum,  but  usually  enter  through  the  mouth,  either  by  accident  or 
design.  A  great  variety  of  substances  have  been  found,  fruit  stones  and 
seeds,  hair,  false  teeth,  needles,  pins,  hairpins,  and  tacks 

Below  the  point  of  obstruction  the  bowel  is  empty  or  nearly  so,  while 
above  it  is  distended  enormously  by  gas  and  fecal  accumulation.  At 

1  Annals  of  Surgery,  32 : 1900:  486  and  676. 


HEMORRHAGE  429 

the  occluded  part  the  mucosa  is  often  eroded  and  the  bowel  wall  anemic 
as  a  result  of  pressure,  while  the  proximal  portion  of  the  intestine  is  con- 
gested, inflamed,  eroded,  or  necrotic.  Not  infrequently  a  considerable 
amount  of  transudate  is  found  free  in  the  abdominal  cavity.  In  chronic 
cases  the  intestinal  wall  is  considerably  thickened  as  well  as  dilated, 
owing  partly  to  functional  hypertrophy  and  partly  to  inflammatory 
infiltration.  In  obstruction  of  the  bowel,  the  virulence  of  the  contained 
microbes  is  greatly  increased,  while  at  the  same  time  the  resisting  power 
of  the  tissues  is  low,  so  that  not  uncommonly  peritonitis  results,  even  in 
the  absence  of  any  solution  of  continuity  of  the  bowel  wall. 


CIRCULATORY  DISTURBANCES. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  occurs  physio- 
logically during  digestion,  and,  pathologically,  as  a  result  of  irritation, 
infection,  or  dilatation  of  the  vessels  under  the  influence'  of  nervous 
stimuli.  The  intestines  are  also  congested  in  cases  of  peritonitis.  The 
mucosa  becomes  markedly  reddened,  but  the  condition  quickly  passes  off 
after  death,  so  that  the  appearances  at  autopsy  may  not  be  very  striking. 

Passive  Hyperemia. — Passive  hyperemia  arises  in  the  course  of  obstruc- 
tion in  the  general  systemic  circulation,  as  well  as  from  hepatic  cirrhosis 
or  similar  interference  with  the  portal  circulation.  Local  passive  con- 
gestion is  also  met  with  as  a  result  of  the  compression  of  the  mesenteric 
veins  in  the  conditions  of  hernia  and  strangulation.  The  affected  bowel 
in  these  cases  is  swollen,  oedematous,  purplish-red,  or  blue  in  color, 
and  the  serosa  is  possibly  slightly  roughened.  In  chronic  congestion 
the  mucosa  may  be  somewhat  pigmented. 

Varices. — Varices  occur  occasionally.  The  commonest  site  is  the 
rectum,  the  condition  being  induced  by  stasis  in  the  inferior  hemorrhoidal 
vein.  The  varices  are  to  be  found  within  the  rectum  or  externally  around 
the  anus,  in  the  form  of  small,  globular  or  polypoid  swellings  of  a  dull, 
bluish  color  (hemorrhoids).  The  chief  causes  are  sedentary  habits, 
chronic  constipation,  the  pressure  of  tumors  in  the  pelvis  or  of  the  preg- 
nant uterus,  and  obstruction  within  the  portal  circulation.  The  hemor- 
rhoids often  become  inflamed  and  ulcerated,  and  may  lead  to  hemorrhage. 
Repeated  loss  of  blood  in  this  way  sometimes  gives  rise  to  severe  anemia, 
the  cause  of  which  may  sometimes  be  overlooked. 

(Edema. — (Edema  results  from  active  or  passive  hyperemia,  acute 
and  chronic  inflammations,  peritonitis,  or  is  a  manifestation  of  general 
anasarca.  The  walls  of  the  intestine  are  firm  and  thickened,  giving  a 
sensation  to  the  fingers  resembling  that  of  wash-leather.  The  muscle 
is  pale  and  gelatinous,  and  the  mucosa  is  anemic  and  swollen,  with  a 
sodden,  watery  appearance.  The  natural  folds  are  accentuated. 

Hemorrhage. — Hemorrhage  into  the  mucosa  is  a  not  uncommon 
event  in  cases  of  active  and  passive  congestion,  in  embolism  or  thrombosis 
of  the  mesenteric  vessels,  in  pernicious  anemia,  hemophilia,  and  the 
hemorrhagic  diatheses. 


430  THE  INTESTINES 

Hemorrhage  into  the  lumen  results  from  inflammation,  ulceration, 
necrosis,  and  new-growths.  Dysentery,  typhoid  fever,  peptic  ulcer, 
ulcerating  carcinomas,  hemorrhoids,  are  the  most  important  causes. 
When  the  blood  is  effused  high  up  it  becomes  black  and  tarry  as  a 
result  of  the  action  of  the  digestive  juices  and  the  sulphuretted  hydro- 
gen in  the  feces;  when  escaping  lower  down  it  is  usually  red.  Severe 
bleeding  into  the  lumen  of  the  intestine  may  occur,  and  may  even  prove 
fatal  without  the  blood  escaping  externally.  This  is  termed  occult  or 
concealed  hemorrhage. 

Embolism  and  Thrombosis. — Embolism  and  thrombosis  of  the  mesen- 
teric  arteries  or  veins  lead  to  hemorrhagic  infarction  of  the  whole  area 
of  the  bowel  supplied  by  the  obstructed  vessels.  Hemorrhage,  often 
extensive,  takes  place  into  the  intestinal  wall,  which  undergoes  necrosis 
and  even  gangrene.  Peritonitis  often  supervenes,  owing  to  infection 
from  the  intestinal  contents.  The  symptoms  are  mainly  those  of  intes- 
tinal obstruction.  Helena  may  occur.  Some  of  the  ordinary  causes 
of  embolism  are  present,  endocarditis  or  arteriosclerosis.  Thrombosis 
is  usually  the  result  of  an  infective  thrombophlebitis  of  the  mesenteric 
veins. 

Several  observers  recently,  notably  Ortner,  have  drawn  attention  to 
the  clinical  importance  of  mesenteric  arteriosclerosis.  The  condition 
gives  rise  to  colic  with  alternating  attacks  of  diarrhoea  and  constipation. 


INFLAMMATIONS. 

The  inflammatory  disturbances  of  the  intestines  are,  on  the  whole, 
strikingly  like  those  of  the  stomach,  both  etiologically  and  anatomically. 
Any  differences  are  to  be  explained  by  variations  in  function  and  mechan- 
ical conditions.  Most  cases  of  inflammation  of  the  intestines  are  due  to 
the  irritative  action  of  the  intestinal  contents,  which  may  be  unsuitable, 
may  have  undergone  abnormal  fermentation,  or,  again,  may  contain 
toxic  substances  of  an  animal,  vegetable,  or  mineral  nature.  Some  cases, 
also,  are  due  to  infectious  or  toxic  agents  carried  to  the  intestine  by  the 
blood-stream  or  body  juices.  These  probably  act  by  depressing  the 
vitality  of  the  specific  tissue  cells,  by  modifying  secretion,  and  by  increas- 
ing the  virulence  of  microorganisms  contained  in  the  feces,  which,  under 
ordinary  circumstances  are  devoid  of  pathogenic  properties. 

The  portions  of  the  intestinal  tract  most  apt  to  suffer  from  inflam- 
matory disturbances  are  the  duodenum,  owing  to  its  close  association 
with  the  stomach,  and  the  large  bowel,  where  feces  and  other  irritating 
substances  are  liable  to  accumulate. 

Enteritis. — Inflammation  of  the  small  intestine  is  termed  enteritis; 
of  the  large,  colitis;  of  both,  enterocolitis.  If  the  stomach  be  involved 
as  well,  we  speak  of  gastro-enteritis.  Any  portion  of  the  bowel  may  be 
inflamed,  but  it  is  rare  for  it  to  be  affected  throughout  its  whole  extent. 
Therefore,  we  speak  of  duodenitis,  jejunitis,  ileitis,  typhlitis,  or  cecitis, 
colitis,  appendicitis,  sigmoiditis,  and  proctitis. 


FOLLICULAR  ENTERITIS  431 

Acute  Catarrhal  Enteritis. — Acute  catarrhal  enteritis  is  due  to  indiscre- 
tions in  diet,  improper  food,  or  food  containing  bacteria  of  certain  kinds 
or  bacterial  toxins.  Climatic  conditions  and  change  of  diet  also  seem 
to  have  a  considerable  effect  in  inducing  the  disease.  Acute  catarrhal 
enteritis  and  gastro-enteritis  is  especially  common  in  young  children,  in 
whom  it  is  produced  by  overfeeding  and  the  use  of  milk  laden  with 
microorganisms.  The  B.  dysenteriae,  B.  enteritidis,  B.  coli,  B.  proteus, 
and  the  streptococcus  are  the  germs  usually  present  in  these  cases. 
Catarrhal  enteritis  also  accompanies  or  complicates  certain  of  the  acute 
infective  fevers,  such  as  typhoid  and  pneumonia.  The  Cholera  Vibrio 
produces  an  acute  serous  enteritis. 

The  postmortem  appearances  in  acute  catarrhal  enteritis  are  not  con- 
stant, for  a  notable  amount  of  catarrh  may  exist  without  producing  any 
gross  lesions  that  are  recognizable  after  death.  In  some  cases  there  is  a 
patchy  congestion  of  the  mucosa  in  the  neighborhood  of  the  lymph-vessels, 
on  the  top  of  the  rugae,  and,  in  severe  cases,  of  the  serosa.  Punctate 
hemorrhages  are  occasionally  to  be  seen.  The  mucosa  is  often  swollen 
and  presents  a  dull,  cloudy,  grayish  appearance,  so  that  the  folds  are  more 
in  evidence  than  usual.  Generally,  though  not  invariably,  the  surface  is 
covered  with  a  mucous  or  serous  exudate  (serous  enteritis')  containing 
relatively  few  leukocytes.  In  other  cases  the  white  cells  are  more  abun- 
dant and  the  exudate  assumes  a  mucopurulent  or  purulent  character. 
The  exudate  may  contain,  also,  desquamated  epithelial  cells  in  varying 
stages  of  degeneration.  This  may,  possibly,  be  to  some  extent  due  to 
postmortem  maceration  of  the  tissues. 

Microscopically,  there  can  be  made  out  marked  hyperemia  of  the 
mucosa  and  submucosa,  with  some  oedema.  The  secreting  cells  of  the 
tubular  glands  show  evidences  of  increased  functional  activity  in  the 
presence  of  great  numbers  of  goblet  cells.  Slight  erosions  of  the  mucosa 
may  occur  and  the  surface  is  covered  with  more  or  less  exudate.  In  the 
severer  forms  collections  of  leukocytes  are  to  be  seen  in  the  mucosa  about 
the  gland-tubules  and  in  the  submucosa  around  the  bloodvessels.  The 
condition  of  simple  catarrh  may  pass  almost  imperceptibly  into  suppura- 
tion, and  considerable  portions  of  the  mucosa  may  slough  away,  leaving 
sharply  defined  ulcers  with  infiltrated  walls.  The  submucosa  contains 
leukocytes  in  great  numbers.  Suppurative  enteritis  may  lead  to  a  diffuse 
phlegmonous  condition  or  to  the  formation  of  localized  abscesses  in  the 
submucosa,  which  burst  into  the  lumen,  leaving  small  ulcers. 

Desquamative  Enteritis. — In  the  so-called  desquamative  enteritis  the 
mucosa  is  cast  off  en  masse  in  the  form  of  a  tubular  cast.  This  occurrence 
has  been  described  in  the  large  bowel  in  connection  with  the  summer 
diarrhoeas  of  children. 

Follicular  Enteritis. — The  part  played  in  enteritis  by  the  solitary 
and  agminated  glands  is  a  varying  one.  In  many  instances  these  struct- 
ures show  no  marked  changes,  but  in  others  they  are  so  greatly  affected 
that  the  disorder  may  properly  be  termed  follicular  enteritis.  In  this 
type  of  intestinal  inflammation,  the  follicles  are  swollen  and  project  above 
the  general  surface  of  the  mucosa,  where  they  are  recognizable  as  dots 


432  THE  INTESTINES 

of  a  grayish  or  grayish-white  color  on  a  more  or  less  hyperemic  back- 
ground. When  the  Peyer's  patches  are  affected,  owing  to  the  unequal 
swelling  of  the  lymphoid  and  connective-tissue  elements,  the  surface 
becomes  somewhat  reticulated  or  traversed  by  shallow  grooves.  Fol- 
licular  enteritis  is  not  infrequently  found  as  a  result  of  typhoidal  or 
tuberculous  infection,  and  is  particularly  common  in  diphtheria. 

Histologically,  the  swelling  of  the  follicles  is  due  to  hyperemia  and 
proliferation  of  the  lymphoid  cells.  There  is,  in  addition,  a  perifollicular 
cellular  infiltration  and  the  neighboring  lymph-vessels  are  filled  with 
cells.  The  follicles  usually  present  no  further  change,  but  now  and 
then  undergo  necrosis.  In  this  way  small,  rounded  abscesses  are 
formed  which  discharge  their  contents  and  give  rise  to  one  form  of 
ulcerative  enteritis. 

Membranous  Enteritis. — Under  the  term  membranous  enteritis  are 
classed  those  severer  forms  of  inflammation  of  the  intestines  which  lead 
to  coagulation-necrosis  and  ulceration.  There  may  be  produced  a 
"croupous"  or  fibrinous  exudation  upon  the  surface  of  the  mucous  mem- 
brane, which  becomes  fused,  as  it  were,  by  coagulative  changes  into  a 
more  or  less  homogeneous  mass — membranous  inflammation,  or  the 
exudate  may  be  both  on  and  within  the  substance  of  the  mucosa — 
diphtheroid  inflammation.  It  is  impossible  always  to  separate  these 
forms  one  from  the  other,  since  they  are  produced  by  the  same  causes, 
and  the  croupous  may  pass  imperceptibly  into  the  diphtheroid. 

Membranous  enteritis  is  usually  met  with  in  the  large  intestine 
(membranous  colitis),  but  occasionally  also  in  the  ileum.  It  probably 
begins  as  an  ordinary  catarrh,  with  congestion  and  swelling  of  the 
mucosa,  which  rapidly  progresses  into  a  more  severe  inflammation.  The 
mucosa  becomes  deeply  reddened  and  a  brawny  sort  of  film  of  a  whitish- 
gray  color  appears  upon  the  surface,  especially  upon  the  top  of  the  rugae. 
At  first  this  membrane  can  be  scraped  off  with  the  knife,  but  later  this 
becomes  impossible,  owing  to  the  fact  that  the  mucosa  undergoes  a 
form  of  coagulation  necrosis  and  the  exudate  and  mucous  membrane 
become  welded  into  an  indistinguishable  mass.  As  the  disease  pro- 
gresses, the  redness  and  swelling  increase,  the  patches  of  membrane 
tend  to  coalesce,  and  the  coagulated  substance  assumes  a  dirty,  yellowish- 
green,  or  brown  color  through  contact  with  the  feces. 

In  course  of  time  portions  of  the  membrane  and  necrotic  mucosa  may 
be  exfoliated,  giving  rise  to  ulcers  which  may  coalesce  and  reach  a  con- 
siderable size.  They  may  extend  superficially,  or,  again,  penetrate 
deeply.  The  denuded  tissues  in  such  cases  may  become  infected  by 
septic  microorganisms  and  a  local  suppurative  condition  or  a  diffused 
phlegmon  may  be  the  result.  If  the  condition  heal,  the  ulcers  cicatrize, 
the  mucosa  is  in  large  part  regenerated,  and  more  or  less  fibrous  thicken- 
ing of  the  intestine  remains. 

Special  Forms  of  Enteritis. — Dysentery. — Under  the  term  dysentery 
are  included  a  number  of  intestinal  inflammations,  which,  while  they 
vary  considerably,  have  this  in  common,  that  they  are  characterized 
clinically  by  severe  and  persistent  diarrhoea  with  tenesmus.  Patho- 


EPIDEMIC  DYSENTERY  DUE  TO  SHIGA'S  BACILLUS         433 

logically  speaking,  they  are  all  forms  of  colitis.  Dysentery  occurs 
epidemically,  endemically,  and  sporadically,  more  especially  in  tropical 
and  subtropical  climes,  but  also  occasionally  in  temperate  regions. 
The  disease  may  be  acute  or  chronic.  The  difficulties  in  the  way  of 
making  an  adequate  classification  of  the  forms  of  dysentery  are  great, 
owing  to  the  fact  that  the  etiological  factors  are  not  entirely  under- 
stood, nor  has  it  been  as  yet  possible  to  satisfactorily  correlate  our  know- 
ledge of  the  subject.  While  it  is  certain  that  the  majority,  if  not  all, 
the  dysenteries  are  to  be  attributed  to  the  activity  of  microorganisms, 
it  is  equally  certain  that  a  variety  of  differing  infectious  processes  have 
been  grouped  under  the  one  head.  It  is  probable  also  that  the  dysen- 
teries occurring  in  different  countries  are  characterized  by  specific 
differences. 

The  multitudinous  investigations  which  have  been  made  into  the 
subject  of  dysentery  during  the  past  few  years  seem  to  have  determined 
that  there  are  three  main  types:  (1)  A  form  due  to  irritation  from 
unsuitable  food,  preformed  poisons,  and  similar  causes;  (2)  a  form  due 
to  bacterial  activity;  and  (3)  a  form  due  to  animal  parasites  of  the 
protozoon  order.  We  know  little  as  yet  in  regard  to  the  irritative  or 
chemical  dysentery,  and  it  seems  to  have  little  to  differentiate  it  from 
other  forms  of  colitis. 

Acute  dysentery  of  bacillary  origin  for  the  most  part  is  due  to  infection 
with  one  of  two  germs,  the  B.  dysenterise  of  Shiga  and  the  Pseudo- 
dysentery  Bacillus  of  Flexner.  Some  few  cases  have  been  found  to  be 
associated  with  the  B.  pyocyaneus  (Calmette,1  Lartigau2),  and  with  a 
spirillum  (Le  Dan  tec3). 

Epidemic  Dysentery  Due  to  Shiga 's  Bacillus. — This  disease  affects 
the  large  intestine  and  the  lower  portion  of  the  ileum,  though  in  some 
cases  the  lesions  are  confined  to  the  rectum,  sigmoid  flexure,  and  lower 
portion  of  the  colon.  In  mild  cases  the  mucous  membrane  is  intensely 
congested  and  swollen  and  the  rugae  are  unusually  prominent.  Numer- 
ous small  hemorrhages  may  be  noted  in  the  mucosa  and  the  surface  of 
the  bowel  is  covered  with  viscid,  blood-stained  mucus.  The  lymph- 
follicles  are  swollen  and  there  are  often  superficial  erosions.  The  process 
may  eventually  assume  an  ulcerative  or  membranous  character.  The 
colon  may  be  of  a  grayish  or  greenish  color,  and  presents  brownish-black 
sloughs  or  eschars.  When  present,  the  membrane  may  be  confined  to 
the  tops  of  the  rugae  or  may  affect  larger  areas  of  mucosa.  In  the 
very  severe  cases,  the  wall  of  the  intestine  is  softened  and  thickened, 
while  the  submucosa  is  densely  infiltrated  with  pus  cells.  Peritonitis 
may  supervene. 

The  specific  cause  is  the  B.  dysenterise  of  Shiga,4  a  microorganism 
somewhat  closely  resembling  that  of  typhoid.  It  is  present  in  a  large 
proportion  of  cases  of  epidemic  dysentery,  is  pathogenic  for  experimental 

1  Quoted  by  Lartigau.  2  Jour,  of  Exper.  Med.,  3 : 1898 :  595. 

3  Comptes  rendus  de  la  Soc.  de  biol.,  1903:  16. 
4Centralbl.  f.  Bakt.,  23:1897:599;  ibid.,  24:1898:817,  870,  913. 
28 


434  THE  INTESTINES 

animals,  and  is  agglutinated  by  the  serum  of  immunized  animals  or  those 
suffering  from  the  disease. 

Epidemic  Dysentery  Due  to  Flexner's  Bacillus  (Institutional  dysentery 
or  Pseudodysentery). — This  form  is  due  to  a  bacillus  first  isolated  by 
Flexner1  from  cases  of  epidemic  dysentery  occurring  in  the  Philippine 
Islands  in  1900,  and  later  by  Kruse2  and  others  in  cases  occurring  in 
Germany.  In  America,  epidemic  dysentery  appears  to  be  more  often 
caused  by  Flexner's  bacillus  than  by  that  of  Shiga.  Flexner's  organism 
is  evidently  closely  allied  to  that  of  Shiga,  but  is  differentiated  from  it  by 
minor  cultural  peculiarities  and  by  the  fact  that  the  two  types  react 
differently  toward  immune  sera  prepared  from  the  two  strains. 

There  is  evidence,  too,  for  believing  that  there  are  a  number  of  micro- 
organisms differing  more  or  less  from  one  another  which  should  be 
included  in  the  dysentery  group.  Park,  Collins,  and  Goodwin3  have 
drawn  attention  to  a  form  intermediate  between  the  Shiga  and  Flexner 
types.  Many  cases  of  summer  diarrhoaa  in  children  have  been  shown 
to  be  due  to  Flexner's  organism.  Some  also  are  due  to  infection  with 
the  B.  pyocyaneus.4 

Endemic  or  Amoabic  Dysentery. — Amcebic  dysentery  is  a  form  confined 
almost  exclusively  to  the  tropics,  due  to  the  Amceba  coli  or  Amoeba 
dysenterise  (Kartulis;  Losch;5  Kruse  and  Pasquale;6  Councilman  and 
Lafleur7).  Small  epidemics  have  been  noted  in  New  York  and  Balti- 
more. The  lesions  are  found  usually  in  the  large  intestine,  particularly 
in  the  neighborhood  of  the  cecum  and  appendix,  and,  occasionally,  in 
the  lower  part  of  the  ileum.  In  the  earlier  stages  the  mucous  membrane 
is  cedematous,  swollen,  and  hyperemic,  and  there  are  local  infiltrations 
that  manifest  themselves  as  hemispherical  elevations  above  the  general 
surface.  Later,  the  mucous  membrane  covering  these  becomes  necrotic 
and  sloughs  away,  leaving  an  ulcer  with  thickened  undermined  edges, 
the  base  of  which  is  formed  of  infiltrated  submucosa  of  a  grayish-yellow, 
gelatinous  appearance.  It  is  this  infiltrated,  yellowish  mucosa  and  sub- 
mucosa that  is  the  most  distinctive  gross  feature  of  this  form  of  colitis. 
The  amount  of  softening  in  the  submucosa  is  often  far  in  excess  of 
the  superficial  necrosis,  so  that  a  kind  of  abscess  communicating  with 
the  lumen  of  the  bowel  by  a  small  sinus  is  produced.  In  severe  cases 
the  ulcers  may  coalesce,  forming  sinuous  tracks  bridged  by  strands  of 
comparatively  healthy  mucosa,  or  large  portions  of  the  mucosa  may 
disappear  leaving  only  small  islets  of  intact  membrane.  The  ulceration 
extends  more  or  less  deeply  and  may  eventually  reach  the  serous  coat. 
Perforation  of  the  bowel  is  rare.  As  a  rule,  in  the  advanced  cases  the  wall 

1  Centralbl.  f.  Bakt.,  28: 1900;  and  Philadelphia  Medical  Journal,  6: 1900:  414. 

2  Deutsche  med.  Woch.,  26:  1900:  637;  ibid.,  27:  1901:  370. 

3  Jour.  Med.  Research.  (6  N.S.) :  1904 : 553. 

4  K.  Cameron,  Montreal  Med.  Jour.,  24: 1895-6:  673. 

5  Virch.  Archiv,  65:  1875:  196. 

6  Zeitschr.  f.  Hyg.  u.  Infect.,  16: 1894: 1. 

7  Johns  Hopkins  Hosp.  Rep.,  2:  1891. 


CHOLERA  ASIATICA  435 

of  the  bowel  is  greatly  thickened.  Occasionally,  the  condition  may  be 
complicated  by  a  membranous  inflammation. 

Histologically,  there  is  a  more  or  less  extensive  necrosis  of  the  cells, 
beginning  in  the  submucosa,  and  an  infiltration  of  the  deeper  layers 
with  leukocytes.  There  is  also  proliferation  of  the  connective  tissue. 
The  walls  of  the  vessels  in  the  affected  area  are  infiltrated,  while  the 
vessels  themselves  are  filled  with  leukocytes  or  are  thrombosed.  The 
amoebae  are  found  in  the  tissues  in  the  base  and  edges  of  the  ulcers, 
but  rarely  in  parts  in  advance  of  the  active  lesions,  also  in  the  lymphatics 
and  bloodvessels. 

Plasma  cells  and  fibrin  may  be  abundant. 

The  stools  in  amoebic  dysentery  are  frequent,  bloody,  and  mucoid, 
later  of  a  greenish-gray  color,  fluid,  and  containing  mucin.  Amoebae 
are  to  be  found  in  the  dejecta. 

Among  the  complications  are  focal  necroses  in  the  liver  and  liver 
abscess.  The  frequency  with  which  liver  abscess  occurs  in  dysentery 
is  somewhat  uncertain.  Manson,1  in  3680  cases  of  tropical  dysentery, 
found  liver  abscess  in  21  per  cent.;  Hirsch,2  in  2377  autopsies  on  tropical 
dysentery,  noted  the  condition  in  19.2  per  cent.  Probably  in  these 
statistics  dysenteries  of  bacterial  origin  are  included  with  the  amoebic 
form.  Dysenteric  abscesses  of  the  liver  may  rupture  into  the  pleural 
cavity  and  into  the  lung.  The  necrotic  substance  is  of  a  peculiar  anchovy 
sauce-like  appearance  and  contains  the  amoebse. 

Cholera  Asiatica. — Cholera  is  an  acute  infectious  disease  charac- 
terized by  cramps,  diarrhoea,  vomiting,  fever,  and  collapse.  The  specific 
cause  is  the  cholera  vibrio  or  comma  bacillus  discovered  by  Koch.  The 
lesions  are  chiefly  confined  to  the  lower  portion  of  the  ileum.  In  general 
they  are  those  characteristic  of  a  serous  enteritis.  In  the  early  stages 
the  mucosa  of  the  small  intestine  is  more  or  less  congested  and  covered 
with  a  slight  amount  of  fibrin.  The  contents  of  the  bowel  are  thin  and 
watery,  cloudy,  alkaline,  and  of  a  grayish  color.  On  standing,  this 
material  deposits  a  quantity  of  small,  whitish  flakes  (rice-water  dis- 
charge), in  which  the  specific  microorganism  may  be  detected.  The 
mucous  membrane  is  usually  pale  and  anemic,  except  perhaps  in  the 
neighborhood  of  the  lymphoid  follicles,  where  it  is  rather  hyperemic. 
The  Peyer's  patches  and  the  solitary  follicles  are  swollen  and  rather 
pale.  Later,  they  shrink  somewhat  and  assume  a  reticulated  appear- 
ance. In  the  more  advanced  stages,  the  hyperemia  becomes  less  marked 
and  the  wall  of  the  intestine  is  more  or  less  swollen.  The  bowel  may 
also  be  nearly  empty.  The  lymphoid  elements  sometimes  present  a  slaty 
pigmentation.  Membranous  enteritis  may  supervene  as  a  secondary 
manifestation. 

Histologically,  one  sees  little  more  than  desquamation  of  the  super- 
ficial epithelium,  with  some  coagulation-necrosis  of  the  villi.  Leuko- 
cytic  infiltration  in  the  mucosa  is  but  slight  and  is  generally  absent  in 
the  deeper  layers. 

1  Quoted  by  Robinson,  Jour.  Amer.  Med.  Assoc.,  36: 1901 : 1319. 

2  Handb.  Gen.  and  Hist.  Path.,  London,  3: 1886:  412. 


436  THE  INTESTINES 

Mucous  Colitis. — Mucous  colitis  is_a  curious  affection  of  the  large 
bowel  in  which  tubular  and  membranous  casts  are  discharged  in  the 
evacuations.  The  condition  appears  to  be  due  to  hypersecretion 
with  inspissation  of  the  mucus  and  proteids  produced.  It  is  found  most 
often  in  hysterical  women  and  is  probably  a  secretory  neurosis.  Only 
rarely  is  it  due  to  inflammation  of  the  bowel. 

Typhoid  Fever. — -Typhoid  fever  is  an  acute  infectious  disease  due 
to  the  Bacillus  typhi  abdominalis  (Eberth-Gaffky).  Properly  speaking, 
it  is  a  disease  of  the  system  as  a  whole,  but  the  most  constant  and  charac- 
teristic lesions  are  to  be  found  in  the  intestines.  The  portal  of  infection 
is  usually  some  part  of  the  bowel,  but  exceptional  cases  have  been 
recorded  where  infection  has  taken  place  through  the  lungs  (Roux, 
Sicard,  Dufaud). 

As  a  rule,  the  lesions  are  to  be  found  in  the  last  three  feet  of  the  ileum 
and  in  the  first  part  of  the  colon.  In  a  few  cases  the  colon  alone  is 
affected  (Hodenpyl1).  In  rare  instances  typhoid  assumes  the  character 
of  a  generalized  septicemia,  the  characteristic  lesions  in  the  intestine 
being  wholly  wanting.  Cases  of  this  kind  have  been  recorded  in  America 
by  Flexner  and  Harris,2  Nicholls  and  Keenan,3  Lartigau,4  McPhedran,5 
Opie  and  Bassett,6  and  others. 

In  typical  cases  of  typhoid,  the  mucous  membrane  of  the  intestine  is 
swollen  and  congested,  presenting  all  the  signs  of  an  acute  catarrhal 
inflammation.  The  characteristic  lesions,  however,  are  to  be  found  in 
the  Peyer's  patches  and  solitary  follicles.  The  lymphoid  structures  are 
invaded  by  the  specific  microorganism,  become  inflamed,  and  proliferate 
actively.  The  Peyer's  glands,  therefore,  are  enlarged,  soft,  and  in- 
tensely reddened,  and  project  above  the  general  surface  as  flattened 
plaques.  The  solitary  follicles  also  may  be  so  much  swollen  that  they 
appear  like  little  polyps  on  a  slender  pedicle.  At  the  height  of  the  first 
stage  the  surface  of  the  affected  plaques  presents  a  curious  grooved 
appearance  (plaques  a  surface  reticulee).  This  appearance  is  due  to 
the  unequal  swelling  of  the  connective-tissue  stroma  and  the  lymphoid 
elements. 

Histologically,  at  this  period,  the  mucous  membrane  is  in  a  catarrhal 
condition  and  there  is  intense  congestion  of  the  vessels  both  of  the  mucosa 
and  the  submucosa.  The  lymphoid  elements  show  hyperplasia,  and  there 
is  a  perifollicular  infiltration  with  lymphocytes  and  leukocytes.  Leuko- 
cytes may  be  discovered  in  all  the  coats  of  the  bowel,  even  in  the  serosa. 

At  the  beginning  of  the  second  week,  portions  of  the  affected  follicles 
undergo  a  form  of  coagulation  necrosis.  The  central  parts  slough  away, 
leaving  superficial  erosions,  which  often  assume  a  brownish  color  from 

1  Brit.  Med.  Jour.,  2 : 1897 : 1850. 

2  Johns  Hopkins  Hosp.  Bull.,  8: 1897:259. 

3  Mont.  Med.  Jour.,  27:  1898:9. 

4  Johns  Hopkins  Hosp.  Bull.,  10:1899:55. 

5  Phila.  Monthly.  Med.  Jour.,  October,  1899. 
8  Phila.  Med.  Jour.,  7: 1901 : 99. 


TYPHOID  FEVER 


437 


FIG.  105 


bile  staining.  In  the  course  of  the  next  few  days,  the  sloughing  process 
gradually  extends  until  well-defined  ulcers  are  produced,  having  a  fairly 
smooth  base,  but  with  swollen  and  infiltrated  edges.  The  ulceration 
tends  to  penetrate  into  the  depths  and  usually  extends  as  far  as  the 
muscularis  mucosse,  which  may  be  recog- 
nized by  its  clean,  ribbed  appearance.  The 
well-defined,  typical,  typhoid  ulcer  is  some- 
what oval,  the  long  axis  running  the  long  way 
of  the  bowel,  the  base  is  smooth,  the  edges 
comparatively  thin  and  undermined.  Small 
ulcers  may  be  irregular,  especially  when  they 
do  not  involve  the  whole  extent  of  the  Peyer's 
patch,  or,  again,  may  be  small  and  round, 
when  involving  a  solitary  follicle.  As  a 
rule,  the  most  advanced  ulcers  are  to  be 
found  in  the  region  of  the  ileocecal  valve 
and  become  less  marked  as  one  passes  up 
the  ileum.  In  very  severe  cases  the  last 
foot  or  so  of  the  small  bowel,  together  with 
the  head  of  the  cecum,  may  be  converted 
into  a  large,  gangrenous  eschar  in  which 
may  be  recognized  here  and  there  small 
islets  of  the  original  mucosa,  the  ulcerating 
process  having  extended  far  beyond  the 
limits  of  the  Peyer's  glands. 

The  process  of  healing  varies  naturally 
with  the  intensity  of  the  process.  The 
plaques  that  are  merely  hyperplastic 
become  soft  again  and  hyperemic  through 
gradual  absorption  of  the  inflammatory 
products.  Not  uncommonly,  there  is  an 
extravasation  of  red  corpuscles  from  the 
vessels,  so  that  hemorrhagic  infiltration 
occurs.  Where  ulceration  has  occurred, 
the  border  of  the  necrotic  areas  becomes 
less  swollen  and  again  suffused  with  blood. 
Not  infrequently,  there  is  hemorrhagic  infil- 
tration of  the  tissues,  a  fact  that  probably 
accounts  for  those  cases  of  intestinal  hemor- 
rhage which  occur  later  on  in  the  stage  of 
beginning  convalescence.  The  glandular 
elements  of  the  still  intact  mucosa  prolifer- 
ate and  gradually  spread  over  the  denuded 
area,  thus  restoring  the  original  continuity 
of  epithelium.  When  the  ulceration  has  been  very  extensive,  the  glandu- 
lar regeneration  may  be  incomplete  or  absent,  so  that  the  floor  of  the 
ulcer  is  covered  by  a  layer  of  connective  tissue  containing  no  or  relatively 
few  imperfectly-formed  glandular  elements.  Healed  ulcers  may  often 


Small  intestine.  Peyer's  plaques 
showing  tumefaction  and  superficial 
ulceration.  Typhoid  fever.  (From 
the  Pathological  Museum  of  McGill 
University.) 


438  THE  INTESTINES 

be  recognized  as  oval  patches,  which  are  smooth,  pigmented,  and 
somewhat  depressed  below  the  general  surface  of  the  bowel.  During 
the  first  eight  or  ten  days  the  specific  bacilli  can  be  recognized  in  the 
Peyer's  patches  in  well-marked  clumps.  Coincidently  with  the  intestinal 
involvement,  the  mesenteric  glands  and  spleen  are  swollen,  hyperplastic, 
and  inflamed.  They  may,  as  a  consequence,  be  greatly  softened. 
Spontaneous  rupture  of  the  spleen  has  occurred,  but  is  rare.  The 
mesenteric  glands  may  suppurate. 

Owing  to  the  tendency  of  the  typhoidal  ulceration  to  penetrate  deeply, 
perforation  of  the  bowel  is  not  uncommon,  leading  usually  to  a  fatal 
peritonitis.  Because  of  the  peculiar  asthenic  type  of  the  typhoidal 
infection  and  the  fact  that  leukocytes  are  scanty,  the  exudate  is  poor  in 

FIG.  106 


Intestine  in  typhoid  fever.  Section  shows  a  sinus  in  a  Peyer's  patch  with  the  great  prolifera- 
tion of  the  epithelial  cells.  Zeiss  obj.  TV,  oil  immersion,  ocular  No.  1.  (From  the  Pathological 
Laboratory  of  McGill  University.) 

fibrin  and  adhesions  do  not  readily  form.  Only  twice  have  we  seen  a 
typhoidal  perforation  closed  by  adhesion  of  the  great  omentum.  Per- 
foration occurred  54  times  in  2036  cases  of  typhoid  treated  at  the 
Royal  Victoria  Hospital,  Montreal;  that  is,  in  2.66  per  cent. 

Another  dangerous  complication  is  hemorrhage  due  to  the  erosion  of 
some  vessel  in  the  ulcerated  area.  This  accident  occurred,  including 
fatal  and  non-fatal  cases,  in  6.04  per  cent.  Other  complications  are 
pneumonia,  pleurisy,  nephritis,  septicemia,  periostitis,  thrombo-phlebitis, 
thrombo-arteritis,  cholecystitis,  cholelithiasis,  endocarditis,  and  menin- 
gitis. A  general  hemorrhagic  diathesis  may  occur.1 

The  pathogenesis  of  typhoid  fever  has  been  the  subject  of  considerable 

1  Nicholls  and  Learmonth,  Lancet,  London,  1 : 1901 :  305. 


TUBERCULOSIS  439 

discussion.  Virchow  thought  that  the  swelling  of  the  plaques  was  due 
to  oedema  and  inflammatory  exudation,  a  view  subsequently  modified 
by  the  researches  of  Rokitansky  and  Hoffmann.  The  more  recent 
studies  of  Mallory1  have  thrown  a  flood  of  light  upon  the  subject  and 
have  placed  it  on  a  more  satisfactory  footing.  According  to  Mallory, 
the  essential  feature  of  typhoid  is  a  proliferation  of  the  endothelial 
cells  throughout  the  body,  a  change  which  he  thinks  is  due  to  the  action 
of  a  diffusible  toxin  derived  from  the  bacilli.  The  lesion  in  question  is 
found  in  the  Peyer's  patches,  mesenteric  glands,  liver,  and  bone-marrow, 
as  well  as  in  the  lymphatics  and  blood-capillaries,  but  is  proportionately 
more  intense  the  nearer  to  the  point  at  which  the  infecting  agent  gained 
entrance.  The  endothelial  plates  attached  to  the  fibrous  meshwork  of 
the  lymphoid  follicles  and  mesenteric  glands,  as  well  as  those  lining  the 
capillaries,  proliferate,  become  fused  into  plasmodial  masses  or  giant 
cells,  and  act  as  phagocytes.  They  ingest  the  bacteria  and  slowly  eat 
up  the  lymphoid  cells,  which  thus  gradually  disappear.  A  few  leuko- 
cytes are  to  be  seen  in  the  follicles  and  within  the  crypts  of  Lieberkiihn, 
but  are  not  an  important  feature.  Owing  to  the  massing  of  these  endo- 
thelial cells  within  the  capillaries  and  the  consequent  obstruction  to  the 
blood  supply,  the  parts  deprived  of  their  nutrition  undergo  necrosis.  The 
focal  necroses  in  the  liver  and  spleen  are  to  be  explained  in  the  same  way. 

Paratyphoid  Fever. — Paratyphoid  fever  is  an  affection  that  can 
be  differentiated  with  difficulty  from  typhoid,  both  as  regards  its  clinical 
course  and  the  anatomical  lesions  produced.  The  intestinal  manifesta- 
tions vary.  As  a  rule,  they  resemble  the  lesions  of  dysentery  rather 
than  typhoid.  In  some  cases  they  are  absent.  The  specific  micro- 
organism is  a  form  intermediate  in  properties  between  the  B.  typhi  and 
the  B.  coli.  The  only  conclusive  point  in  the  differential  diagnosis 
between  typhoid  and  paratyphoid  fever  is  the  agglutination  test. 

Tuberculosis. — This  is  one  of  the  most  frequent  conditions  found 
in  the  intestines.  Three  anatomical  forms  may  be  differentiated,  the 
ulcerative,  miliary,  and  the  hyperplastic.  In  the  vast  majority  of  cases 
the  condition  is  a  secondary  one,  being  due  to  the  infection  of  the  bowel 
by  bacilli  derived  from  a  more  or  less  distant  focus.  The  most  common 
event  of  this  nature  is  the  ulceration  of  the  intestines  that  accompanies 
pulmonary  tuberculosis,  the  result  of  swallowing  infective  sputum. 
Occasionally,  caseous  glands  rupture  into  the  oesophagus  or  intestines 
and  bring  about  infection.  A  hematogenous  origin  is  certainly  rarer. 

With  regard  to  primary  tuberculosis  of  the  intestines,  statistics  are 
conflicting,  and  it  is  difficult  to  get  reliable  information  as  to  its  frequency. 
The  preponderance  of  the  evidence  at  present  available  goes  to  show 
that  the  milk  of  tuberculous  cattle  often  contains  virulent  bacilli  which 
are  competent  to  produce  intestinal  disease  in  animals  fed  upon  the 
infective  material.  It  is,  therefore,  fairly  generally  held  that  tuberculous 
cattle  are  a  grave  menace  to  the  health  of  the  community,  and  more 
especially  to  children  in  whose  dietary  milk  forms  such  an  important 
part.  At  present  it  seems  impossible  to  decide  with  certainty  to  what 
1  Jour.  Exper.  Med.,  3:  1898:  611. 


440  THE  INTESTINES 

extent  primary  infection  of  the  intestine  takes  place,  though  we  must 
undoubtedly  agree  with  Koch  that  it  is  comparatively  rare.  In  1230 
autopsies  at  the  Royal  Victoria  Hospital,  Montreal,  potentially  infective 
tuberculous  lesions  were  found  in  285  cases,  but  only  two  were  un- 
doubted instances  of  primary  intestinal  infection,1  although  seven  others 
were  probably  of  this  nature.  During  five  years  at  the  Charite  Hospital 
at  Berlin,  Koch  only  met  with  ten  examples.  Kossel,2  in  286  consecutive 
autopsies  on  children,  of  whom  22  had  died  of  tuberculosis,  in  only 
one  found  the  affection  confined  to  the  intestinal  tract.  Hunter3  in 
5142  autopsies  in  China  found  only  five  instances.  On  the  other  hand, 
Spengler4  refers  to  92  cases  of  tuberculosis,  in  4  of  which  the  intestinal 
tract  was  alone  affected.  Still,5  in  269  autopsies  on  cases  of  tuberculosis 
in  children  under  twelve,  found  that  where  the  course  of  infection  could 
be  determined  with  some  certainty  the  lungs  were  attacked  primarily 
in  105  cases,  the  intestines  in  53,  the  ear  in  9,  the  bones  and  joints  in  5. 
Shennon6  in  355  cases  of  tuberculosis  in  children  found  the  primary 
seat  of  infection  to  be  intestinal  in  28.1  per  cent.  Apparently  primary 
intestinal  infection  is  more  common  in  England  and  France  than  it  is 
in  America  and  Germany.7 

In  typical  cases  of  tuberculosis  of  the  intestines  resulting  from  infection 
of  the  alimentary  type,  the  process  begins  in  the  Peyer's  plaques  and 
solitary  follicles  in  the  form  of  small  nodular  elevations  beneath  the 
mucous  membrane.  These  are  tubercles  or  specific  granulomata.  In 
a  short  time  the  central  portion  changes  to  a  yellowish-white  color, 
an  evidence  of  central  necrosis  and  caseation.  Later,  this  softens  and 
is  discharged,  so  that  a  small  ulcer  with  infiltrated  borders  is  produced, 
in  the  neighborhood  of  which  other  minute  caseous  foci  appear.  These 
ultimately  coalesce,  forming  a  larger  ulcer.  A  typical  tuberculous  ulcer 
of  the  intestine  has  the  following  peculiarities:  the  edges  are  irregular, 
infiltrated,  but  not  usually  undermined;  the  base  is  uneven,  ragged, 
and  necrotic,  and  in  it  can  often  be  seen  small,  yellowish,  rounded  masses, 
which  are  caseous  tubercles;  the  ulcers  tend  to  encircle  the  bowel,  owing 
to  the  fact  that  extension  of  the  infection  takes  place  along  the  course 
of  the  lymphatics;  the  ulcers  do  not  tend  to  perforate;  small  isolated 
tubercles  can  often  be  seen  upon  the  serous  surface  of  the  bowel;  the 
mesenteric  lymph  nodes  are  often  caseous.  The  tissues  about  the  ulcer 
usually  show  little  disturbance,  but  there  may  be,  in  addition,  a  catarrhal 
enteritis.  Certain  catarrhal  and  ulcerative  processes  in  the  bowel  and 
even  membranous  and  gangrenous  enteritis  have  also  been  attributed 
to  the  action  of  the  tubercle  bacillus,  but  it  is  uncertain  in  how  far  these 
may  be  due  to  associated  microorganisms.  We  have  met  with  one  case 
of  follicular  enteritis  in  which  the  puriform  material  from  the  inflamed 
follicles  contained  tubercle  bacilli. 

1  Nicholls,  Mont.  Med.  Jour.,  31:1902:327.  2  Zeit.  f.  Hyg.,  12:  59. 

3  Brit.  Med.  Jour.,  1 :  1904: 1126.  4  Zeit.  f.  Hyg.,  13: 1893:  346. 

5  Brit.  Med.  Jour.,  2 : 1899 : 455.  6  Edin.  Hosp.  Rep.,  1900. 

7  A  useful  resume"  of  recent  work  on  this  controverted  subject  will  be  found  in  the 
British  Medical  Journal,  1 :  1909 :  epitome,  7. 


TUBERCULOSIS 


441 


FIG.  107 


The  tuberculous  process  usually  extends  into  the  muscular  coats  and 
even  into  the  serous  membrane,  infection  taking  place  along  the  course 
of  the  lymphatics.  Thus,  we  may  find  a  small  crop  of  tubercles  on 
the  serous  surface,  surrounded  by  congested  and  newly-formed  capil- 
laries. Not  infrequently,  there  can  be  seen  also  about  these  a  little  knot 
of  dilated  lymph-channels  filled  with  clear  fluid  or  cellular  and  caseous 
material,  vessels  which  have  become 
blocked  from  the  tuberculous  infiltra- 
tion. In  the  neighborhood  of  these 
subserous  tubercles  there  is  often  a  local 
peritonitis,  evidenced  by  a  slight  dulling 
of  the  peritoneal  membrane  and  possibly 
a  deposit  of  delicate  fibrin.  As  the  ulcers 
coalesce  and  enlarge,  they  tend  to  extend 
transversely  around  the  bowel,  forming 
a  girdle-shaped  area  of  erosion.  In 
cases  where  the  Peyer's  patches  are 
involved,  especially  where  the  process 
has  not  had  time  to  progress  very  far, 
the  ulcers  may  resemble  the  typhoidal 
form,  in  that  they  are  oval  or  rounded, 
their  long  axes  running  the  long  way  of 
the  bowel. 

Histologically,  the  structures  at  the 
edges  and  base  of  the  ulcers  present  the 
ordinary  features  of  granulation  tissue. 
In  this  can  be  made  out  a  varying 
number  of  tubercles,  composed  of  the 
usual  leukocytes,  epithelioid  and  giant 
cells,  with  a  certain  amount  of  central 
caseation.  In  the  more  advanced  cases 
similar  tubercles  can  be  found  between 
the  muscle  bundles  and  along  the  course 
of  the  lymphatics  in  the  serous  coat. 
Great  numbers  of  "  Mast-zellen"  are 
also  to  be  seen  in  the  submucosa  and 

muscularis.  The  muscular  coat  shows  a  certain  amount  of  fatty 
degeneration.  By  appropriate  methods  tubercle  bacilli  can  be  demon- 
strated in  the  granulomatous  areas. 

An  interesting  and  important  form,  that  has  of  late  been  attracting 
some  little  attention,  is  the  so-called  chronic  productive  or  hyperplastic 
tuberculosis  of  the  intestines.  In  this  type  tissue  destruction  is  trifling, 
while  cell  proliferation  is  in  excess.  The  condition  may  involve  the  peri- 
toneal membrane  as  well,  either  wholly  or  in  part,  or,  again,  may  be  one 
manifestation  of  a  widespread  tuberculosis  of  all  the  serous  sacs.1  When 


Tuberculous  ulceration  of  the  bowel. 
(From  the  Pathological  Museum  of 
McGill  University.) 


1  Nicholls,  Some  Rare  Forms  of  Peritonitis  Associated  with  Productive  Fibrosis 
and  Hyaline  Degeneration,  Jour.  Amer.  Med.  Assoc.,  40:1903:696. 


442 


THE  INTESTINES 


localized,  the  lesions  are  generally  found  in  the  cecum  and  appendix 
or  in  that  neighborhood.  Occasionally,  certain  coils  of  the  large  or  small 
intestine  are  matted  together,  thickened,  and  rolled  up  into  a  ball,  owing 
to  sclerotic  induration  and  retraction  of  the  mesentery,  and  the  omentum 
may  be  converted  into  a  thick  cord.  The  wall  of  the  affected  bowel  is 
enormously  thickened,  sometimes  measuring  as  much  as  1  cm.  The 
infiltration  may  in  fact  be  so  great  that  a  tumor-like  mass  is  formed 
which  can  be  felt  through  the  abdominal  wall.  This  has  been  mistaken 
for  a  carcinoma.  The  infiltrated  bowel  may  be  surrounded  by  dense 
fibroid  adhesions,  which  may  anchor  it  to  the  parietes.  If  caseation  be  at 
all  active  in  such  cases,  fistulous  communications  may  be  formed  with  the 

FIG.  108 


Intestine  showing  two  small  caseous  foci  of  tubercle  in  the  submucosa.     Zeiss  obj.  DD, 
ocular  No.  1.     (From  the  Pathological  Laboratory,  McGill  University.) 

exterior  of  the  body  and  between  the  loops  of  the  gut.  The  lumen  of  the 
intestine  is  sometimes  narrowed  to  such  an  extent  as  to  cause  obstruction, 
but  there  is  rarely  ulceration  of  the  mucosa.  Histologically,  the  main 
feature  is  extensive  hyperplasia  of  the  connective  tissue  in  which  typical 
tubercles  are  scanty  or  even  absent.  Caseation  is  rarely  extensive,  and 
the  specific  bacilli  may  be  hard  to  demonstrate.  A  striking  feature  in 
some  cases  is  a  hyaline  degeneration  of  the  exudate  and  of  the  newly- 
formed  connective-tissue  fibrils.1 

1  The  following  papers  may  be  consulted  on  this  subject:  Lartigau:  Jour,  of 
Exper.  Med.,  6:  1901-5:  23;  Koerte:  Deut.  Zeit.  f.  Chir.,  1894  to  1895:  60:  562; 
Coquet:  These  de  Paris,  De  la  varied  chir.  de  Tumeurs  cecales  tuber.,  1894:28; 
Itie":  These  de  Montpellier,  De  la'  tuber,  intest.  &  forme  hyper.,  1898: 12. 


OF   THE 

UNIVERSITY 

OF 

^  ooENirrs  443 

Syphilis. — Syphilis  of  the  intestines  may  be  hereditary  or  acquired.. 
In  the  hereditary  form,  the  small  intestine  is  perhaps  the  part  most 
frequently  involved,  and,  moreover,  unlike  what  occurs  in  other  infectious 
diseases,  it  is  the  upper  portion,  usually  the  jejunum,  that  is  affected. 
Cellular  infiltrations  and  gummata  have  been  observed,  which  lead 
to  ulceration. 

The  lesions  of  acquired  syphilis  are  most  frequently  localized  in  the 
rectum,  rarely  in  the  colon  and  small  intestine.  The  chancre  has  under 
certain  circumstances  been  found  in  the  rectum,  as  well  as  specific 
condylomas  and  papules.  The  common  lesion  is,  however,  the  gumma. 
Here  the  inflammatory  process  begins  in  the  submucosa  and  leads  to 
extensive  ulceration.  In  severe  cases  the  mucosa  for  a  distance  of  10 
to  12  cm.  above  the  anus  may  be  almost  completely  destroyed,  only  a 
few  shreds  being  left.  In  recent  cases  the  ulcerated  surface  exudes 
pus.  Later,  extensive  fibrous  proliferation  takes  place,  which  eventually 
leads  to  marked  stricture  of  the  rectum,  with  all  its  attendant  disorders 
of  obstruction,  dilatation,  hypertrophy,  and  ulceration.  Gummas  may 
also  develop  in  the  perirectal  cellular  tissue  and  lead  to  the  formation 
of  external  or  internal  fistule.  About  half  the  cases  of  stricture  of  the 
rectum  are  said  to  be  of  syphilitic  origin.  The  condition  is  twice  as 
common  in  women  as  in  men. 

Actinomycosis.1 — Actinomycosis  of  the  intestines  in  about  60  per 
cent,  of  cases  is  to  be  found  in  the  neighborhood  of  the  appendix  and 
cecum.  In  many  cases  of  abdominal  actinomycosis  with  external 
sinuses  and  peritoneal  adhesions  it  is  impossible  to  determine  the  exact 
starting  point  of  the  process.  Hinglais2  has  collected  120  cases  of  actino- 
mycosis of  the  appendix  and  cecal  region.  In  such  cases  the  affection 
begins  with  symptoms  of  appendicitis,  but  subsequently  large  rectocecal 
abscesses  or  external  fistulse  have  developed.  Actinomycosis  of  the 
intestines  begins  by  the  formation  of  whitish  patches  in  the  mucosa 
with  proliferation  of  the  submucosa.  As  the  disease  progresses,  ulcera- 
tion takes  place,  with  not  infrequently  the  formation  of  external  fistulse, 
in  the  discharge  from  which  the  characteristic  "sulphur  grains"  or 
actinomyces  can  usually  be  demonstrated.3  In  other  cases,  instead  of 
suppuration  or  ulceration,  hard,  tumor-like  masses,  sometimes  peduncu- 
lated,  may  be  produced. 

The  infection  is  supposed  to  be  due  to  the  use  of  contaminated  water 
or  vegetables. 

Inflammation  of  Special  Regions. 

Duodenitis. — Inflammation  of  the  duodenum — duodenitis — is  nearly 
always  associated  with  gastritis.  In  this  affection,  infective  agents  may 
travel  up  the  common  bile  duct  and  the  pancreatic  duct.  In  this 
way  a  catarrhal  or  suppurative  cholangitis  or  sialodochitis  may  be  pro- 

1  Leith,  Edin.  Hosp.  Rep.,  2: 1894: 128.  2  ThSse  de  Lyon,  1897. 

3  Krasnobajew,  Arch.  f.  Kinderheilk.,  23:  1  to  3. 


444  THE  INTESTINES 

duced.  Duodenitis  not  infrequently  leads  to  acute  interstitial  hepatitis 
and  occasionally  to  acute  suppurative  pancreatitis  (q.  v.).  Catarrh  of 
the  ducts  in  question  predisposes  to  the  formation  of  calculi  also.  Catar- 
rhal  jaundice  is  due  to  a  plug  of  mucus  in  the  ampulla  of  Vater  and 
probably  is  attributable  always  to  a  preexisting  gastroduodenitis. 

Ileitis. — Ileitis  is  practically  the  same  thing  as  the  enteritis  already 
described,  the  inflammation  being  commonly  localized  to  this  portion 
of  the  intestinal  tract. 

Colitis. — Colitis,  or  inflammation  of  the  colon,  is  a  comparatively 
frequent  condition,  being  a  notable  feature  in  cases  of  dysentery.  The 
colon  may  be  alone  involved  or  in  association  with  other  portions 
of  the  bowel.  Ileocolitis  is  the  ordinary  lesion  of  the  so-called  cholera 
infantum.  Many  cases  of  colitis  are  associated  with  the  infections 
and  various  forms  of  intoxication.  Colitis  is  not  uncommon  in  advanced 
Bright's  disease.  Anatomically,  we  may  recognize  catarrhal,  follicular, 
membranous,  hemorrhagic,  mucous,  ulcerative,  and  gangrenous  forms. 
The  reason  why  colitis  is  so  common  is  perhaps  not  far  to  seek,  when  we 
consider  the  opportunities  afforded  for  traumatism  and  infection.  The 
feces  tend  to  accumulate  in  the  large  bowel,  they  are  harder  there  than 
elsewhere,  and  their  progress  is  slower,  and  the  antibacterial  action  of 
the  digestive  ferments  is  much  lessened.  A  frequent  cause,  therefore, 
of  colitis  is  the  presence  of  dense,  scybalous  masses,  the  accumulation  of 
which  leads  to  dilatation  of  the  intestine,  with  possibly  the  formation  of 
diverticula.  Hard,  fecal  masses  are  retained  in  the  little  pockets  thus 
formed,  and  lead  to  ulceration  through  pressure  necrosis. 

Typhlitis. — Typhlitis  is  inflammation  of  the  cecum.  When  the  cellular 
tissue  about  the  cecum  is  involved  as  well,  the  condition  is  termed 
perityphlitis.  When  the  inflammation  is  extraperitoneal  and  behind  the 
cecum,  it  is  spoken  of  as  paratyphlitis.  Typhlitis  may  occasionally 
be  due  to  the  presence  of  hardened  feces  in  the  cecum,  but  apart  from 
this,  the  three  conditions  are  nearly  always  the  concomitants  of  inflam- 
mation of  the  appendix  vermiformis.  We  have  met  with  a  membranous 
typhlitis  in  the  case  of  a  cerebral  lesion,  however,  where  the  patient  had 
been  unconscious  for  some  days  before  death.  It  is  also  met  with  in 
mercurial  poisoning  and  uremia. 

Appendicitis. — Not  many  years  ago  the  terms  typhlitis,  perityph- 
litis, paratyphlitis,  and  the  still  vaguer  appellation,  "inflammation  of  the 
bowels,"  were  employed  to  designate  almost  all  affections  of  an  inflam- 
matory character  occurring  in  the  right  iliac  fossa.  We  owe  to  Fitz's 
classical  monograph  the  recognition  of  the  fact  that  the  vast  majority 
of  these  cases  have  their  origin  in  a  diseased  appendix  vermiformis, 
from  which  naturally  followed  the  adoption  of  more  rational  measures 
of  treatment. 

Inflammation  of  the  appendix — appendicitis — is  much  more  common 
in  males  than  in  females.  Most  cases  come  to  operation  between  the 
ages  of  twenty  and  thirty.  Where  previous  attacks  have  been  recorded 
in  the  histories  they  have  occurred  in  the  second  decade  of  life. 

We  shall  perhaps  be  better  prepared  to  understand  the  etiology  of 


APPENDICITIS  445 

inflammation  of  the  appendix  if  we  consider  the  anatomical  structure 
and  the  situation  of  the  organ  concerned. 

The  appendix  is  situated  at  the  head  of  the  cecum;  its  cavity  is 
continuous  with  that  of  the  large  bowel,  being  divided  from  it  by 
a  more  or  less  inconstant  fold  of  mucous  membrane,  the  valve  of 
Gerlach.  It  is  long  and  narrow  and  apt  to  be  somewhat  curved 
upon  itself.  The  appendix  appears  to  be  the  relic  of  a  large  cecal 
pouch,  such  as  is  still  to  be  found  in  the  ruminants  and  others  of 
the  lower  animals,  and  considerable  evidence  has  been  adduced  to  show 
that  it  is  gradually  involuting.  Ribbert,  in  a  study  of  400  appendices 
removed  at  post  mortems,  found  retrograde  and  atrophic  changes  in 
about  25  per  cent,  in  the  absence  of  any  indications  of  previous  in- 
flammatory change.  A.  O.  J.  Kelly1  has  found  more  or  less  obliteration 
of  the  lumen  in  about  one-quarter  of  the  cases.  Being  an  organ,  there- 
fore, that  is  disappearing  one  need  not  be  surprised  that  the  appendix  is 
a  common  seat  of  disease.  In  view  of  its  situation,  moreover,  it  is  apt 
to  be  a  depository  for  foreign  bodies  or  inflammatory  exudates,  which, 
owing  to  its  dependent  position  and  narrow  lumen,  are  almost  certain 
to  be  retained  within  it.  A  slight  inflammation  or  kinking  at  the  neck 
will  also  favor  the  retention  of  its  contents.  Thus,  the  appendix  prac- 
tically becomes  at  times  a  culture  tube  for  various  microorganisms, 
and  the  subject  of  traumatic  and  chemical  irritation.  Considerable 
stress  used  to  be  laid  upon  vascular  disturbances  as  a  cause  of  appendi- 
citis, but  the  importance  of  these  has  undoubtedly  been  overestimated. 
Thrombosis  and  embolism  might  conceivably  bring  about  anemic  necro- 
sis of  the  organ,  which  would  thereupon  become  an  easy  prey  to  patho- 
genic microorganisms,  but  this  appears  to  be  a  very  rare  occurrence. 
Breuer  has  shown  that  the  artery  of  the  appendix  is  not  an  end-artery, 
and  that,  contrary  to  the  usual  opinion,  there  is  a  fairly  effective  anasto- 
mosis in  the  various  coats  of  the  organ.  Again,  the  endarteritis  and 
periarteritis  held  by  some  to  be  a  chief  cause  in  bringing  about  necrosis 
have  not  been  found  constantly  or  even  frequently  present.  In  any  case, 
to  bring  about  the  condition  in  question,  obstruction  of  the  vessels  would 
have  to  be  somewhat  widespread.  The  vascular  changes  are  more 
reasonably  to  be  interpreted  as  the  result  rather  than  the  cause  of  the 
inflammation.  Possibly,  however,  kinks  or  other  constrictions  of  the 
appendix  at  its  orifice,  by  interfering  with  the  free  outflow  of  blood, 
may  in  some  few  cases  play  a  leading  role. 

At  one  time  it  was  almost  universally  held  that  foreign  bodies  or 
fecal  concretions  within  the  appendix  were  the  important  factor.  Apart 
from  fecal  concretions,  foreign  bodies  are  quite  rare  in  the  appendix, 
and  when  present  are  to  be  classed  as  accidental  occurrences  rather 
than  causative  agents.  James  Bell,2  in  between  900  and  1000  cases  of 
appendicitis  operated  upon,  found  foreign  bodies  within  the  appendix 
in  only  7,  and  thinks  that  this  is  probably  more  than  the  usual  pro- 
portion. Foreign  bodies  have  also  been  met  with  in  appendices  that 

1  Phila.  Med.  Jour.,  4 : 1899 :  928,  983 : 1032.          2  Mont.  Med.  Jour.,  31 :  1902 :  765, 


446  THE  INTESTINES 

presented  no  obvious  appearances  of  disease.  Among  the  substances 
found  may  be  mentioned  apple-pips,  grape-seeds,  hair,  bits  of  bone,  pins, 
bits  of  glass,  gallstones,  wood-fiber,  segments  of  tenia,  thread  worms, 
and  round  worms.  In  the  Pathological  Museum  of  McGill  University 
is  an  appendix  containing  a  large  number  of  shot,  the  owner  of  which 
never  suffered  from  appendicitis.  Sharp  substances,  such  as  pins 
or  glass,  might,  however,  be  expected  to  give  trouble.  Fecal  concre- 
tions consist  of  fecal  material,  desquamated  cells,  and  leukocytes,  inspis- 
sated in  a  mucoid  matrix,  and  sometimes  infiltrated  with  calcium  salts. 
They  are  found  in  normal  appendices,  and  in  themselves  appear  to  be 
comparatively  unimportant.  It  is  quite  likely,  however,  in  appendices 
that  are  inflamed  and  swollen,  concretions  may  bring  about  necrosis 
through  pressure  and,  as  a  matter  or  fact  they  are  more  numerous  in 
appendicitis  of  the  ulcerative  and  gangrenous  type.  E.  W.  Archibald,1 
in  his  analysis  of  89  cases,  found  concretions  in  only  3  out  of  38  non- 
perforative  cases,  while  in  41  perforative  cases  concretions  were  found 
in  22.  It  should  be  pointed  out  that  concretions  are  an  expression  of  a 
previous  or  co-existent  inflammatory  process  rather  than  the  exciting 
cause  of  inflammation.  As  in  the  case  of  biliary  or  urinary  calculi, 
there  must  have  been  a  preexisting  catarrh  of  the  mucosa  producing 
an  excess  of  mucus  with  exudation  and  desquamation  of  cells  to  pro- 
vide a  nidus  in  which  the  salts  may  be  deposited.  Once  formed,  of 
course,  the  concretion  might  be  expected  to  perpetuate  or  aggravate 
the  condition.  As  a  rule,  however,  it  is  only  the  larger  concretions 
which,  by  pressure  upon  the  mucosa  and  obstructing  the  free  discharge 
of  retained  secretions,  lead  to  trouble. 

Another  point  of  etiological  moment  is  the  fact  that  the  lymphoid 
elements  of  the  appendix  undergo  involution  along  with  the  other 
structures.  The  appendix,  at  first,  is  much  more  rich  in  lymphoid  ele- 
ments than  the  rest  of  the  intestine,  the  cells  in  question  being  diffused 
throughout  the  mucosa  or  aggregated  into  follicles.  These  elements 
are  probably  concerned  in  the  manufacture  of  substances  that  immunize 
the  body  against  bacterial  infection  from  the  lumen  of  the  bowels. 
Ribbert  and  Kelynack  have  pointed  out  that  this  lymphoid  tissue  is 
most  marked  in  childhood  and  atrophies  after  the  thirtieth  year  or  in 
exceptional  cases  as  early  as  the  twentieth.  The  fact,  therefore,  that 
the  defensive  powers  of  the  appendix  against  infection  under  these 
circumstances  are  beginning  to  wane  will  explain  the  greater  prevalence 
of  appendicitis  after  early  adult  life. 

The  most  important  single  cause  of  appendicitis  is  infection,  the 
activity  of  the  microorganisms  being  aided  by  the  anatomical  peculiarities 
just  detailed.  Aschoff,  the  most  recent  investigator  of  the  subject,  holds 
that  the  disease  begins  as  an  enterogenous  infection,  beginning  :it  the 
bottom  of  the  crypts.  To  produce  this  result  only  the  slightest  abrasion 
of  the  epithelium  is  necessary.  Of  the  bacteria  at  fault,  the  most  im- 
portant are  the  B.  coli  communior  (in  Montreal),  Staphylococcus  pyogenes 

1  Mont.  Med.  Jour.,  29:1900:81. 


ACUTE  DIFFUSE  APPEXDICi:  447 

albus  and  aureus,  Streptococcus  pyogenes.  Dipk>ooccus  pneumonia?, 
and  B.  pyocyaneus.  In  Kelly's  400  cases  the  B.  coli  was  present  in 
92  per  cent. 

Tavd  and  Lanz.  Barnacd,  and  Welch  have  pointed  out  the  frequency 
of  mixed  infection.  Probably  the  vast  majority  of  appendicitis  cases  are 
due  to  mixed  infection,  and  the  reason  that  this  is  not  generally  realized  is 
to  be  looked  for  in  faulty  bacteriological  technique.  The  B.'coli  readily 
overgrows  and  destroys  less  vigorous  germs.  Therefore,  plate  cultures 
should  be  made  at  the  time  of  operation  in  order  that  the  various  forms 
may  be  properly  isolated.  Cultures  should  also  be  made  under  anaero- 
bic conditions.  "  It  is  likely,  also,  that  during  life  the  same  destruction 
of  the  weaker  organisms  by  the  B.  coli  takes  place,  so  that  die  culture 
methods  may  reveal  only  a  single  germ  when  the  disease  has  really  been 
brought  about  by  several.  It  may  be  shown  that  microscopic  sections  of 
the  appendix,  appropriately  stained  to  show  bacteria,  often  reveal  the 
presence  of  microorganisms  within  the  lumen  which  have  failed  to  de- 
velop in  the  cultures  taken.  Where  mixed  infection  can  be  recognized, 
the  B.  coli  and  the  Staphylococcus  are  usually  found  together.  In  a 
few  cases,  the  Staphylococcus,  Pneumococcus,  or  B.  pyocyaneus  have 
been  found  alone. 

In  regard  to  the  extent  of  the  inflammatory  process  itself,  the  whole 
appendix  may  be  involved  or  only  a  portion  of  it.  usually  the  distal 
pan.  It  is  difficult  to  give  an  accurate  classification  of  the  forms, 
inasmuch  as  the  types  met  with  imperceptibly  pass  one  into  the  other. 
The  following  may  be  suggested  as  a  convenient  grouping: 


A:  *  . 


Ouooic Dii-e~ 

-  { 


KJ* 


(JIrxmf*if{ 


CaUrrtal  Appendicitis.— Catarrhal  appendicitis  may  be  acute  or 
chronic.  In  the  acute  form  the  appendix  is  swollen,  the  external  venules 
are  congested,  and  the  organ  is  often  kinked  or  twisted.  The  mucous 
membrane  is  swollen,  succulent,  congested,  with  possibly  minute  hemor- 
rhages, and  there  may  be  even  slight  roughening  of  its  surface.  The 
cavity  contains  thin  mucus  with  a  few  leukocytes,  and  there  may  be  con- 
cretions. Occasionally,  there  are  a  few  old  adhesions  about  the  appendix. 
On  histological  examination,  die  mucosa  is  congested,  oedematoos,  and 
the  epithelium  is  in  a  state  of  catarrh.  The  lymphotd  follicles  are 
proliferating  and  the  submucosa  is  also  oedematous. 

Acute  Ditoe  Appe«tkirii  —This  form  is  more  severe.  The  a] 
is  swollen  in  all  its  thickness  and  may  be  covered  with  fibrinous 
externally.  All  the  features  of  the  acute  catarrhal  form  are  present,  hot 


448 


THE  INTESTINES 


FIG.  109 


in  addition  there  is  a  diffuse  infiltration  of  leukocytes  in  all  the  coats, 
which,  therefore  are  greatly  thickened  and  cedematous.  The  lymphoid 
elements  are  also  actively  proliferating.  Here  and  there  there  may  be 
small  superficial  erosions  of  the  mucosa.  The  cavity  may  contain 
mucopus.  The  meso-appendix  is  often  involved  as  well. 

Acute  Ulcerating  Appendicitis. — In  the  acute  ulcerative  type  there  is 
at  some  point  or  other  an  area  of  necrosis,  usually  corresponding  to  the 
situation  of  a  fecal  concretion.  This  ulceration  may  be  of  varying  depth, 
and  not  infrequently  perforates.  The  appendix  is  often  discolored  at  the 
point  of  necrosis  and  is  bathed  externally  in  pus. 

Gangrenous  Appendicitis. — Gangrenous  appendicitis  is  an  extreme 
and  fulminating  variety,  in  which  the  whole  appendix  or  some  part  of 

it  rapidly  necroses  and  is  converted 
into  a  blackish,  sloughy  mass.  This 
form  may  be  primary  or  may  be  en- 
grafted upon  other  types  of  appen- 
dicitis. It  is  due  to  a  particularly 
virulent  form  of  infection  or  to  vascu- 
lar obstruction. 

Typhoidal  ulceration  of  the  appendix 
resembles  typhoidal  ulceration  else- 
where. It  may  lead  to  perforation 
and  peritonitis. 

Chronic  Appendicitis. — Chronic  ap- 
pendicitis may  be  insidious  in  its  onset 
or  result  from  an  acute  or  subacute 
attack.  In  not  a  few  of  the  cases  we 
find  a  succession  of  attacks  of  more 
or  less  acute  inflammation,  in  any  of 
which  ulcerative  and  gangrenous  pro- 
cesses may  supervene,  placing  the 
patient's  life  in  jeopardy.  Both  the 
chronic  and  the  relapsing  cases  lead 
to  proliferation  of  connective  tissue, 
whereby  the  appendix  becomes  indu- 
rated and  the  lumen  in  some  cases 
entirely  obliterated,  so  that  the  whole 
organ  is  converted  into  a  fibrous  cord 
(sclerosing  appendicitis).  Here  and 
there  collections  of  round  cells  may  be 
seen  in  the  various  coats,  relics  of  a 
more  active  stage  of'  inflammation. 
Obliteration  of  the  proximal  portion 

Acute     appendicitis,      with     extensive  j      d   t      ^     retention  of   SCCretion 

round-celled  infiltration  of  all  of  the  coats  rj  .     n 

of  the  appendix.    (Stengel.)  and  inflammatory  products,  so  that  the 

remainder  of   the   appendix  becomes 

dilated  into  the  form  of  a  cyst  of  cylindrical  or  globular  shape.  The 
contents  of  the  cyst  are  either  serous,  mucous,  or  purulent,  and  may 


CHRONIC  APPENDICITIS 


449 


become  inspissated.  In  all  chronic  and  relapsing  cases  fibrous  adhesions 
form  in  the  neighborhood  of  the  appendix.  By  obliteration  of  the  lumen 
spontaneous  cure  may  result.  This,  however,  is  not  to  be  expected. 

The  danger  from  appendicitis  is  largely  dependent  on  perforation, 
which  occurs  sooner  or  later  in  a  large  proportion  of  acute  cases.  If 
the  inflammation  be  a  fulminating  one  and  adhesions  have  not  formed, 
a  septic  peritonitis  supervenes,  which,  in  the  vast  majority  of  cases  is 
fatal.  A  general  peritonitis  due  to  the  B.  coli,  or  in  which  the  cultures 
are  sterile,  appears  to  be  less  virulent  and  some  few  cases  get  well  after 
appropriate  surgical  treatment.  Where  fibrinous  adhesions  have  formed, 

FIG.  110 


Early  ulcerative  appendicitis.  The  section  shows  slight  erosion  of  the  mucosa  with  catarrh  of 
the  crypts  and  hyperplasia  of  the  lymphoid  elements  of  the  Peyer's  patches.  Zeiss  obj.  A, 
without  ocular.  (From  the  Pathological  Laboratory  of  McGill  University.) 

perforation  of  the  appendix  leads  to  the  formation  of  a  localized  abscess 
in  which  concretions  or  portions  of  the  appendix  may  be  found.  The 
patient  will  frequently  recover  in  such  cases  if  the  abscess  be  promptly 
evacuated  and  drained.  More  favorable  still  are  those  cases  in  which 
dense,  fibrous  adhesions  wall  off  the  diseased  appendix  from  the  general 
abdominal  cavity.  The  appendix  may  lie  behind  the  cecum,  pointing 
upward,  and  lead  to  the  formation  of  an  abscess  in  the  region  of  the 
liver.  In  other  cases  the  organ  lies  in  a  little  pocket  of  peritoneum  or 
even  entirely  behind  the  peritoneum.  Such  cases  are  relatively  favor- 
able. Periappendicular  abscesses  may  burst  into  the  bowel,  giving 
rise  to  fecal  fistulse,  or  may  evacuate  themselves  externally.  Septic 
29 


450 


THE  INTESTINES 


thrombosis  of  the  mesenteric  and  omental  veins  is  a  common  accompani- 
ment, as  are  also  septic  portal  pylephlebitis  and  abscesses  in  the  liver. 
Empyema  of  the  right  pleural  cavity  is  occasionally  met  with. 

Tuberculosis. — -Tuberculosis  of  the  appendix  is  found  in  association 
with  tuberculosis  of  other  parts  of  the  intestinal  tract,  and  does  not 
differ  in  any  way  from  it.  Here  also  the  hyperplastic  form  has  been 
observed. 

FIG.  Ill 


Gangrene  of  the  appendix  vermiformis  in  acute  appendicitis;  concretion.     (Pathological 
Museum,  McGill  University.) 

Proctitis. — Proctitis,  or  inflammation  of  the  rectum,  is  commonly 
brought  about  by  traumatism,  such  as  may  be  caused  by  impacted  feces, 
fruit-stones,  fish-bones,  or  foreign  bodies  introduced  into  the  rectum. 
Dilated  hemorrhoidal  veins  may  also  become  thrombosed  and  infected, 
thus  leading  to  inflammation.  Proctitis  is  also  caused  by  gonorrhoea, 
syphilis,  tuberculosis,  and  certain  mineral  substances  such  as  arsenic 
and  mercury.  Ulceration  is  a  common  feature,  but  is  of  chronic  type 
and  leads  to  thickening  of  the  wall,  polypoid  outgrowths,  and  more 
or  less  atresia.  The  ulcers  may  penetrate  deeply  and  give  rise  to  ab- 
scesses about  the  rectum  (periproctal  abscesses),  which  may  burst  into 


TUMORS  451 

the  bladder  or  vagina  (rectovesical  and  rectovaginal  fistulci),  or  make 
their  way  to  the  external  surface  (complete  or  external  fistulce).  Where 
the  abscess  communicates  only  with  the  lumen  of  the  bowel  and  has  no 
external  opening,  the  condition  is  termed  by  surgeons  blind  or  internal 
fistula. 

RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  intestines  is  not  uncommon.  It  may 
affect  the  mucosa  only  or  the  whole  thickness  of  the  bowel. 

Degeneration. — Fatty  degeneration  and  hyaline  degeneration  are 
occasionally  met  with  in  the  muscular  coat. 

Amyloid  Infiltration. — Amyloid  infiltration  is  not  uncommon  in 
advanced  cases  of  amyloid  disease.  It  affects  the  walls  of  the  smaller 
bloodvessels,  principally  of  the  mucosa  and  submucosa. 

Enterolithiasis. — The  curious  condition,  known  as  enterolithiasis,  in- 
cludes the  formation  of  large  concretions  within  the  cavity  of  the  bowel, 
and  of  fine  granular  particles,  or  intestinal  sand. 

Intestinal  sand  is  composed  of  a  large  proportion  of  organic  matter 
with  about  33  per  cent,  of  calcium  salts.  Myer  and  Cook1  have  shown 
that,  in  some  cases  at  least,  certain  articles  of  diet,  notably  bananas, 
may  be  responsible  for  the  condition.  A  vegetable  resin  together  with 
tannic  acid  is  found  in  this  fruit  and  under  the  influence  of  digestion 
an  insoluble  tannate  is  produced  which  appears  in  the  feces. 

Concretions  or  enteroliths  are  usually  found  in  the  appendix  or  diver- 
ticula  of  the  intestine  and  consist  of  inspissated  feces  and  desquamated 
cells,  which  have  formed  a  nidus  for  the  deposition  of  calcareous  salts. 

Necrosis,  ulceration,  and  gangrene  are  not  uncommon  in  the  intestine, 
and  result  from  inflammation,  pressure,  and  circulatory  disturbances. 

PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Hypertrophy  of  the  intestinal  wall  occurs  as  the 
result  of  chronic  obstruction. 

Tumors. — Benign  tumors  are  comparatively  rare  and  of  no  great 
importance.  The  forms  met  with  are  the  adenoma,  fibroma,  lipoma, 
myoma,  osteoma,  hemangioma,  lymphangioma,  and  chylangioma.  The 
connective-tissue  tumors  usually  spring  from  the  submucosa  and  pro- 
ject outward  into  the  peritoneal  cavity.  Polypoid  or  pedunculated 
masses  are  not  infrequently  produced,  which  may  break  loose,  forming 
free  bodies  in  the  abdomen,  or  may  be  passed  per  anum.  Occasionally, 
they  induce  intussusception  and  intestinal  obstruction. 

The  enterocystoma  is  a  curious  cystic  growth  resulting  from  a  develop- 
mental anomaly  in  the  form  of  partial  persistence  of  the  omphalomesen- 
teric  duct. 

A  rare  condition  is  the  presence  of  an  accessory  pancreas  lying  concealed 
in  the  substance  of  the  intestinal  wall,  or  forming  a  small  nodular  mass 

1  Amer.  Jour.  Med.  Sci.,  137: 1909: 383. 


452 


THE  INTESTINES 


FIG.  112 


projecting  on  the  serous  aspect.  Such  tumors  are  the  result  of  devel- 
opmental errors,  and  are  usually  found  in  the  upper  alimentary  tract, 
duodenum  or  jejunum.  When  in  the  jejunum  they  may  be  situated 
at  the  tip  of  a  diverticulum.1 

Polypoid  outgrowths,  often  pedunculated,  and  having  the  general 
structure  of  an  adenoma,  are  not  uncommon  in  the  intestine,  especially 
in  the  duodenum  and  in  the  large  bowel  near  the  ileocecal  valve  or  in 
the  rectum.  They  are  found  especially  at  the  margins  of  chronic  ulcers 
and  in  connection  with  long-standing  enteritis.  It  is  questionable 

whether  they  are  ever  true 
tumors,  and  they  should  certainly 
be  classed  with  the  inflammatory 
hyperplasias. 

The  malignant  growths  are  sar- 
coma, lymphosarcoma,  endotheli- 
oma,  and  carcinoma.  Sarcomas 
are  round,  spindle-celled,  alveo- 
lar, and  melanotic.  The  round- 
celled  form  is  the  commonest  of 
the  primary  sarcomas,  but  is  still 
rare.  It  usually  occurs  between 
the  ages  of  forty  and  fifty.  E.  A. 
Robertson2  has  recorded  a  case 
in  a  child  of  four.  The  growth 
was  situated  in  the  neighborhood 
of  the  ileocecal  valve.  Melanotic 
sarcomas  are  single  or  multiple, 
and  are  found  studding  the  serous 
surface.  They  are  secondary  to 
melanotic  sarcomas  of  the  skin  or 
choroid  of  the  eye. 

Lymphosarcoma    may    be    pri- 
mary or  secondary.      It  usually 
starts  in  the  lymph-follicles,  but 
may  begin  independently  of  them. 
Sooner  or  later   the  new-growth 
extends  beyond  the  limits  of  the 
follicles  and  invades  the  mucosa 
and  other  coats  of  the  intestine. 
The  bowel  may  also  be  invaded 
secondarily     from     sarcomatous 
mesenteric  glands. 
Carcinoma. — By  far  the  commonest  new-growth  of  the  intestine  is 
the  carcinoma.     In  the  large  majority  of  cases  it  is  found  in  the  large 
intestine,  and  usually  in  the  rectum.     It  is  occasionally  met  with  about 


Chronic  colitis  with  polypoid  outgrowths. 
(From  the  Pathological  Museum  of  McGill 
University.) 


1  Nicholls,  A  Case  of  Accessory  Pancreas,  Mont.  Med.  Jour.,  29 : 1900 : 903. 

2  Mont.  Med.  Jour.,  27 : 1898 :  31. 


PLATE  V 


Adenoma  (Papillomatous)  of  the  Colon  with  Early 
Carcinomatous  Change.     (Nicholls.) 


CARCINOMA 


453 


the  ileocecal  valve,  the  iliac,  splenic,  and  hepatic  flexures,  and  the 
cecum.  Of  late  years  a  considerable  number  of  cases  of  primary 
carcinoma  of  the  appendix  have  been  recorded.1  They  are  curious  in 
that  they  do  not  tend  to  form  distant  metastases.  Primary,  round- 
celled  sarcoma  of  this  structure  has  also  been  described.2  When  in  the 
small  intestine,  a  rare  occurrence  comparatively,  the  site  of  election  is 
in  the  neighborhood  of  the  bile  papilla.  A  note-  worthy  fact  is  the 
relative  frequency  with  which  intestinal  carcinomas  are  met  with  in 
young  people.  This  perhaps  is  to  be  explained  in  connection  with  the 
common  occurrence  of  mucous  polyps  of  the  intestines  in  children. 

FIG.  113 


Carcinoma  of  the  rectum.     Zeiss  obj.  A,  without  ocular.     New  formation  of  tubules  is  seen 
well  below  the  muscularis  mucosse.     (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

The  carcinomas  of  the  intestine  are  of  the  medullary,  scirrhous,  or 
colloid  type.  Histologically,  with  the  exception  of  the  squamous-celled 
form  starting  at  the  anus  and  that  originating  in  the  Brunner's  glands, 
they  are  cylindrical-celled  and  of  glandular  appearance. 

The  new-growth  forms  a  solitary,  localized,  sharply  defined,  fungating 
mass,  or,  again,  may  involve  a  considerable  area  of  the  bowel.  The 
softer  cancers  have  little  tendency  to  obstruct  the  lumen.  In  some  cases, 
the  infiltration  is  more  restricted,  but  forms  a  ring-like  mass  encircling 
the  bowel.  The  muscular  wall  is  infiltrated  and  hardened,  so  that  the 
intestine  is  converted  into  a  stiff,  uncollapsible  tube.  The  surface 

1  Elting,  Annals  of  Surgery,  37 : 1903 : 549 ;  also,  Harte,  Annals  of  Surgery,  June : 
1908,  and  Rolleston,  Lancet,  Lond.,  2:1900: 11. 

2  Paterson,  Practitioner,  70:  1903:  515. 


454  THE  INTESTINES 

generally  ulcerates,  producing  a  shallow  erosion,  the  edges  and  base  of 
which  are  densely  fibrous,  leading  to  a  cicatricial  constriction  of  the 
bowel.  The  affected  part  may  become  adherent  to  neighboring  structures, 
or  the  ulcer  may  perforate,  leading  to  peritonitis.  Metastases  in  the 
liver  (through  the  portal  system),  lymph-nodes,  and  peritoneum  are 
common.  Secondary  carcinomas  are  as  rare  as  the  primary  are  common. 
They  are  metastatic  or  arise  by  implantation  of  carcinomas  of  the 
pancreas,  stomach,  uterus,  and  vagina. 


CHAPTER    XXI. 

THE  LIVER. 
ANOMALIES. 

Congenital  Anomalies. — The  liver  may  be  completely  absent,  or  may 
deviate  considerably  from  the  normal  in  form  and  size.  The  organ 
is  occasionally  thin  and  flat,  or  the  shape  of  a  short  truncated  pyramid. 
A  somewhat  common  anomaly  is  for  the  left  lobe  to  be  prolonged  back- 
ward and  downward  in  the  form  of  a  lingula.  The  lobes  may  be  increased 
in  number  and  aberrant  lobes  attached  by  a  narrow  pedicle  have  been 
found  in  hernial  sacs  at  the  umbilicus,  where  they  have  been  mistaken 
for  tumors.  Accessory  livers  are  occasionally  met  with,  situated  in  the 
suspensory  ligament.  The  liver  may  be  reversed  and  situated  on  the 
left  side  of  the  abdomen  in  the  condition  of  transposition  of  the  viscera. 
It  may  also  be  dislocated  in  various  forms  of  congenital  hernias. 

The  gall-bladder  may  be  absent  or  buried  in  the  liver  substance,  or, 
again,  directed  backward.  The  biliary  ducts  may  be  absent,  abnormally 
dilated,  or  occluded  at  some  point.  The  ductus  communis  choledo- 
chus  may  be  reduplicated  and  the  single  or  double  duct  may  discharge 
into  the  stomach  or  at  some  unusual  situation  in  the  bowel. 

Acquired  Abnormalities  of  Shape  and  Position. — Of  acquired 
deformities  the  most  common  is  the  so-called  "lacing-lobe."  The  press- 
ure of  a  tight  corset  forces  in  the  lower  portion  of  the  thoracic  wall,  so 
that  a  transverse  fissure  is  produced,  which  divides  the  right  lobe  into 
an  upper  and  a  lower  portion.  The  Glisson's  capsule  over  the  fissure 
is  thickened  and  of  a  pearly  white  color,  while  the  subjacent  acini  are 
atrophic.  A  portion  of  the  liver  may  be  almost  if  not  quite  separated 
from  the  main  mass,  being  connected  merely  by  a  fibrous  band.  The 
lacing-lobe  is  generally  seen  in  persons  having  a  short  thorax,  tight  lacing 
in  those  with  long  waists  being  more  likely  to  produce  a  movable  kidney. 

The  so-called  Liebermeister  grooves  are  common  also,  occurring  in 
our  experience  in  7.26  per  cent,  of  all  autopsies.  These  consist  in  a 
variable  number  of  parallel  grooves  on  the  outer  convexity  of  the  right 
lobe,  which  are  directed  forward.  They  do  not  correspond  in  direction 
with  the  ribs.  Several  explanations  have  been  offered  for  their  occur- 
rence. Liebermeister  thought  they  were  due  to  pressure  from  difficult 
respiratory  movements,  while  Zahn  attributed  them  to  the  action  of  a 
hypertrophied  diaphragm.  In  some  cases  the  depressions  can  be 
shown  to  correspond  with  thickened  bands  of  muscle.  Possibly  a  few 
instances  are  congenital. 

Owing  to  a  laxness  of  the  suspensory  ligament  the  liver  may  be  more 
or  less  remote  from  its  natural  position  (hepatoptosis;  mobile  or  floating 


/jr,f;  y •///•;  LI  v Kit. 

liver;.      The    or^ui    may    be    anf.evert.ed,    it  ,    ant'-rior    border    pa 
downward     and     forwa.nl,    the    posterior     border    remaining    praet  jc;i|]  •. 
unafl'erted.      The    displacement,    in;;  •    be   oblique,    the    left.   lobe    rje- 

/•  n'lin;',  or,  a.f/a.in,  the  whole  or</an  may  be  affecte,].  The  condition 
of  dislocated  liver  is  commonly  associated  with  gastropto  i  or  < -nt< -ro- 
ptosis. 

The  liver  may  be  pu  ,hrd  downward  by  the  pressure  of  pleura!  <•/. u- 
dates  or  effusions,  intrathoracic  growths,  or  emphysema.     It  may  be 
forced  upward  in  cases  of  ascites,  intra-abdominal  cysts,  or  tumor 
The  traction  of  intra-abdominal  adhesions  also  may  pull  the  organ  out 
of  its  natural  position. 


CIRCULATORY  DISTURBANCES.       , 

Anemia. — Anemia  of  the  liver  Is  either  a  manifest at  ion  of  a 
ized  or  systemic  anemia,  or  is  due  to  some  local  disturbance,  such  as 
pressure  upon  the  organ  or  swelling  of  the  parenchyma. 

Hyperemia.  -Active  Hyperemia. — Active  hyperemia  is  met  with  as 

;.    |,|j  .    io!ofrjr-;i|   condition   jri'lf-r  }i.  Jfiejil,  ;i.n«J,   p;i .t.liolo^if-;jHy,   in    tli*    r-;j.rly 

stages  of  acute  inflammations  of  the  liver,  and  in  all  cases  of  congestion 

of  flie  M;r,fro-int<--,tin;il    v;i  ,rul;ir      .    K-ni. 

Passive  Hyperemia. — ^Passive  hyperemia  is  brought  about  by  any  cuu  e 
that  raises  the  blood  pressure  within  the  hepatic  vein  and  inferior  vena 
cava.  Among  the  important  conditions  that  should  be  mention ed  in 
this  connection  are  stenosis  or  insufficiency  of  the  mitral  or  tricuspd 
valves,  cardiac  weakness,  emphysema  of  the  lungs,  indurative  pneumonia, 
right-sided  pleural  effusions,  aneurisms,  tumors,  or  enlarged  gland 
pressing  upon  the  inferior  vena  cava. 

In  the  early  stages  the  liver  is  enlarged,  often  attaining  a  considerable 
si/e,  soft,  jind  full  of  blood,  which  drips  out,  of  it.  on  section,  hi  eolor 
it  is  dark,  purplish-red.  After  the  blood  has  been  drained  out  the  or^a  n 
will  be  found  to  be  somewhat  shrunken  (red  atrophy).  Later,  the  liver 
is  more  or  !<• ,  ;  diminished  in  si/e,  firm,  with  a  finely  gninubir  surfnee. 
On  section,  the  appearance  is  like  that  of  a  nutmeg,  whence  the  term 
"nutmeg-liver."  This  is  due  to  the  fact  that  the  centnd  portion  of  the 
lobule  is  congested  and  somewhat  depressed  below  the  general  surface, 
while  the  periphery  is  pale  yellow  or  yello-.v  i  li-hrown,  ;md  swollen, 
owing  to  the  presence  of  fat  within  the  secreting  cells.  Here  ;md 
there  redder  patches  are  to  be  seen  which  are  regenerated  lobules.  In 
the  most  advanced  stage  the  liver  is  small  and  hard,  owing  to  the  pro- 
duction of  fibrous  tissue  (cyanotic  induration).  In  this  way  a  form  of 
cirrhosis  may  be  produced — the  "cirrhose  cardiarjue"  of  the  Freneh 
writers.  In  our  experience  this  affection  is  rare. 

On  histological  examination ,  the  main  changes  an-  at  fir., I.  to  be  found 
in  the  central  portion  of  the  lohule..,.  The  centrilohular  veins  with  their 
capillaries  arc;  disl.ended  with  blood,  and  the  eolumns  of  liver  eells  be- 
tween arc  compressed.  The  secref.ing  cells,  therefore,  heroine  atrophic, 


LEUKEMIA 


457 


I.-;.    Ill 


and  contain  yellowish-brown  granules  of  pigment,  together  with  minute 
globules  of  fat.  The  degeneration  is,  as  one  might  expect,  most  marked 
in  the  central  and  intermediate  zones 
of  the  lobules.  Here  and  there  groups 
of  cells  are  seen,  much  larger  than  the 
ordinary  parenchymatous  cells,  having 
also  large  nuclei,  which  take  an  in- 
tense stain.  These  indicate  an  attempt 
at  regeneration.  In  the  more  ad  va  need 
forms  practically  all  the  capillaries  of 
the  lobule  are  congested  and  the  great 
bulk  of  the  liver  cells  disappear,  being 
represented  only  by  fragmented  nuclei 
and  masses  of  pigment.  In  certain 
cases,  where  the  congestion  is  gradual 
in  its  onset,  concomitant  with  the 
degeneration  there  is  proliferation  of 
connective  tissue,  leading  to  indura- 
tion (replacement  fibrosis),  a  condition 
found  usually  about  the  radicles  of 
the  hepatic  vein,  but  to  some  extent 
also  in  the  periportal  districts.  The  portal  sheaths  may  present 
evidences  of  a  round-celled  infiltration. 

Leukemia. — In   leukemia  the  capillaries  everywhere  are  filled  with 
leukocytes,  and  particularly  large  accumulations  may  be  noted  in  the 

I  i'..  115 


Nutmeg  liver.  (From  the  Pathological 
Department  of  the  Royal  Victoria  Hos- 
pital.) 


Leukemia  of  the  liver.      The  capillaries  are  filled  with  leukocytes.     Zeus  obj.  A,  without 
ocular.     (From  the  collection  of  the  Pathological  Department,  McGill  University.) 


458  THE  LIVER 

portal  districts.  There  has  been  a  debate  whether  these  represent 
a  hyperplasia  of  lymphoid  tissue  normally  present  there  or  a  multiplica- 
tion of  leukocytes  that  have  migrated  there. 

(Edema. — (Edema  of  the  liver  is  met  with  occasionally  and  leads 
to  enlargement  of  the  organ.  The  condition  has  not  attracted  much 
attention,  but  is  more  common  than  has  usually  been  thought.  The 
liver  substance  is  more  succulent  than  usual,  and  on  section  has  a  pale, 
dull,  shiny  appearance.  Microscopically,  one  can  see  clear  spaces  be- 
tween the  capillaries  and  the  parenchymatous  cells  of  the  liver.  The 
condition  can  be  reproduced  experimentally  by  stimulation  of  the  liver, 
and  apparently  may  be  readily  brought  about.  Birch-Hirschfeld  attrib- 
uted icterus  neonatorum  to  compression  of  the  bile  ducts  by  cedematous 
connective  tissue. 

Hemorrhage. — Hemorrhage  into  the  substance  of  the  liver  may 
occur  from  traumatism,  the  hemorrhagic  diathesis,  infarction,  acute 
yellow  atrophy,  and  various  infections. 

Embolism  and  Thrombosis. — Embolism  or  thrombosis  of  the  hepatic 
artery  or  its  branches,  especially  when  associated  with  cardiac  weakness, 
leads  to  the  formation  of  an  infarct  (Chiari).  The  infarction  is  rarely 
typical,  for  the  reason  that  the  liver  is  well  supplied  with  blood  also  from 
the  portal  vein. 

Thrombosis  of  the  portal  vein  is  a  not  uncommon  condition.  In  the 
majority  of  instances  it  is  really  a  thrombophlebitis.  The  main  trunk  of 
the  vessel  or  any  of  its  branches  may  be  affected.  Infective  processes 
occurring  in  the  organs  or  structures  included  in  the  portal  system,  as, 
for  example,  the  spleen,  intestines,  stomach,  or  mesentery,  particularly 
when  septic  in  character,  may  lead  to  it.  It  is  especially  common  in 
connection  with  appendicitis.  Inflammations,  too,  about  the  bile  ducts, 
or  in  the  retroperitoneal  tissues  in  that  neighborhood,  may  extend  to  the 
portal  vein  and  induce  thrombosis.  If  septic,  the  thrombus  may  break 
down  and  lead  to  the  formation  of  an  abscess,  and  minute  particles  of 
infected  material  may  be  carried  as  emboli  into  the  liver.  Or,  again, 
if  non-infective,  the  thrombus  may  gradually  soften  and  disappear,  or 
may  organize.  In  one  case  which  we  observed,  following  typhoid  fever, 
the  main  trunk  of  the  portal  vein  was  completely  occluded,  and  here  and 
there  in  the  smaller  branches  strands  and  tags  of  fibrous  tissue  could 
be  detected,  the  remains  of  the  previously  existing  thrombus. 

Thrombosis  is  also  occasionally  observed  in  cases  of  -portal  cirrhosis, 
and  where  pressure  is  exerted  upon  the  portal  vein  by  tumors  extending 
from  the  biliary  passages,  pancreas,  stomach,  or  intestines. 

Where  the  obstruction  to  the  circulation  is  complete,  ascites  comes  on 
rapidly  and  may  be  intense.  Important  structural  changes  in  the  liver 
do  not  usually  occur  in  the  liver  itself,  since  it  is  supplied  with  blood 
from  the  hepatic  artery. 

Infarcts. — Infarcts  of  the  liver  of  the  same  type  as  those  occurring 
in  other  organs  are  rare.  They  may  be  produced  by  embolism,  throm- 
bosis, or  other  causes  leading  to  occlusion  of  the  portal  vein,  hepatic 
artery,  or  hepatic  vein.  The  condition  seems  to  be  more  common  after 


ACUTE  PARENCHYMATOUS  HEPATITIS  459 

occlusion  of  the  artery  than  of  the  vein,  and  in  this  case  is  of  the  anemic 
variety.  Hemorrhagic  infarction,  so-called,  is  believed  to  be  more  fre- 
quently due  to  portal  embolism,  though  it  may  also  result  from  obstruc- 
tion of  the  hepatic  artery,  The  condition  is  not  a  true  infarction,  however, 
inasmuch  as  the  liver  cells  still  retain  their  staining  properties.  More 
properly  it  is  an  intense  capillary  congestion.  When  the  obstruction 
involves  the  vessels  in  the  intermediate  portion  of  the  lobules,  formed  by 
the  union  of  the  capillaries  of  the  portal  vein  and  hepatic  artery,  it 
leads  to  the  production  of  one  variety  of  focal  necrosis.  Schmorl  and 
Prutz  have  described  this  condition  in  connection  with  eclampsia.  Such 
necrotic  areas  are  usually  demarcated  by  a  zone  of  reactive  inflammation. 
If  the  part  be  infected,  abscess  formation  will  follow,  but  if  not,  and  the 
patient  survive,  the  degenerated  cells  are  absorbed  and  replaced  by  con- 
nective tissue. 

INFLAMMATIONS. 

Hepatitis. — The  subject  of  hepatitis,  or  inflammation  of  the  liver, 
is  one  of  the  most  difficult  in  the  domain  of  special  pathology.  This, 
in  part,  arises  from  the  fact  that  we  are  still  to  some  extent  ignorant  of 
the  etiology  of  many  of  the  diseases  coming  under  this  category,  and,  in 
part,  from  the  circumstance  that  the  terminology  has  been  and  still  is 
greatly  confused.  It  is  impossible,  therefore,  to  establish  a  classifica- 
tion that  is  entirely  satisfactory,  and  any  that  may  be  proposed  must 
be  largely  tentative. 

From  the  standpoint  of  the  morbid  histologist,  we  may  divide  hepatitis 
into  the  acute  parenchymatous,  the  acute  interstitial,  and  the  chronic 
interstitial,  an  arrangement  that  is,  perhaps,  as  little  open  to  cavil  as 
any.  It  should  be  remarked,  however,  that  these  types  are  not  always 
sharply  defined  one  from  the  other,  and  that  various  combined  or  mixed 
forms  occur. 

Acute  Parenchymatous  Hepatitis. — Acute  parenchymatous  hepatitis, 
an  affection  with  difficulty  distinguishable  from  cloudy  swelling,  is  a 
fairly  frequent  occurrence  in  the  course  of  the  infective  fevers,  particu- 
larly typhoid,  tuberculosis,  pneumonia,  and  septicemia.  The  liver  is 
slightly  enlarged,  and  on  section  is  pale,  grayish,  and  friable.  Micro- 
scopically, the  parenchymatous  cells  are  swollen  and  granular,  while 
the  nuclei  are  slightly  obscured.  Not  infrequently,  one  can  see  in  the 
portal  sheaths  small  collections  of  leukocytes,  which  may  even  extend 
for  some  little  distance  between  the  columns  of  liver  cells.  At  the  height 
of  the  process  the  parenchymatous  cells  become  fatty,  hyaline,  and 
often  vacuolated.  While  certain  of  them  necrose  and  disappear,  others 
seem  to  proliferate,  as  there  is  an  increase  in  the  number  of  the  nuclei 
and  also  in  their  size.  The  condition  approximates  to  the  type  of 
the  inflammatory  necroses  accompanied  by  a  reactive  and  reparative 
inflammation. 

Allied  to  this  affection  is  the  rarer  condition  in  which  necrosis  and 
atrophy  are  the  prominent  features,  the  regenerative  processes  being 


460  THE  LIVER 

for  a  time  at  least  in  the  background.  Wasting  is  marked  and  the 
shrinkage  of  the  liver  can  be  watched  clinically  day  by  day.  Of  this  the 
type  is  the  so-called  acute  yellow  atrophy  of  the  liver.  This  disease  begins 
with  all  the  signs  of  a  severe  systemic  intoxication — high  fever,  delirium, 
a  quickly-developing  jaundice,  multiple  hemorrhages,  and  finally,  con- 
vulsions and  coma.  A  fatal  termination  generally  takes  place.  In  the 
early  stages  the  liver  is  usually  enlarged,  but  quickly  diminishes  in  size. 
The  liver  may  be  reduced  to  one-half  its  natural  size,  its  consistency 
is  increased,  and  the  capsule  is  thrown  into  folds,  owing  to  the  shrinkage 
of  the  parenchyma.  On  section,  the  surface  is  of  a  bright,  lemon- 
yellow  color,  with  numerous  patches  of  a  darker  brownish-red.  The 

Fio.  116 


1 


Acute  yellow  atrophy  of  the  liver.  Section  shows  atrophy  of  parenchymatous  cells,  fragment- 
ation of  nuclei,  and  pseudobile  ducts.  Zeiss  obj.  A,  without  ocular.  (From  the  collection  of 
the  Royal  Victoria  Hospital.) 

reddish  color  usually  predominates  in  the  left  lobe.  The  yellow  areas 
are  soft  and  somewhat  swollen,  while  the  red  are  firmer  and  rather 
of  a  leathery  consistency.  Small  hemorrhages  are  common  throughout 
the  substance.  The  vessels  contain  thin  blood  and  the  bile  capillaries 
thin  bile. 

Histologically,  the  parenchymatous  cells  in  the  yellow  patches  show 
extensive  fatty  changes,  together  with  an  accumulation  of  bile  pigment 
and  crystals  of  bilirubin.  The  fatty  droplets  can  be  found  not  only 
within  the  cytoplasm,  but  also  within  the  nucleus.  The  reddish  areas 
are  composed  mainly  of  a  network  of  capillaries,  in  the  meshes  of  which 
can  be  seen  isolated,  degenerating  liver  cells,  and  a  detritus  of  broken- 
down  cells,  fragmented  nuclei,  masses  of  pigment,  chromatin,  and  blood. 


SUPPURATIVE  HEPATITIS  OF  ARTERIAL  ORIGIN  461 

Where  death  has  been  more  than  usually  delayed,  some  evidences  of 
regeneration  can  be  made  out.  Small  nodes  of  newly-formed  liver 
cells  may  be  found  here  and  there,  recognizable  by  the  fact  that  they  are 
larger  than  normal,  stain  more  intensely,  and  have  large,  deeply-staining 
nuclei,  some  of  them  exhibiting  mitosis.  A  round-celled  infiltration 
can  be  made  out  in  the  portal  sheaths  together  with  some  proliferation 
of  the  connective  tissue.  Pseudobile  capillaries  may  also  be  present  in 
considerable  numbers,  giving  the  section  somewhat  the  appearance  of 
a  cirrhosis. 

Acute  yellow  atrophy,  in  most  cases,  is  almost  certainly  an  acute 
degeneration,  the  result  of  a  circulating  toxin.  From  the  fact  that  cases 
sometimes  occur  in  epidemic  form,  though  rarely,  this  toxin  is,  probably, 
in  some  instances,  bacterial  in  origin.  The  disease  is  most  common 
between  the  ages  of  twenty  and  thirty,  and  is  usually  found  in  women 
during  the  puerperium.  Very  similar  forms  of  acute  degeneration 
and  necrosis  of  the  liver  parenchyma  occur  in  connection  with  poisoning 
by  phosphorus,  arsenic,  and  chloroform.  Several  cases  have  been  re- 
ported lately  resulting  from  chloroform  anesthesia.  They  usually  are  met 
with  in  children,  the  subjects  of  long-standing  and  debilitating  disease. 

Acute  Interstitial  Hepatitis. — Acute  interstitial  hepatitis  may  be  simple, 
but  is  more  often  suppurative.  It  is  due  to  infection  of  the  liver  by 
microorganisms  that  gain  entrance  to  it  through  the  general  blood  stream, 
the  portal  vein,  or  the  bile  passages.  In  newborn  children  infection 
through  the  umbilical  cord  is  an  occasional  cause.  In  rare  cases  a  retro- 
grade metastasis  of  bacterial  agents  may  take  place  from  the  superior 
vena  cava. 

Simple  interstitial  hepatitis  is  occasionally  seen  in  the  livers  of  persons 
dying  from  infectious  disease,  notably  typhoid  and  tuberculosis.  Besides 
cloudy  swelling  of  the  parenchyma,  small  collections  of  inflammatory 
round  cells,  of  greater  or  less  extent,  are  to  be  found  in  the  portal  sheaths. 
Some  authorities  are  inclined  to  attribute  some  importance  to  this 
manifestation  of  infectious  disease  in  the  etiology  of  cirrhosis. 

Acute  suppurative  interstitial  hepatitis  may  be  primary  or  secondary. 
The  primary  form  is  rather  rare  in  temperate  regions  but  is  more  common 
in  the  tropics,  where  it  is  known  under  the  name  of  tropical  abscess. 
The  cases  occurring  in  this  country  are  almost  invariably  due  to  trauma, 
such  as  severe  crushing  injuries  to  the  liver,  or  are  examples  of  wound 
infection.  The  vast  majority  of  cases,  as  we  meet  them  here,  are  second- 
ary to  infection  elsewhere,  and  are  either  metastatic  in  nature  or  arise 
by  direct  extension.  In  the  metastatic  forms  the  infective  agents  reach 
the  liver  by  way  of  the  hepatic  artery,  the  portal  vein,  or  the  bile  ducts. 
In  1474  autopsies  of  which  we  have  notes,  performed  at  the  Montreal 
General  and  Royal  Victoria  Hospitals,  there  were  46  cases  of  liver 
abscess;  22  were  due  to  portal  infection,  8  were  biliary,  3  gave  a  history 
of  dysentery,  3  cases  were  arterial,  7  occurred  from  extension,  and  1  was 
apparently  primary. 

Suppurative  Hepatitis  of  Arterial  Origin. — This  form  is  usually  but 
one  manifestation  of  a  generalized  septicemia,  the  infective  agents 


462  THE  LIVER 

reaching  the  liver  by  way  of  the  hepatic  artery.  The  etiology  of  this 
form  is  that  of  septicemia  in  general.  The  disease  may,  therefore,  com- 
plicate all  kinds  of  wound  infection,  ulcerative  endocarditis  and  aortitis, 
osteitis,  osteomyelitis,  putrid  bronchitis,  gangrene  of  the  lung,  infection 
of  the  puerperal  uterus,  and  suppurative  lesions  about  the  bladder, 
prostate,  and  urethra.  It  has  been  known  to  follow  carbuncle  and 
whitlow.  The  infective  agents  usually  at  work  are  the  Streptococcus 
pyogenes  and  the  Staphylococcus  albus  and  aureus. 

Suppurative  Hepatitis  of  Portal  Origin. — Here  the  infective  agents  come 
from  any  part  of  the  district  drained  by  the  portal  system.  Septic 
processes  in  the  appendix,  cecum,  small  intestine,  stomach,  pancreas, 
and  spleen  are  important  in  this  connection.  The  most  frequent  single 
cause  is  appendicitis  (peri typhlitis).  Combining  the  statistics  of  Arm- 
strong,1 Einhorn,  Langheld,  and  Fitz  in  546  cases  of  appendicitis  that 
were  examined  post  mortem,  pylephlebitis  and  abscess  of  the  liver  were 
found  in  28,  or  rather  more  than  5  per  cent.  Exceptionally,  in  cases  of 
cholecystitis  and  pericholecystitis,  extension  of  the  process  to  the  portal 
vein  may  result  in  an  embolic  infection  of  the  liver.  The  lesion  is  in 
many  instances  essentially  an  acute  thrombophlebitis  of  the  portal  vein 
or  a  purulent  infiltration  of  the  vessel  wall  and  adjacent  parts — suppura- 
tive portal  pyelephlebitis.  In  a  typical  case  of  septic  portal  infection 
the  liver  contains  a  number  of  abscesses  of  varying  size.  These  are 
grouped  about  the  branches  of  the  portal  vein,  after  the  fashion  of 
currants  upon  a  stem.  The  larger  abscesses  are  often  irregular  or 
lobular  in  appearance,  owing  to  the  fact  that  they  result  from  the  con- 
fluence of  smaller  foci.  Small,  isolated  abscesses  are  often  to  be  found 
grouped  about  the  larger  ones,  but  separated  from  them  by  a  small  amount 
of  comparatively  unaltered  liver  tissue.  The  abscesses  are  filled  with 
yellowish-green,  viscid  pus,  often  mixed  with  blood,  having  a  foul  odor, 
and  sometimes  containing  sequestra  of  liver  substance.  Round  about  the 
abscesses  is  a  narrow  zone  of  a  yellowish-white  color,  or,  in  cases  of 
a  gangrenous  type,  of  a  dirty  green  appearance.  The  portal  vein  and 
its  branches  within  the  liver  usually  contain  more  or  less  dirty-looking 
septic  clot.  The  abscesses  are  chiefly  to  be  found  in  the  right  lobe. 
Where  the  infection  has  taken  place  through  the  hepatic  artery  the  foci  of 
suppuration  are  usually  smaller  and  more  widely  and  evenly  distributed. 

Histologically,  in  the  more  recent  lesions,  the  smaller  branches  of  the 
portal  vein  or  the  arterial  capillaries,  as  the  case  may  be,  are  packed 
with  microorganisms.  Round  about  are  extensive  accumulations  of 
inflammatory  leukocytes,  tending  to  stain  badly  toward  the  centre.  In 
the  larger  abscesses  the  central  portion  has  broken  down  and  the  cells 
composing  it  are  in  various  stages  of  necrosis  and  disintegration.  At 
the  periphery  of  the  abscess  the  leukocytes  gradually  lose  themselves 
between  the  liver  cells.  The  parenchyma  of  the  liver  in  the  neighborhood 
presents  the  appearance  of  coagulation  necrosis.  The  specific  cells  are 
swollen,  hyaline,  or  compressed,  containing  nuclei  that  stain  badly. 

1  Brit.  Med.  Jour.,  2:1897:945. 


SUPPURATIVE  HEPATITIS  ARISING  PER  EXTENSIONEM       463 

\\hen  the  abscesses  are  near  the  surface,  the  Glisson's  capsule  is  often 
infiltrated,  the  endothelial  cells  are  in  process  of  desquamation,  and 
there  may  be  a  deposit  of  fibrinopurulent  exudate  (perihepatitis) .  The 
liver,  as  a  whole,  is  congested  and  more  or  less  swollen  and  cloudy.  The 
cases  are  usually  fatal,  but  instances  are  on  record  where  the  abscesses 
have  ruptured  into  the  lung  or  intestine  with  spontaneous  cure.  Healing 
may  take  place  with  the  evacuation  or  absorption  of  the  pus  and  the  for- 
mation of  fibrous  scars. 

FIG.  117 


Multiple  abscesses  of  the  liver.      Zei.ss  obj.  DD,  without  ocular.     (From  the  collection 
of  Dr.  A.  G.  Nicholls.) 

Suppurative  Hepatitis  of  Biliary  Origin. — Here  infection  takes  place 
through  the  bile  passages.  The  most  common  cause  is  cholelithiasis 
with  associated  cholangitis;  in  other  cases  the  infection  results  upon 
obstruction  of  the  bile  duct  from  tumors  or  cysts.  The  bile  passages 
may  occasionally  be  infected  from  the  intestine,  as  in  dysentery  and 
typhoid.  Lannois1  has  recorded  a  case  of  liver  abscess  occurring  in 
typhoid.  Rarely,  ascarides  may  invade  the  ductus  communis  and, 
dying  there,  give  rise  to  marked  disturbance.  Leick  has  collected  19 
instances  of  this  kind. 

The  description  of  the  various  abscesses  given  above  holds  fairly 
well  for  this  variety.  The  pus,  however,  is  usually  mixed  with  bile, 
and  may  contain  gritty  matter  or  concretions. 

Suppurative  Hepatitis  Arising  per  Extensionem. — This  is  a  fairly  common 
form  of  suppuration  in  the  liver.  The  process  is  usually  superficial, 

1  Revue  de  Medecine,  15: 1895: 909. 


464  THE  LIVER 

affecting  the  capsule  and  that  portion  of  the  liver  immediately  beneath 
it.  Diaphragmatic  and  subdiaphragmatic  abscesses,  empyema,  suppu- 
rative  cholecystitis  and  choledochitis,  perinephric  abscess,  abscess  of  the 
head  of  tthe  pancreas,  perforating  ulcers  of  the  stomach  and  duodenum 
invade  the  liver.  Foreign  bodies,  such  as  fish-bones,  needles,  or  other 
sharp-pointed  articles,  may  pass  through  the  stomach  or  intestines  and 
become  lodged  in  the  liver,  where  they  may  set  up  suppurative  inflam- 
mation. 

Tropical  Abscess. — This  variety  is  characterized  by  the  formation  of 
one  or  more  foci  of  necrosis  and  softening  in  the  liver,  either  with  or 
without  a  relation  to  dysentery.  Unlike  the  forms  of  abscess  we  have 
been  describing,  the  rule  here  is  for  one  large  or  at  most  but  a  few 
abscesses  to  be  developed. 

The  cases  are  met  with  usually  in  tropical  and  subtropical  countries, 
whence  the  name.  The  etiological  factors  are  as  yet  but  imperfectly 
understood.  Some  few  cases  appear  to  be  due  to  dietetic  errors;  free 
living,  the  excessive  use  of  animal  food,  and  particularly  excess  in  alco- 
hol, are  thought  by  some  to  be  particularly  potent  causes.  We  must, 
in  these  cases,  assume  that  there  is  some  previous  deterioration  of  the 
resisting  power  of  the  liver  that  renders  it  a  more  easy  prey  to  toxins  and 
invading  microorganisms.  The  great  majority  of  cases  are  associated 
with  tropical  dysentery.  In  this  connection  Kartulis1  states  that  in 
500  cases  of  liver  abscess  coming  under  his  notice,  from  50  to  60  per  cent, 
gave  a  history  of  dysentery.  There  must  be  something  peculiar  about 
the  condition,  for  in  the  catarrhal  and  ulcerative  dysenteries  found  in 
temperate  climes  liver  abscess  is  excessively  rare.  The  studies  of  Kartu- 
lis,  Losch,2  Kruse  and  Pasquale,3  Councilman  and  Lafleur,4  have  shown 
that  a  fairly  large  proportion  of  cases  of  dysenteric  origin  are  due  to 
the  amoeba  coli.  What  this  proportion  is  is  uncertain.  Flexner  is  of 
the  opinion  that  the  great  majority  of  dysenteric  cases  are  of  amoebic 
origin.  In  a  large  experience  he  did  not  find  a  single  case  due  to  the 
Shiga  bacillus.  A  few  writers,  however,  refer  to  the  discovery  of  a 
typhoid-like  bacillus  which  possibly  was  this  organism,  and  Pansini  and 
Babes  seem  to  have  obtained  it  or  a  similar  germ  in  several  cases  of 
liver  abscess. 

The  amoebic  abscess  in  about  75  per  cent,  of  the  cases  is  solitary, 
and  in  about  the  same  proportion  is  situated  in  the  right  lobe,  usually  in 
the  dome  or  under  surface  near  the  hepatic  flexure  of  the  colon.  In 
early  cases  the  necrotic  area  may  be  scarcely  liquefied,  and  is  hyperemic, 
spongy,  and  infiltrated  with  a  glairy  tenacious  material.  Later,  a  regu- 
lar abscess  is  formed,  its  walls  formed  of  necrotic  liver  substance  and 
shreddy,  connective  tissue.  The  contents  of  the  abscess  vary  much. 
In  some  few  cases  the  fluid  is  serous,  but  in  most  there  is  a  mixture  of 
pus  and  necrotic  material.  The  pus  is  somewhat  glairy  and  translucent 

1  Centralb.  f.  Bakter.,  2:1887;  Virch.  Archiv,  118:1889. 

2  Virch.  Archiv.,  65:1875.  3  Zeitschr.  f.  Hyg.  u.  Infectionskrankh.,  16:1. 
4  Johns  Hopkins  Hosp.  Rep.,  2:1891. 


CIRRHOSIS  465 

in  some  cases,  in  others,  grayish  or  brownish-red,  so  that  its  appearance 
has  been  compared  to  anchovy  sauce.  The  quantity  varies  from  a  few 
ounces  to  many  pints.  Practically  the  whole  of  the  right  lobe  of  the  liver 
has  been  found  in  some  cases  to  be  occupied  by  a  huge  abscess  enclosed 
in  a  thin  shell  of  liver  substance.  In  long-standing  cases,  the  abscess 
becomes  walled  off  by  pyogenic  membrane  or  a  fibrous  capsule.  The 
liver  substance  in  the  neighborhood  is  generally  congested,  softened, 
cloudy,  and  friable,  and  shows  other  signs  of  degeneration,  but  in  some 
cases  has  been  found  to  be  practically  normal. 

Histologically,  the  contents  of  the  abscess  are  a  finely  granular  detritus, 
broken-down  liver  cells,  red  and  white  blood-corpuscles,  and  hematoidin. 
There  is  usually  a  more  or  less  widespread  necrosis  of  the  liver  paren- 
chyma. The  amoebse  are  found  chiefly  at  the  periphery  of  the  abscess, 
in  the  capillaries,  and  about  the  portal  sheaths.  They  are  more  numerous 
in  the  smaller  foci.  An  important  point  is  that  leukocytes  are  scanty  in 
the  contents  of  the  abscess  and  in  the  wall,  except  in  cases  where  second- 
ary infection  has  taken  place,  showing  that  the  lesion  is  essentially  a 
non-suppurative  one.  In  many  cases  bacteria  are  found  in  the  abscesses, 
notably  staphylococci  and  streptococci.  The  earlier  observers  were 
of  the  opinion  that  the  necrosis  was  due  to  the  pyogenic  cocci,  while 
some  others  held  that  the  amoebse  acted  as  carriers  for  the  germs  and  by 
their  growth  and  movement,  which  ruptured  the  capillaries,  paved  the 
way  for  bacterial  infection.  The  preponderance  of  evidence  at  the  present 
day,  however,  is  in  favor  of  the  view  that  the  amoebse  are  the  direct  cause 
of  the  lesions. 

Most  cases  end  fatally,  but  if  death  do  not  take  place  soon,  or  if 
the  condition  be  not  relieved  by  operative  interference,  the  abscess  may 
rupture  and  give  rise  to  further  serious  consequences.  The  most  fre- 
quent event  is  for  the  rupture  to  take  place  into  the  right  pleura  or 
lung,  the  fluid  subsequently  being  discharged  through  a  bronchus;  next 
to  that,  into  the  peritoneal  cavity.  More  rarely,  the  abscess  may  dis- 
charge into  the  pericardium,  sudden  death  being  the  result.  Again, 
the  abscess  may  point  externally  or  empty  into  the  transverse  colon, 
stomach,  or  duodenum;  very  rarely,  into  the  bile  passages,  the  hepatic 
vein,  the  vena  cava,  or  the  pelvis  of  the  right  kidney. 

In  exceptional  cases  the  process  becomes  latent  or  comes  to  an  end. 
Small  foci  of  suppuration  may  be  absorbed  with  the  formation  of  a  scar. 
In  the  case  of  larger  ones,  the  fluid  part  of  the  contents  is  absorbed,  and 
we  get  a  small  cavity  walled  in  by  a  connective-tissue  capsule  and 
filled  with  a  cheesy  substance  in  which  lime  salts  may  subsequently 
be  deposited. 

Cirrhosis. — Under  the  terms  cirrhosis,  chronic  interstitial  hepatitis, 
productive  hepatitis,  are  included  a  variety  of  morbid  conditions,  which, 
while  they  differ  in  regard  to  their  etiology  and  the  minuter  details  of 
structural  change,  have  this  in  common,  that  there  is  a  hyperplasia  of 
connective  tissue,  which,  in  time,  becomes  sufficient  to  markedly  inter- 
fere with  the  functions  of  the  organ.  Much  confusion  of  mind  has  existed 
with  regard  to  these  conditions,  owing  to  the  difficulty  of  bringing  clinical 
30 


466  THE  LIVER 

symptoms  into  harmony  with  the  anatomical  appearances  found  post 
mortem,  but  perhaps  even  more  from  the  lax  use  of  terms.  Particu- 
larly unfortunate  are  the  terms  "  hypertrophic"  and  "atrophic"  as 
applied  to  cirrhosis,  for  at  best  they  are  merely  relative,  and,  moreover, 
have  been  used  in  an  erroneous  manner  as  synonymous  with  enlargement 
or  diminution  in  size. 

All  the  evidence  at  our  disposal,  whether  clinical  or  experimental, 
clearly  indicates  that  cirrhosis  or  fibrosis  of  the  liver  is  in  most  cases  the 
result  of  the  irritation  of  some  toxin,  bacterial  or  otherwise,  which  may 
reach  the  liver  in  one  or  other  of  three  ways,  i.  e.,  through  the  hepatic 
artery,  through  the  portal  vein,  or  through  the  bile  passages.  The 
occurrence  of  cirrhosis  as  a  result  of  infection  or  intoxication  operating 
through  the  hepatic  artery,  while  theoretically  possible,  is  not  established 
by  positive  evidence.  The  cirrhosis  resulting  from  the  action  of  coal 
dust — cirrhosis  anthracotica — may  perhaps  be  of  this  nature.  It  is  beyond 
question,  however,  that  irritants  may  reach  the  liver  through  the  portal 
vein  and  the  bile  ducts,  so  that  we  can  at  once  recognize  two  important 
classes  of  cases — portal  cirrhosis  and  biliary  cirrhosis.  A  fourth  form, 
one  that  is  included  with  doubtful  propriety  among  the  cirrhoses,  is 
called  capsular  cirrhosis  or  perihepatitis  with  cirrhosis,  in  which  the  irri- 
tants affect  primarily  the  capsule.  A  few  cases  also  are  met  with  of 
fibrosis  of  the  liver  that  are  more  akin  to  degeneration  than  to  inflam- 
mation, of  which  the  cirrhosis  following  chronic  passive  congestion 
is  the  most  important  form.  The  following  classification,  based  mainly 
upon  anatomical  and  histological  grounds,  will  be  found  useful,  and 
has  at  least  the  merit  that  it  fits  in  fairly  well  with  clinical  observations : 

I.  Portal  cirrhosis  with  enlargement. 

II.  Portal  cirrhosis  with  contraction   (Laennec's  cirrhosis;  hob- 
nailed liver,  gin-drinker's  liver). 

III.  True  biliary  cirrhosis  (Hanot's  form) 

IV.  Obstructive  biliary  cirrhosis  with  enlargement. 
V.  Obstructive  biliary  cirrhosis  with  contraction. 

VI.  Pericellular  or  diffuse  cirrhosis. 
VII.  Capsular  cirrhosis  (perihepatitis  with  cirrhosis). 

VIII.  Senile  atrophy  and  arteriosclerosis. 
IX.  Cirrhosis  from  passive  congestion. 

Portal  Cirrhosis  with  Enlargement. — The  first  form  mentioned  is  in 
many  cases,  in  our  opinion,  simply  an  early  stage  of  Laennec's  cirrhosis. 
We  have  met  with  one  or  two  instances  in  which  the  liver  was  at  first 
greatly  enlarged,  and  at  death,  many  months  later,  the  ordinary,  small, 
hob-nailed  liver  was  discovered.  This  preliminary  enlargement  is  not 
likely  to  be  due  to  hypertrophy,  but  rather  to  acute  congestion  and 
inflammatory  infiltration.  Hypertrophy  and  hyperplasia  of  the  liver 
cells  proper  undoubtedly  occurs  in  the  various  forms  of  cirrhosis,  but  is 
particularly  marked  in  the  contracted  or  hob-nailed  liver,  called  im- 
properly the  liver  of  "  atrophic"  cirrhosis.  Such  hyperplasia  occurs  some- 
what late  in  the  disease  and  always  falls  short  of  the  amount  necessary 
to  restore  the  perfect  function  of  the  organ.  Conversely,  hyperplasia 


LAENNEC'S  CIRRHOSIS 


467 


is  not  a  marked  feature  in  cases  of  cirrhosis  with  enlargement,  sometimes 
called  "  hypertrophic"  cirrhosis.  As  a  matter  of  fact,  hyperplasia  of  the 
liver  parenchyma  and,  of  course,  of  the  connective  tissue  is  present  in 
a  variable  degree  in  all  forms  of  cirrhosis,  so  that  it  would  conduce  to 
clearness  if  the  terms  "atrophic"  and  "hypertrophic"  were  discontinued 
in  connection  with  this  subject. 

A  second  and  important  form  of  portal  cirrhosis  with  enlargement 
is  the  so-called  fatty  cirrhosis.  Here,  in  addition  to  the  ordinary  anatom- 
ical changes  characteristic  of  portal  cirrhosis,  there  is  more  or  less  extreme 
fatty  infiltration. 

FIG.  118 


Slight  cirrhosis  of  the  liver  with  fatty  infiltration.     Zeiss  obj.  A,  without  ocular, 
the  collection  of  Dr.  A.  G.  Nicholls.) 


(From 


The  organ,  in  a  well-marked  case,  is  much  enlarged,  with  a  smooth, 
or,  possibly,  a  slightly  granular  surface.  The  edges  are  rounded  and  the 
color  is  yellow.  The  appearance  is  that  of  the  fattily  infiltrated  liver, 
but  with  this  difference,  that  the  organ  is  very  firm,  is  not  friable,  and 
cuts  with  difficulty,  owing  to  the  excess  of  fibrous  tissue  that  is  present. 

Microscopically,  there  is  usually  a  moderate  amount  of  cirrhosis,  of 
the  type  to  be  described  under  the  next  head,  but  the  remaining  liver 
parenchyma  is  more  or  less  fatty,  the  cells  being  filled  with  large  fatty 
globules.  In  some  instances  there  may  be  no  normal  liver  cells  left. 
The  fatty,  cirrhotic  liver  may  be  latent,  being  found  accidentally  at 
autopsy,  but  in  some  cases  gives  rise  to  a  train  of  symptoms  similar  to 
those  in  Laennec's  cirrhosis.  This  form  of  liver  is  met  with  usually  in 
connection  with  pronounced  alcoholism. 

Portal  Cirrhosis  with  Contraction. — This  is  the  form  variously  designated 
as  Laennec's  cirrhosis,  atrophic  cirrhosis,  hob-nailed  liver,  gin-drinker's 


468  THE  LIVER 

liver,  contracted  liver.  While  alcohol  has  been  credited  with  being  the 
etiological  factor  in  about  60  percent,  of  cases,  experimental  investigations 
do  not  bear  this  out.  Probably  alcohol  acts  not  directly  but  indirectly, 
like  a  variety  of  other  substances,  in  bringing  about  cirrhosis.  Alcohol 
produces  gastro-intestinal  catarrh,  this  leads  to  abnormal  fermentative 
processes  and  the  elaboration  of  toxins,  which  toxins,  in  turn,  act  upon 
the  liver.  The  finer  details  of  the  process  have  led  to  much  discussion, 
but  it  is  probable  that  the  toxins  lead  primarily  to  degenerative  changes 
in  the  parenchymatous  cells,  which  necrose  and  are  replaced  by  new 

FIG.  119 


Laennec's  cirrhosis,  portal  cirrhosis,  hobnailed  liver,  gin-drinker's  liver.  A  transverse  section 
is  made  through  the  organ  and  the  upper  portion  is  turned  upward.  (From  the  Pathological 
Museum  of  McGill  University.) 

connective  tissue.  It  is  not  impossible,  however,  that  a  vicious  circle 
is  produced  and  that  irritants  in  the  blood  act  through  the  vessels  upon 
the  fibrous  structure  of  the  portal  sheaths,  stimulating  it  directly  to  hyper- 
plasia  (cf.  p.  377  et  seq.). 

To  gross  appearance  the  liver  is  more  or  less  diminished  in  size,  in 
advanced  cases  perhaps  not  weighing  more  than  half  the  normal  amount. 
The  organ  is  deformed  and  the  surface  warty,  being  covered  with  gran- 
ulations or  nodules,  ranging  in  size  from  that  of  a  pin-head  to  a  bean. 
The  color  is  yellowish,  orange,  or  yellowish-green,  whence  the  term 
"cirrhosis,"  according  to  the  amount  of  bile  staining  that  has  occurred. 


LAENNEC'S  CIRRHOSIS  469 

The  granulation  is  often  most  marked  in  the  left  lobe  and  on  the  anterior 
border.  The  capsule  is  usually  thickened,  and  there  are  commonly 
adhesions  between  the  liver  and  the  diaphragm.  The  gall-bladder 
is  generally  diminished  in  size.  On  section,  the  organ  cuts  firmly,  with 
a  resistant  feel,  and  it  can  then  be  seen  that  the  depressions  on  the 
surface  are  associated  with  bands  of  fibrous  tissue  which  form  a  net- 
work throughout  the  liver,  dividing  the  parenchyma  into  a  great  number 
of  sharply  defined  islets.  The  fibrous  bands  are  of  a  whitish  or  pinkish- 
ii'ruv  color,  slightly  translucent  in  appearance,  and  project  above  the 
general  level,  thus  giving  the  cut  surface  an  irregular,  granular  appear- 
ance. 

Histologically,  the  most  striking  feature  is  the  enormous  proliferation 
of  the  connective  tissue  which  forms  a  series  of  connected  bands  enclosing 
the  lobules.  These  bands  may  be  distributed  so  as  to  enclose  several 
lobules  (polylobular  cirrhosis)  or  to  isolate  each  lobule  separately 
(monolobular  cirrhosis).  Both  conditions  are  usually  to  be  made  out 
in  cases  of  Laennec's  cirrhosis,  and  may  be  included  under  the  terms 
"  perilobular"  or  "  interlobular"  cirrhosis.  In  advanced  cases,  the  fibrous 
tissue  may  insinuate  itself  between  the  individual  columns  of  liver  cells 
(intralobular  cirrhosis) ;  but  this  is  never  a  marked  feature,  although  it 
is  not  at  all  uncommon  in  the  biliary  forms.  Where  the  inflammatory 
process  is  recent  or  active,  the  portal  sheaths  are  more  or  less  densely 
infiltrated  with  clusters  of  round  cells,  in  part,  fibroblasts,  and,  probably, 
in  part  leukocytes.  In  the  older  portions  or  where  the  disease  has  about 
run  its  course,  the  round  cells  are  scanty,  and  we  find  dense  bands  of 
fibrous  tissue  having  the  characteristic  fibrillation  of  the  adult  tissue, 
with  relatively  few  elongated  nuclei.  In  the  bands  of  fibrous  tissue  are 
found  groups  of  cells  arranged  in  parallel  rows  closely  resembling  bile 
capillaries.  Their  origin  is  somewhat  debatable.  They  are  generally 
more  abundant  in  the  parts  where  cellular  infiltration  is  conspicuous, 
for,  as  the  fibrous  tissue  is  formed  it  seems  to  crush  them  out  of  existence. 
These  apparent  bile  capillaries  may  be  fairly  numerous,  but  in  some  cases, 
again,  are  scanty  or  even  absent.  Three  modes  of  origin  are  possible: 
they  may  be  new  ducts  derived  by  proliferation  of  the  original  bile 
capillaries;  they  may  be  bile  capillaries  that  have  persisted  after  destruc- 
tion of  the  lobules  to  which  they  belonged;  or  they  may  indicate  rever- 
sionary degeneration  of  the  liver  cells.  On  careful  examination,  one 
will  often  see  that  these  apparently  newly-formed  capillaries  do  not  re- 
semble capillaries  so  much  as  they  do  columns  of  liver  cells  that  have 
undergone  atrophy,  for  the  protoplasm  of  their  component  cells  strikes 
a  color  with  hematoxylin-eosin  similar  to  that  of  the  normal  liver 
parenchyma.  Further,  certain  of  them  will  be  found  to  be  in  direct 
connection  with  the  cells  of  the  periphery  of  the  liver  lobules.  Such 
pseudocapillaries,  therefore,  probably  represent  columns  of  liver  cells 
that  have  reverted  to  a  more  primitive  condition. 

In  addition,  bloodvessels  can  be  made  out  in  the  fibrous  septa,  which 
are  numerous,  large,  and  thin-walled.  These  can  be  injected  through 
the  hepatic  artery.  The  branches  of  the  portal  vein,  on  the  other  hand, 


470  THE  LIVER 

either  from  external  pressure  or  changes  in  the  intima,  are  more  or  less 
occluded,  thus  accounting  for  the  portal  obstruction  that  sooner  or  later 
becomes  so  marked  a  feature  in  this  form  of  cirrhosis.  The  liver  cells 
themselves  often  present  little  alteration,  but  at  the  periphery  of  the  lob- 
ules many  of  them  present  atrophic  changes,  being  fattily  degenerated  or 
reduced  to  flattened  plates,  or,  perhaps,  little  more  than  nuclei.  The 
atrophy  is  to  some  extent  due  to  the  encroachment  of  the  fibrous  tissue, 
but  also,  no  doubt,  to  the  influence  of  circulating  toxins  and  impaired 
nutrition.  Occasionally,  there  is  marked  fatty  infiltration,  as  in  the 
fatty  cirrhosis  above  described.  Here  and  there  attempts  at  compensa- 
tion can  be  made  out,  for  masses  of  liver  cells,  more  or  less  perfectly 
reproducing  the  lobules,  can  be  seen,  composed  of  newly-formed  cells, 
which  are  large,  stain  deeply,  and  show  nuclei  in  various  stages  of 
mitosis.  Pigment  granules  of  a  yellowish-brown  or  yellowish-green  color 
are  also  to  be  seen  within  the  specific  cells,  in  the  interstices  of  the 
connective  tissue,  and  in  the  endothelial  cells  lining  the  vessels.  Some 
of  these  may  be  biliary  pigment,  but  not  infrequently  they  can  be  shown 
to  contain  iron,  probably,  therefore,  being  hemosiderin  derived  from 
broken-down  red  blood  corpuscles.  Cases  are  on  record  where  sufficient 
of  this  iron-containing  pigment  has  been  produced  to  stain  most  of 
the  tissues  of  the  body,  including  the  skin,  a  bluish-gray  or  leaden  color. 
This  is  the  condition  known  as  hemochromatosis  (see  p.  481). 

In  well-marked  cases  of  Laennec's  cirrhosis  the  portal  vein  and  its 
branches  are  dilated.  Dilated  veins  may  often,  also,  be  seen  in  the 
skin,  especially  on  the  sides  of  the  abdomen  and  thorax  and  around  the 
umbilicus  (caput  Medusa).  The  cesophageal,  gastric,  and  hemor- 
rhoidal  veins  are  usually  dilated  and  may  be  varicosed.  Rupture  of 
the  cesophageal  vessels  may  give  rise  to  fatal  hemorrhage.  The  spleen 
is  moderately  enlarged,  its  capsule  thickened,  and  it  is  sometimes 
embedded  in  adhesions.  Not  infrequently  the  pancreas  is  cirrhotic  as 
well.  The  gastro-intestinal  mucosa  is  in  a  state  of  passive  congestion. 
Ascites  is  present  in  about  half  the  cases.  Jaundice  occurs  in  about 
27  per  cent.,  and  is  usually  slight.  A  not  unusual  complication  is 
tuberculous  peritonitis. 

Hanot's  Cirrhosis. — Hanot's  cirrhosis,  sometimes  called,  though  erro- 
neously, hypertrophic  cirrhosis,  and  sometimes  "true"  biliary  cirrhosis, 
is  a  rare  form  in  this  country.  In  fact,  it  seems  to  be  uncommon  outside 
of  France.  The  disease  is  characterized  by  enlargement  of  the  liver 
and  jaundice,  which  persists  for  months  or  even  years.  The  jaundice 
is  often  intense,  as  in  the  obstructive  form,  but  the  stools  are  usually 
colored.  In  some  cases  the  jaundice  comes  on  acutely  and  the  disease 
runs  a  course  like  that  of  acute  febrile  icterus,  or,  again,  of  acute  yellow 
atrophy. 

The  liver  is  greatly  enlarged,  weighing  from  2  to  4  kg.  The  surface 
is  smooth,  or  at  most  covered  with  flat  prominences.  On  section,  the 
organ  is  firm  and  grates  under  the  knife.  The  cut  surface  is  yellowish 
or  yellowish-green,  owing  to  bile  staining,  and  has  a  finely  granulated 
appearance  resembling  shagreen  leather. 


PERICELLULAR  CIRRHOSIS  471 

Histologically,  there  is  an  overgrowth  of  connective  tissue,  often 
rather  poor  in  nuclei,  which  is  diffuse  in  character,  tending  to  invade 
the  lobules  (intralobular  cirrhosis)  or  isolate  small  groups  of  liver  cells. 
The  islets  of  parenchyma  remaining  are  small,  and  single  cells  or  small 
groups  of  cells  are  seen  separated  one  from  the  other  by  delicate  strands 
of  connective  tissue.  There  is  usually  a  considerable  production  of 
pseudobile  capillaries.  The  cells  contain  bile  pigment.  The  process 
seems  to  be  essentially  due  to  a  catarrh  of  the  finer  bile  capillaries  and 
the  portal  system  appears  to  be  free.  An  infective  origin  is  probable, 
but  the  etiology  has  not  yet  been  thoroughly  worked  out. 

Obstructive  Biliary  Cirrhosis. — This  form  is  due  to  some  gross  obstruc- 
tion to  the  free  outflow  of  bile,  either  within  the  ducts,  as  from  a  calculus, 
carcinoma,  or  a  fibrous  stricture;  or  from  without,  as  from  a  new-growth 
in  the  head  of  the  pancreas  or  in  the  periportal  lymph-nodes.  Obstruc- 
tion alone  does  not  appear  to  be  an  adequate  cause,  for  complete  occlusion 
of  the  ducts  may  occur  without  the  production  of  cirrhosis.  Another 
factor  is  necessary,  and  this  is  apparently  inflammation  in  the  form 
of  a  cholangitis.  This  is  easily  accounted  for,  since  bacteria  are  invari- 
ably to  be  found  in  the  bile  passages  in  such  cases,  a  catarrhal  inflam- 
mation, originally  dependent  on  microbic  infection,  being  the  cause  of 
the  calculus  formation  in  the  first  place. 

In  the  earlier  stages  of  the  disease  the  liver  is  much  enlarged,  closely 
resembling  the  liver  in  Hanot's  cirrhosis.  In  one  of  our  cases,  in  which 
a  stone  was  found  in  the  hepatic  duct,  the  organ  weighed  4850  grams. 

To  gross  appearance,  the  liver  is  enlarged,  smooth,  and  bile-stained. 
It  cuts  firmly,  the  cut  surface  being  finely  granulated  and  of  a  yellowish- 
green  color.  The  bile  ducts  can  be  seen  to  be  dilated  and  their  walls 
thickened. 

Microscopically,  the  newly-formed  connective  tissue  is  both  inter- 
lobular  and  intralobular  in  its  distribution  and  is  rather  dense.  It  is 
characterized  by  the  presence  of  central  dilated  bile  ducts  surrounded  by 
chaplets  of  pseudobile  ducts  which  are  conspicuous  by  their  numbers. 
Degenerative  changes  can  be  made  out  in  the  parenchymatous  cells. 
Focal  necroses  may  be  found.  Ascites  is  rare,  and  the  spleen  is  not 
greatly  enlarged.  The  portal  circulation  appears  to  be  not  notably 
interfered  with.  Pyogenic  infection  of  the  bile  passages  may  occur 
with  the  production  of  a  suppurative  cholangitis  and  the  formation  of 
small  abscesses  along  the  course  of  the  infected  ducts.1 

In  another  type  of  the  affection,  probably  a  later  stage  of  that  just 
described,  the  liver  is  contracted,  bile-stained,  and  has  a  fairly  diffuse 
granulation,  the  granulations  being  of  medium  size  and  rather  even  in 
character. 

Pericellular  Cirrhosis.— In  this  form  of  cirrhosis  there  is  a  singularly 
diffuse  hyperplasia  of  connective  tissue,  which  tends  to  surround  and 
isolate  very  small  groups  of  liver  cells  or  even  individual  cells.  The 

1  For  a  useful  consideration  of  this  form  of  cirrhosis,  see  Ford,  Amer.  Journ.  Med. 
Sci.,  121 : 1901 :  60. 


472  THE  LIVER 

fibrous  tissue  is  curiously  transparent  and  almost  hyaline  looking,  with 
but  little  fibrillation  and  relatively  few  nuclei.  Degenerative  changes 
in  the  parenchyma,  save  atrophy,  are  not  pronounced. 

Pericellular  cirrhosis  is  met  with  occasionally  in  syphilis  and  tuber- 
culosis of  the  liver  (q.  v.),  and  is  also  well  exemplified  in  a  curious  affec- 
tion of  cattle,  studied  by  Osier,  Wyatt  Johnston,  and  Adami,1  known  as 
"Pictou  Cattle  Disease."  This  disease  is  restricted  to  a  particular 
district  of  Pictou  County,  Nova  Scotia,  and  appears  to  be  infectious. 
In  some  particulars  it  is  not  unlike  Laennec's  cirrhosis  in  human  beings. 
There  is  a  moderate  ascites,  a  right-sided  pleural  effusion,  without 


FIG.  120 


" 
*•• 


:f  -» 

-,o., 


Pericellular  cirrhosis  of  the  liver  from  a  case  of  Pictou  cattle  disease.  Note  the  great  relative 
isolation  of  the  liver  cells  and  the  delicate  connective  tissue  with  scanty  nuclei.  Reichert  obj. 
7a,  without  ocular.  (From  the  collection  of  Dr.  J.  G.  Adami.) 

jaundice,  and  the  abdominal  and  periportal  lymph-nodes  are  enlarged 
and  succulent.  There  is  a  curious  gelatinous  oedema  of  the  mesentery 
and  the  intestinal  walls.  The  liver  is  moderately  enlarged,  with  a 
smooth,  or  occasionally  a  slightly  granular  surface.  Recent  and  healing 
ulcers  are  found  in  the  fourth  stomach. 

Capsular  Cirrhosis  (Perihepatitis  ;  Pseudocirrhosis  ;  "  Zuckergussebler"  I 
Glissonitis).  —  This  is  a  somewhat  rare  affection  of  the  liver,  in  which 
the  surface  is  more  or  less  completely  covered  with  a  thick,  fibrous 
investment,  of  pearly  white,  cartilaginous  appearance,  and  hyaline 
structure.  The  liver  is  at  first  enlarged,  but  subsequently  undergoes 
marked  contraction.  In  the  majority  of  instances  the  affection  is  simply 

1  Montreal  Med.  Journ.,  31  :  1902:  105. 


SENILE  ATROPHY  OF  THE  LIVER  473 

a  peculiar  form  of  perihepatitis,  and  is  usually  associated  with  a  similar 
transformation  of  the  capsule  of  the  spleen,  the  peritoneum,  and  the 
other  serous  membranes.  In  fact,  the  condition  is  often  part  and  parcel 
of  a  chronic  progressive  multiserositis,  or,  as  one  of  us  (A.  G.  N.)  has 
called  it,  "hyaloserositis."  The  relationship  of  cirrhosis  of  the  liver  to 
this  form  of  capsulitis  or  perihepatitis  is  still  somewhat  obscure.  It  is 
rare  to  find  a  true  cirrhosis  associated  with  the  condition.  The  liver, 
rather,  shows  brown  atrophy  and  passive  congestion.  At  most,  there 
may  be  a  slight  invasion  of  the  liver  substance  by  small  fibrous  bands 
passing  down  from  and  in  connection  with  the  capsule.  When  cirrhosis 

FIG.  121 


Chronic  perihepatitis  with  hyaline  transformation  of  the  exudate  (hyaloserositis).  The 
Glisson's  capsule  is  thrown  into  folds  from  atrophy  of  the  liver  parenchyma.  Leitz  obj.  No.  7, 
without  ocular.  (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

is  present  in  these  cases,  as  it  undoubtedly  may  be,  it  is  more  probably 
due  to  passive  congestion,  or  at  all  events  is  to  be  regarded  as  a  con- 
dition quite  distinct  from  the  perihepatitis.1 

Senile  Atrophy  of  the  Liver. — Here  the  liver  is  small  and  shrunken, 
the  capsule  thrown  into  folds,  and  the  whole  organ  is  firm.  It  is  often 
also  more  deeply  pigmented  than  normal.  Microscopically,  the  con- 
nective tissue  is  increased.  It  is  questionable  whether  this  is  not  an 
apparent  increase  due  to  the  paucity  of  liver  cells,  rather  than  a  true 
hyperplasia.  It  is  not  to  be  denied,  however,  that  the  disappearance  of 
the  liver  parenchyma  may  be  accompanied  by  a  compensatory  new- 

1  The  relationship  of  perihepatitis  to  cirrhosis  is  discussed  at  some  length  by 
Nicholls,  Studies  from  the  Royal  Victoria  Hospital,  Montreal,  1 : 3: 1902:  60. 


474  THE  LIVER 

growth  of  connective  tissue,  an  example  of  what  one  of  us  (J.  G.  A.) 
has  elsewhere  termed  "  replacement  fibrosis."  If  this  be  true,  the 
condition  might  be  termed  an  interstitial  fibrosis  or  cirrhosis,  though  it 
should  be  remembered  that  it  is  primarily  of  a  degenerative  rather 
than  an  inflammatory  origin.  The  atrophy  in  such  cases  may  possibly 
be  referred  to  lack  of  nutrition,  due  to  thickening  of  the  branches  of  the 
hepatic  artery.  It  must  be  admitted,  however,  that  we  have  practically 
no  anatomical  evidence  in  favor  of  an  arteriosclerotic  cirrhosis  of  the 
liver  per  se. 

Cirrhosis  from  Passive  Congestion  (Cirrhose  Cardiaque). — This  is  a  form 
of  cirrhosis  described  mainly  by  the  French  school  of  pathologists. 
The  Germans  in  general  are  disposed  to  deny  its  existence.  It  is  a 
fact,  however,  that  in  moderately  advanced  and  long-continued  cases 
of  passive  congestion  of  the  liver,  associated  more  especially  with  cardiac 
weakness,  there  may  be  a  certain  amount  of  fibrosis  in  the  neighborhood 
of  the  centrilobular  vein,  following  a  preliminary  atrophy  of  the  liver 
parenchyma  of  these  parts.  This  is,  in  our  experience,  a  rare  occur- 
rence, but  unquestionably  does  occur.  It  may  well  be  doubted,  however, 
whether  such  a  fibrosis  is  ever  sufficiently  extensive  to  give  rise  to  clinical 
symptoms. 

Tuberculosis. — The  occurrence  of  primary  tuberculosis  of  the  liver 
is  doubtful.  Cases  of  this  disease  can  usually  be  traced  to  foci  of  infec- 
tion in  some  other  region  of  the  body.  The  path  of  infection  may  be 
through  the  hepatic  artery,  the  portal  vein,  the  lymphatics,  or  by  con- 
tiguity. 

Anatomically,  three  forms  can  be  recognized — disseminated  miliary 
tuberculosis,  solitary  tuber culomas,  and  chronic  tuberculous  sclerosing 
hepatitis.  If  the  liver  be  fatty,  as  it  so  often  is  in  cases  of  advanced 
tuberculosis,  miliary  tubercles  may  readily  be  overlooked.  When 
visible,  they  are  present  as  minute,  gray,  or  yellowish  dots,  often  slightly 
bile-stained.  The  milia  are  situated  for  the  most  part  in  the  periportal 
connective  tissue  and  may  encroach  to  some  extent  upon  the  lobules. 
Exceptionally,  they  are  found  within  the  lobules.  .The  foci  have  the 
appearance  of  lymphoid  nodules  with  epithelioid  cells  and  possibly  giant 
cells.  Caseation  is  not  uncommon.  In  the  centre  of  the  tubercle  the 
liver  cells  are  destroyed,  while  those  at  the  periphery  show  fatty  changes 
and  atrophy.  Care  should  be  taken  not  to  mistake  collapsed  bile  ducts 
for  giant  cells  in  cases  where  they  have  become  included  within  the 
granuloma,  for  there  is  a  superficial  resemblance  between  the  two  struc- 
tures. In  some  cases,  the  tubercles  are  not  fully  formed,  and  we  find 
merely  a  round-celled  infiltration  in  the  periportal  sheaths  with  some 
cirrhosis  and  the  formation  of  pseudobile  capillaries.  The  cirrhosis 
is  often  of  the  pericellular  type. 

Solitary  tubercles,  usually  few  in  number,  are  occasionally  observed, 
but  are  rare.  They  may  be  of  considerable  size,  are  caseous  in  the  centre, 
and  are  enclosed  in  a  more  or  less  dense  fibrous  capsule.  Occasionally, 
they  soften  or  become  secondarily  infected,  giving  rise  to  abscesses. 

In  the  third  form,  the  liver  is  the  seat  of  a  diffuse  and  extensive  over- 


SYPHILIS 


475 


growth  of  connective  tissue,  in  which  can  be  seen,  here  and  there,  gray 
or  grayish-yellow  tubercles. 

Syphilis. — Both  congenital  and  acquired  syphilis  may  affect  the  liver. 
The  lesions  of  congenital  syphilis  in  the  main  are  comparable  with  those 
of  tuberculosis.  We  can  differentiate  anatomically,  disseminated  miliary 
gummas,  large,  well-formed  gummas,  and  syphilitic  cirrhosis. 

In  the  form  with  numerous  miliary  gummas  the  liver  is  often  enlarged. 
The  lesions  affect  either  the  whole  organ  or  circumscribed  areas  of 
it.  The  milia,  histologically,  are  identical  with  the  milia  of  tuberculosis. 
The  absence  of  the  tubercle  bacillus  in  the  former  is  practically  the 
only  way  to  differentiate  between  them.  There  is  usually  marked 
fibrosis  in  the  neighborhood  of  miliary  gummas  also.  Large  gummas 


FIG.  122 


Gumiria  of  the  liver.     Zeiss  obj.  A,  without  ocular.      The  gumma  is  the  dark  mass  to  the 
right.     (From  the  collection  of  the  Montreal  General  Hospital.) 

are  occasionally  met  with  in  congenital  syphilis,  but  are  more  common 
in  the  acquired  disease.  They  are  of  moderate  size,  as  a  rule,  easily 
visible  to  the  naked  eye,  and  are  scattered  irregularly  through  the  organ. 
They  also  present  a  yellowish  appearance,  with  broken-down  centres, 
usually  surrounded  by  dense,  often  radiating  scars.  In  advanced  cases 
the  liver  is  greatly  deformed  and  divided  into  numerous  lobules  of 
varying  size  by  deep  fissures,  which  represent  contracting  fibrous  cica- 
trices, the  sole  remains  of  the  previously  existing  gummas.  This  gives 
rise  to  the  so-called  hepar  lobatum.  The  comparatively  unaltered  liver 
substance  is  often  of  a  dark  brown  color. 

Histologically,   gummas    closely  resemble    tuberculous  granulomas, 


476  THE  LIVER 

having  a  necrotic  centre,  a  cellular  periphery  with  giant  cells,  and 
being  enclosed  in  a  fibrous  capsule.  The  capsule  is  somewhat  irregular, 
sending  out  processes  into  the  liver  substance  in  the  neighborhood,  and 
contains  collections  of  small,  round  cells,  and,  often,  numerous  pseudo- 
bile  capillaries.  The  liver  cells  proper  usually  show  brown  atrophy, 
and  there  may  be  evidences  of  amyloid  transformation.  In  advanced 
cases  nothing  but  the  scar  is  visible,  the  necrotic  material  having  been 
completely  absorbed. 

In  infants  a  diffuse  cirrhosis  is  the  common  lesion  of  hepatic  syphilis, 
and  is  of  the  pericellular  and  intra-acinous  type.  In  the  early  stages,  there 
is  a  cellular  infiltration  of  the  interlobular  and  intralobular  connective 
tissue.  This  may  be  observed  throughout  the  organ  as  a  whole,  or, 
again,  may  be  restricted  to  scattered  areas.  The  liver  is  somewhat 
enlarged,  hard,  and  the  capsule  is  smooth  or,  exceptionally,  finely 
granular.  On  section,  the  color  is  yellow,  yellowish  or  reddish-brown, 
somewhat  recalling  the  appearance  of  flint.  The  lobules  are  not  readily 
recognized.  Histologically,  there  is  a  periportal  proliferation  of  con- 
nective tissue,  which  tends  to  invade  the  lobules,  destroying  their  sub- 
stance, and  containing  numerous  foci  of  round  cells  with  pseudobile 
capillaries.  The  newly-formed  connective  tissue  is  delicate  and  trans- 
lucent, containing  relatively  few  nuclei,  and  is  laid  down  diffusely,  insinu- 
ating its  way  between  the  liver  cells,  so  that  they  are  found  isolated  one 
from  the  other,  or  in  little  groups  of  twos  and  threes.  There  is,  apparently, 
not  much  tendency  to  contraction  in  this  form.  Hoffmann  and  others 
have  demonstrated  the  presence  of  the  spirocheta  pallida  in  the  livers 
of  children  dying  of  congenital  syphilis. 

The  manifestations  of  acquired  syphilis  in  the  liver  differ  but  little, 
on  the  whole,  from  those  of  the  congenital  form.  Any  differences  are 
probably  to  be  explained  on  the  basis  of  time  and  the  difference  in  the 
reactive  powers  of  the  liver  at  the  different  life  periods. 

The  condition  of  the  liver  in  the  secondary  stage  is,  of  course,  difficult 
to  ascertain.  The  occurrence  of  jaundice  and  hepatic  enlargement 
at  this  period  suggests  the  presence  of  an  acute  interstitial  hepatitis, 
perhaps  associated  with  a  cholangitis,  or  even  a  more  severe  affection, 
an  acute  parenchymatous  hepatitis,  closely  resembling  acute  yellow 
atrophy. 

The  common  type  in  the  tertiary  stage  is  the  multiple  gumma  with 
cirrhosis.  This  is  often  associated  with  jaundice  and  gives  rise  to  a 
nodular  or  fissured,  contracted  liver,  which  may  be  bile-stained.  Occa- 
sionally, the  gummas  are  so  large  as  to  be  mistaken  for  malignant 
growths.  In  such  cases  there  is  a  dense  mass  of  fibrous  tissue,  with 
more  or  less  gummy  degeneration,  surrounded  by  an  outer  layer  of 
liver  tissue,  which  is  infiltrated  with  round  cells.  These  seem  to  be 
derived  from  isolated  gummas,  presenting  a  reactive  new-formation  of 
liver  substance  at  the  periphery,  which  is  dotted  with  numerous  miliary 
gummas  and  eventually  destroyed  and  replaced  by  fibrous  tissue. 

Actinomycosis. — Actinomycosis  of  the  liver  may  be  primary  or 
secondary.  There  are  but  few  undoubted  cases  of  the  primary  disease 


PARASITES 


477 


FIG.  123 


on  record.1     The  infection  is  generally  thought  to  be  from  the  intestine 
through  the  portal  system.     Less  often  it  is  from  the  kidney  or  lung. 

The  lesion  takes  the  form  of  more  or  less  numerous  abscesses,  which 
often  become  aggregated  into  lobular  areas,  owing  to  the  coalescence  of 
smaller  foci.  With  this  there  is  a  proliferation  of  connective  tissue, 
giving  the  larger  abscesses  a  curious  and  characteristic  appearance. 
After  the  necrotic  material  is  washed  out,  the  larger  cavities  can  be  seen 
to  be  traversed  in  all  directions 
by  rather  coarse  communicating 
bands  of  connective  tissue,  so  that 
an  appearance  rather  like  that  of 
a  loofah  sponge  is  produced.  The 
specific  ray-fungus  can  be  recog- 
nized in  the  tissues  and  in  the 
necrotic  and  liquefied  material. 
The  liver  becomes  adherent  to  the 
diaphragm,  abdominal  wall,  or  a 
neighboring  viscus,  and  fistulous 
communications  may  be  opened 
with  the  exterior  or  some  organ. 
Discharge  of  the  abscesses  into 
the  lung  is  perhaps  the  most,  fre- 
quent occurrence.  The  infection 
in  some  cases  becomes  fairly  gen- 
eralized. In  one  case  which  we 
had  the  opportunity  of  sectioning, 
the  liver  was  adherent  to  the  dia- 
phragm and  the  right  lobe  was 
occupied  by  a  very  large  trabecular 
abscess  of  the  type  just  described. 
Multiple  small  abscesses,  resem- 
bling those  found  in  pyemia,  were 
found  in  the  lungs  and  kidneys,  in 
which  the  specific  organism  was 
detected. 

Parasites.  —  The  most  import- 
ant are  the  Echinococcus,  the  Pen- 
tastomum  denticulatum,  and  the 
Distoma  hepaticum.  Echinococcus 

disease  is  not  so  frequently  met  with  in  America  as  in  Europe.  It  is  occa- 
sionally found,  however,  among  the  Mennonites  of  Manitoba  and  the 
Northwest.  Echinococcus  cysts  are  unilocular  or  multilocular,  and  are 
bounded  by  a  laminated  connective-tissue  capsule.  The  liver  cells  in 
the  immediate  neighborhood  of  the  cysts  are  flattened  from  pressure. 
Within  the  fibrous  capsule  is  the  gelatinous,  transparent,  echinococcus 


Echinococcus  cysts  of  the  liver  in  a  hog. 
(From  the  Pathological  Museum  of  McGill 
University.) 


1  See  Moser,  New  York  Med.  Journ.,  60:  1894:  176,  and  Auvray,  Rev.  de  Chir., 
28:1903:  1. 


478  THE  LIVER 

membrane,  which,  under  the  microscope  is  of  hyaline  appearance,  with 
a  characteristic  concentric  lamination.  The  contained  fluid  is  clear, 
watery,  or  slightly  tinted.  It  may  contain  numerous  small  bladders  or 
daughter-cysts.  Owing  to  absorption  of  the  fluid  and  inspissation  of  the 
contents,  the  cysts  may  contain  a  caseous,  gritty  substance,  resembling 
mortar. 

RETROGRESSIVE  METAMORPHOSES. 

Simple  Atrophy. — Simple  atrophy  of  the  liver,  the  result  of  mal- 
nutrition, is  met  with  in  marasmus,  old  age,  and  cachexia.  The  form 
due  to  pressure  has  already  been  referred  to.  Carcinoma  of  the  oesopha- 
gus and  stomach,  by  inducing  starvation,  has  a  special  tendency  to 
cause  atrophy  of  the  liver.  In  such  cases  the  diminution  in  size  may 
be  very  marked,  the  organ  in  some  instances  being  only  one-third  of  the 
normal  weight.  The  atrophy  affects  the  organ  as  a  whole,  but  the  ante- 
rior border  suffers  most.  The  liver,  therefore,  is  small,  the  edges  are 
sharp,  and  the  capsule  wrinkled.  The  gall-bladder  sometimes  projects 
considerably  beyond  the  liver  margin.  In  advanced  cases,  little  may 
be  left  at  the  borders  but  the  connective-tissue  capsule,  which  is  recog- 
nizable as  a  thin,  semitransparent  membrane  containing  bloodvessels. 
The  liver-substance  remaining  may  be  firmer  than  normal,  owing  to  the 
relative  increase  of  the  connective  tissue. 

Histologically,  the  parenchymatous  cells  are  shrunken,  stain  rather 
badly,  and  are  somewhat  pigmented  (brown  atrophy).  The  fibrous  tissue 
appears  to  be  increased,  but  this  appearance  is  largely  due  to  the  dis- 
appearance of  the  liver  cells  proper.  Frerichs  has  called  attention  to  a 
form  of  atrophy — melanemic  atrophy — believed  to  be  due  to  blocking  of 
the  capillaries  with  black  pigment.  Atrophy  of  the  liver  is  a  marked 
feature  in  the  disease  known  as  acute  yellow  atrophy  (q.  v.). 

Cloudy  Swelling. — Cloudy  swelling,  a  condition  hardly  distinguish- 
able from  acute  parenchymatous  hepatitis,  is  a  common  occurrence  in 
the  course  of  the  infective  fevers,  particularly  typhoid  and  scarlatina. 
It  may  also  be  due  to  the  action  of  toxins,  apart  from  elevation  of  tem- 
perature. A  liver  so  affected  is  somewhat  moist  on  section  and  the 
lobules  are  no  longer  distinguishable,  the  cut  surface  having  a  peculiar 
glassy  appearance,  as  if  smeared  with  thin  glue.  It  is  surprising, 
however,  how  little  change  can  be  recognized  microscopically,  the  cells 
being  at  most  a  little  swollen  and  rounded,  while  the  nuclei  tend  to 
stain  badly. 

Fatty  Liver. — Fatty  degeneration  of  the  liver  is  a  common  sequel 
of  cloudy  swelling  and  occurs  in  the  course  of  severe  anemias,  such  as 
pernicious  anemia,  in  acute  yellow  atrophy  of  the  liver,  from  congestion, 
and  from  the  action  of  various  toxic  substances,  notably  phosphorus. 
In  advanced  cases  the  liver  is  diminished  in  size,  it  feels  doughy  under 
the  fingers,  and  the  capsule  is  thrown  into  folds.  The  color  is  bright 
yellow  or  yellowish-brown.  On  section,  the  tissue  is  soft  and  friable, 
and  fat  droplets  can  be  scraped  off  the  surface  with  the  knife. 


AMYLOID  OR  LARDACEOUS  TRANSFORMATION  479 

Histologically,  the  liver  cells,  particularly  those  of  the  centre  and  middle 
zone  of  the  lobules,  contain  a  variable  number  of  small  clear  vesicles,  which 
represent  the  spaces  occupied  by  the  fat  when  the  tissue  was  in  the  fresh 
state.  Material  hardened  in  formalin  and  cut  on  the  freezing  microtome 
may  be  stained  by  Sudan  III,  a  selective  stain  for  fat.  The  parenchy- 
matous  cells  appear  then  to  be  filled  with  globules  of  a  carmine-red  or 
yellow  color.  In  the  most  advanced  condition,  it  may  be  hard  to  recog- 
nize any  normal  liver  substance  except  the  portal  sheaths.  Slight  grades 
of  fatty  degeneration  can  be  seen  in  the  centre  of  the  lobules  in  cases  of 
passive  congestion  of  the  liver,  and  in  the  neighborhood  of  inflammatory 
foci  and  new-growths. 

Not  unlike  fatty  degeneration  in  many  ways  is  fatty  infiltration.  The 
liver  is  normally  a  storehouse  for  fat,  especially  that  taken  into  the 
economy  in  the  way  of  food,  or  elaborated  from  other  nutritive  substances. 
A  certain  amount  of  fat,  therefore,  can  ordinarily  be  observed  in  every 
liver  section,  even  under  normal  conditions,  and,  indeed,  after  a  meal  the 
amount  may  be  quite  noteworthy.  An  excessive  amount  of  fat  may, 
however,  be  deposited  in  the  liver  cells  in  cases  where  an  excess  of 
fat  or  of  its  precursors  has  been  ingested,  or  where  there  is  a  lessened 
oxidation  of  the  fat  ordinarily  supplied.  The  latter  occurrence  is  seen 
in  long-standing  and  wasting  diseases,  such  as  tuberculosis,  anemias, 
and  various  cachexias.  The  use  of  alcohol  is  especially  prone  to  induce 
this  form  of  fatty  liver 

Macroscopically,  the  liver  is  enlarged  and  its  weight  increased.  Its 
specific  gravity,  however,  is  diminished,  and  may  be  reduced  to  such 
an  extent  that  the  organ  will  float  in  water.  The  edges  are  rounded,  the 
capsule  smooth  and  tense,  and  the  surface  can  readily  be  indented.  The 
color  is  yellowish-brown  or  bright  yellow  according  to  the  amount  of 
fat  present.  On  section,  the  organ  is  pale,  yellow,  bloodless,  and  dis- 
tinctly unctuous. 

Histologically,  the  appearances  are  much  the  same  as  in  fatty  de- 
generation, and,  in  fact,  in  advanced  cases  one  condition  can  hardly 
be  distinguished  from  the  other.  In  fatty  degeneration  the  tendency  is 
for  small  droplets  of  fat  to  form,  which  ultimately  coalesce  and  increase 
in  size  until  they  fill  the  cell.  In  fatty  infiltration  the  globules  are  apt 
to  be  more  uniformly  large,  and  are  usually  situated  at  the  periphery 
of  the  lobules.  The  nucleus  is  pushed  to  one  side  so  that  the  liver  cell 
involved  is  swollen,  rounded,  and  presents  a  signet-ring  appearance. 
The  nucleus  usually  stains  well.  A  point  of  some  importance  in  differ- 
entiating the  two  conditions  is  that  in  fatty  degeneration  the  compara- 
tively unaffected  cells  show  cloudy  and  other  degenerative  changes, 
while  in  fatty  infiltration  the  cells  devoid  of  fat  present  a  practically 
normal  appearance.  In  the  former,  too,  the  nucleus  shows  degenerative 
changes  as  well  as  the  cytoplasm. 

Amyloid  or  Lardaceous  Transformation. — Amyloid  or  lardaceous 
transformation  of  the  liver  occurs  under  the  same  circumstances  as 
amyloid  disease  elsewhere,  that  is  to  say,  in  connection  with  suppuration 
and  prolonged  wasting  disease. 


480  THE  LIVER 

The  organ  is  enlarged,  its  edges  are  rounded,  and  its  consistence  is 
increased,  so  that  it  has  a  somewhat  elastic  or  rubbery  feel.  It  does  not 
lie  flat  on  the  table  and  looks  as  if  forced  into  a  capsule  that  was  too 
small  for  it.  On  section,  the  cut  surface,  if  the  amyloid  change  be 
extensive,  looks  as  if  it  were  covered  with  a  thin  layer  of  gelatin, 
and  the  edges  are  translucent  when  held  up  to  the  light.  The  amyloid 
masses  can  be  recognized  by  their  semitranslucent  glue-like  appearance. 
The  remaining  liver  substance  varies  in  appearance  according  to  the 
amount  of  fat  present  and  the  degree  of  congestion.  The  gall-bladder 
usually  contains  clear,  thin  bile. 

Microscopically,  in  a  moderately  advanced  case,  the  amyloid  material 
is  found  to  occupy  by  preference  the  intermediate  zone  of  the  lobules, 
the  centre  and  periphery  being  free.  The  substance  appears  to  be  of 
the  nature  of  a  deposit  and  is  laid  down  in  glistening  scales  in  the  walls 
of  the  capillaries  beneath  the  endothelium.  In  advanced  cases  the  larger 
arterioles  of  the  portal  districts  are  affected,  particularly  in  their  middle 
coats.  The  parenchymatous  cells,  lying  between  the  thickened  capil- 
laries, present  various  stages  of  fatty  degeneration  and  atrophy. 

Pigmentary  Infiltration. — When  we  examine  sections  of  liver  under 
a  high  power,  we  find  normally  a  certain  amount  of  golden-yellow 
pigment  in  the  form  of  granules  within  the  secreting  cells.  This  may 
be  the  natural  coloring  matter  of  the  liver  cells,  bile  pigment,  or,  as  one 
of  us  (J.  G.  A.1)  has  suggested,  bacteria  in  a  dead  or  dying  condition  which 
have  become  bile-stained. 

Under  pathological  conditions,  the  pigment  of  the  liver  may  be 
much  increased  and  may  be  of  very  varying  nature.  In  icterus  or 
jaundice  the  liver  may  be  stained  a  bright  yellow  or  green.  This 
coloration  is  due  to  the  retention  of  bile  and  its  passage  out  of  the 
bile  capillaries,  owing  to  some  obstruction  to  the  free  discharge  of  the 
secretion.  Cholelithiasis,  cholangitis,  tumors,  and  aneurisms  pressing 
upon  the  bile  ducts  are  among  the  main  conditions  to  be  mentioned  in 
this  connection.  Icterus  of  the  liver  also  occurs  in  some  forms  of  cirrhosis, 
in  severe  blood  destruction,  and  in  acute  yellow  atrophy  of  the  liver. 
In  a  well-marked  case  of  icterus,  the  finer  bile  ducts  on  section  are  found 
to  be  distended  and  bile  flows  from  them  readily,  while  the  central  portion 
of  the  lobule  shows  a  diffuse  bile  staining.  Microscopically,  the  liver 
cells  are  of  a  diffuse  bright  yellow  color  or  contain  yellowish  or  yellowish- 
brown  granules,  more  rarely  a  crystalline  deposit.  According  to 
Nauwerck  and  Fiitterer,  dilatation  of  the  minute  intracellular  passages 
is  to  be  observed.  The  pigment  in  question  is  called  bilirubin,  and  is 
believed  to  be  identical  chemically  with  hematoidin. 

Another  important  class  of  pigments  found  in  the  liver  is  that  due  to 
disintegration  of  the  red  blood-corpuscles.  In  such  cases  the  hemoglobin 
is  set  free  and  carried  to  the  liver,  where  it  is  metamorphosed  in  different 
ways.  Two  main  forms  of  pigment  may  be  mentioned,  hematoidin 
and  hemosiderin.  The  principal  point  of  difference  between  the  two  is 

1  Lancet,  2: 1898;  Montreal  Med.  Journ.,  27: 1898:898. 


NECROSIS  481 

that  the  latter  contains  iron.  Should  the  blood  destruction  be  moderate 
in  degree,  the  liver  appears  to  be  able  to  cope  with  the  condition  to  some 
extent,  so  that  the  hematoidin  is  excreted  in  the  bile,  while  the  hemosiderin 
is  retained.  In  fact,  a  minute  quantity  of  iron  can  be  detected  even  in 
normal  livers.  A  pathological  increase  of  pigmentation  is  met  with  in 
all  cases  of  advanced  passive  congestion  of  the  liver. 

Hemosiderosis. — Hemosiderosis  in  the  liver  results  in  all  cases  where 
there  is  extensive  destruction  of  the  red  corpuscles  of  the  blood,  such 
as  occurs  in  severe  sepsis,  pernicious  anemia,  malaria,  hemoglobinemia, 
in  certain  cases  of  cirrhosis  of  the  liver,  severe  burns,  and  in  various 
intoxications.  The  iron-containing  pigment  is  deposited  in  the  liver 
cells,  particularly  at  the  periphery  of  the  lobules,  but  also  in  the  tissue 
spaces.  The  pigment  is  of  a  golden  color  and  may  be  differentiated  from 
bilirubin  by  the  fact  that  when  sections  are  treated  with  hydrochloric 
acid  and  potassium  ferrocyanide  the  Prussian  blue  reaction  is  obtained 
(Perls'  test).  In  exceptional  instances,  hemosiderin  is  found  in  the 
skin,  mucous  membranes,  the  intestinal  wall,  the  pancreas,  spleen,  and 
other  organs  as  well  as  in  the  liver.  This  condition  has  been  termed 
hemochromatosis  (v.  Recklinghausen)  and  has  been  studied  in  this 
continent  especially  by  Opie1  and  Maude  Abbott.2  Many  cases  are 
associated  with  cirrhosis  of  the  liver  (cirrhose  pigmentaire)  or  pancreas 
(diabete  bronze),  or  both. 

In  malaria,  the  pigment  found  in  the  liver  and  other  organs  is,  in 
part,  hemosiderin  and,  in  part,  melanin,  the  latter  a  pigment  elaborated 
by  the  protozoon  that  causes  the  disease. 

Other  pigments  found  in  the  liver  are  of  extraneous  origin,  as,  for 
example,  coal  dust  and  silver  (argyria).  In  some  cases  the  amount  of 
anthracotic  pigment  taken  into  the  system  has  been  excessive  and  has 
reached  the  general  circulation,  some  of  it,  therefore,  being  deposited  in 
the  liver,  or,  an  anthracotic  lymph-node  may  soften  and  rupture  into  a 
vein.  Welch  has  reported  a  case  of  cirrhosis  of  the  liver  due  to  coal 
pigment.  One  of  us  (J.  G.  A.)  has  met  with  a  case  of  silicosis  of  the 
liver.3 

Necrosis. — Necrosis  of  the  type  commonly  known  as  coagulation 
necrosis  is  not  uncommon  in  the  liver.  Large  areas  of  a  pale  yellowish 
color  and  opaque  appearance  are  frequently  to  be  observed  in  connection 
with  septic  processes  within  the  district  drained  by  the  portal  system, 
notably  in  cases  of  appendicitis.  They  are  found  also  in  eclampsia  and 
acute  yellow  atrophy.  Less  extensive  necrosis  is  seen  in  the  various 
infective  fevers,  typhoid,  diphtheria,  variola,  cholera,  glanders  and 
septicemia,  and  as  a  result  of  various  intoxications.  Sometimes  multiple 
small  areas  of  necrosis — focal  necroses — are  met  with,  which  are  visible  to 
the  naked  eye  as  grayish  or  opaque  yellow  dots  the  size  of  a  pin-head  or 
less.  These  are  small  areas  of  necrosis  of  the  liver  cells,  or  perivascular 

1  Journ.  of  Exper.  Med.,  4:  1899:279. 

2  Journ.  of  Pathology,  6:  1900:  315. 

3  Adami,  Sajous'  Encyclopedia,  article  Cirrhosis. 
31 


482  THE  LIVER 

collections  of  small  round  cells  resembling  lymph-follicles.  The  death 
of  the  cells  appears  to  be  due  partly  to  the  direct  action  of  the  toxin 
and  partly  to  obstructive  conditions  within  the  capillaries  (see  vol.  i, 
p.  901). 

FIG.  124 


Multiple  focal  necroses  in  the  liver  of  a  rabbit  subjected  to  experimental  glanders.     (Duval.) 


PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — The  liver  may  be  enlarged  in  a  variety  of  conditions, 
such  as  passive  congestion,  inflammatory  infiltration,  cysts,  and  tumors. 
Every  increase  in  size,  therefore,  is  not  to  be  regarded  as  hypertrophy. 
The  term  hypertrophy,  strictly  speaking,  applies  to  the  enlargement  of 
individual  cells,  and  this  is  seen  to  a  limited  extent  in  many  reparative 
processes  going  on  in  the  liver,  but  the  condition  is  practically  always 
combined  with  an  increase  in  the  number  of  the  cells — numerical  hyper- 
trophy or  hyperplasia. 

Hypertrophy  of  the  liver  in  this  sense  is  essentially  a  compensatory 
process,  and,  moreover,  local.  The  existence  of  a  generalized  hyper- 
trophy is  doubtful.  The  liver  is  an  organ  that  exhibits  under  certain 
circumstances  a  striking  degree  the  power  of  repair.  According  to 
Ponfick  and  von  Meister,  one-half  to  three-quarters  of  the  liver  can  be 
removed,  and,  while  the  original  shape  is  not  restored,  the  remaining 
substance  undergoes  compensatory  hyperplasia.  The  preexisting 
lobules  become  greatly  enlarged,  there  being,  however,  but  little  new 
formation  of  parts.  Regeneration  of  this  order  is  well  seen  in  cases 
where  portions  of  the  liver  are  destroyed,  as  by  traumatism,  pressure, 
or  various  degenerative  processes,  for  example,  in  advanced  passive 


TUMORS  483 

congestion,  cirrhosis,  acute  yellow  atrophy,  and  in  the  necrosis  resulting 
from  certain  infective  fevers.  The  nodular  projections  found  in  what 
is  often  spoken  of  as  the  "hob-nail"  liver  are  to  be  regarded  as  local 
attempts  at  regeneration,  rather  than  as  the  effect  of  the  contraction  of 
the  newly-formed  connective  tissue,  an  explanation  that  has  often  been 
advanced.  The  proliferating  liver  cells  are  recognized  by  the  fact  that 
they  are  larger  than  normal,  the  cytoplasm  stains  deeply,  the  nucleus 
is  large,  rich  in  chromatin,  and  often  shows  evidences  of  mitosis.  The 
regular  arrangement  of  the  liver  lobules  is,  however,  not  altogether 
reproduced,  with  the  possible  exception  of  acute  yellow  atrophy  where 
the  new  cells  grow  into  the  stroma  and  replace  those  that  have  under- 
gone necrosis. 

Tumors. — The  new-growths  found  in  the  liver  may  be  primary  or 
secondary,  and  are  of  connective-tissue  or  epithelial  type.  Primary 
tumors  of  all  kinds  are  rare.  The  primary  benign  growths  are  the 
angioma,  cavernoma,  adenoma,  fibroma,  myxoma,  and  lipoma. 

The  cavernoma  is  of  relatively  frequent  occurrence  and  usually  is  met 
with  in  the  shrunken  livers  of  elderly  people.  It  is  not  strictly  a  new- 
growth,  as  a  rule,  and  is  rarely  of  large  size.  It  is  recognized  by  its  dark 


FIG.  125 


Cavernoma  of  liver.     Gross  appearance.     (After  Ribbert.) 

brown  or  purplish-red  color  and  is  sharply  defined  from  the  liver  sub- 
stance. It  may  be  surrounded  by  a  fibrous  capsule.  When  cut  into, 
blood  can  be  squeezed  out,  leaving  a  spongy  network  of  connective  tissue, 
sometimes  containing  bands  of  muscle  fibers.  The  tumor  is  formed  by 
a  relatively  enormous  dilatation  of  the  capillaries  which  results  in  atrophy 
of  the  intervening  liver  cells.  Proliferation  of  the  vessel  walls  and  the 
fibrous  stroma  can  be  made  out  in  some  few  cases,  showing  the  formation 
of  a  true  hemangioma.  The  vascular  spaces  can  be  injected  from  the 
portal  vein  or  the  hepatic  artery.  Thrombosis  is  frequently  observed  in 
the  enlarged  vessels  with  consecutive  organization  of  the  clot.  Lymph- 
angioma  has  been  described  by  Klebs. 

Adenoma  occurs  in  the  form  of  single  or  multiple  nodules  of  a  grayish, 
yellowish-white,  or  reddish-brown  color.  These,  microscopically,  con- 
sist of  branched  and  convoluted  gland-tubules,  or  of  bands  and  clumps 
of  liver  cells,  which,  however,  are  not  arranged  after  the  fashion  of  the 
normal  lobules.  Some  of  the  tubules  may  possess  a  small  lumen. 
Certain  authors  consider  that  adenomas  are  derived  both  from  the 
epithelium  of  the  bile  ducts  and  from  the  liver  cells  proper,  although 
Rindfleisch  holds  to  the  former  view.  Owing  to  dilatation  of  the  tubules, 


484  THE  LIVER 

we  may  get  an  adenocystoma  consisting  of  more  or  less  numerous  cysts 
or  groups  of  cysts  rilled  with  clear  fluid.  Such  tumors  appear  to  be 
developed  from  outgrowths  of  the  bile  ducts  in  the  periportal  connective 
tissue. 

We  have  recently  met  with  a  case  of  lipoma.  The  growth  was  the 
size  of  a  green  pea,  situated  on  the  dome  of  the  liver,  and  was  well 
encapsulated. 

FIG.  126 


-*•       m 


-Jg^L 


Cavernous  angioma  of  the  liver.  Winckel  No.  3,  without  ocular.  Normal  liver  tissue  is 
shown  above  and  to  the  left.  The  fibrous  trabeculse  of  the  blood  tumor  are  well  seen.  (From 
Dr.  A.  G.  Nicholls'  collection.) 

Primary  sarcoma  is  excessively  rare  in  the  liver.  It  occurs  as  a  single 
large  mass  or  as  multiple  circumscribed  nodules.  Round,  spindle- 
celled,  and  melanotic  forms  are  described.  They  seem  to  begin  in  the 
neighborhood  of  vessels.  Ford1  has  recorded  a  case  of  primary  sarcoma 
occurring  in  a  cirrhotic  liver. 

In  lymphosarcoma  a  large  number  of  lymphoid  cells  are  found  in  the 
portal  sheaths  and  in  the  intralobular  capillaries,  while,  in  addition, 
lymphomatous  nodules  are  scattered  throughout  the  organ. 

Secondary  sarcomas  are  not  uncommon  in  the  liver,  and  are  of  all 
types.  The  pigmented  forms  are,  however,  the  most  important.  With 
the  exception  of  the  lung,  the  liver  is  the  most  frequent  seat  of  metas- 
tatic  deposit  in  the  case  of  the  melanotic  new-growths.  We  get  then 
either  circumscribed  pigmented  nodules,  or  a  diffuse  infiltration,  in  the 
form  of  yellow,  gray,  or  blackish-brown  streaks,  which  give  the  liver 
an  appearance  somewhat  like  granite  on  section. 

1  Amer.  Journ.  Med.  Sci.f  120:  1900:  413. 


CARCINOMA  485 

Mixed  forms,  fibrosarcoma  and  myosarcoma,  are  also  described. 

Block  has  described  a  melanotic  endothelioma. 

Hypernephromas  are  occasionally  met  with  in  the  liver. 

Carcinoma. — By  far  the  most  important  and  frequent  tumor  of  the  liver 
is  the  carcinoma,  which  may  be  primary  or  secondary.  The  com- 
parative frequency  of  the  two  forms  may  be  gathered  from  Hale  White's 
statistics.  In  10,000  autopsies  he  found  10  cases  of  primary  carcinoma 
and  240  of  secondary.  Primary  carcinoma  seems,  therefore,  to  be  de- 
cidedly rare. 

Three  main  types  of  primary  cancer  are  described.  In  the  first — 
cancer  massif — there  is  usually  a  single  large  mass  of  new-growth  occupy- 
ing the  greater  part  of  a  lobe,  usually  the  right.  Not  infrequently,  in  the 

FIG.  127 


Liver.     Lymphosarcoma  nodule.     Zeiss  obj.  A,  without  ocular.     (From  the  collection  ot 

Dr.  A.  G.  Nicholls.) 

neighborhood  of  this  are  to  be  seen  a  few  isolated  nodules  representing 
local  metastases.  The  tumor  is  of  somewhat  firm  consistency  and  of  a 
whitish  or  whitish- yellow  color,  occasionally  somewhat  reddened.  Com- 
paratively little  cancer  juice  can  be  scraped  away.  As  a  rule,  the  mass 
is  sharply  differentiated  from  the  liver  substance,  but  at  parts  of  the 
periphery  some  infiltration  can  be  made  out.  The  larger  nodules  of  cancer 
often  present  degeneration  and  softening  at  the  centre,  and,  if  situated 
on  the  surface  of  the  liver,  may  show  umbilication.  General  metastasis 
is  not  common.  The  liver  substance  in  the  immediate  neighborhood  is 
compressed,  atrophic,  and  the  vessels  are  often  occluded. 

The  second  form  simulates  a  nodular  cirrhosis — the  infiltrating  car- 
cinomatous  cirrhosis  of  Perls.     The  liver  is  more  or  less  enlarged,  the 


486 


THE  LIVER 


capsule  thickened,  and  the  surface  warty.  On  section,  numerous 
bands  of  connective  tissue  are  to  be  seen,  in  which  a  few  islets  of  liver 
substance  still  remain,  but  which  contain  nodules  of  cancer,  the  size  of 
a  pea  or  larger,  of  a  whitish  or  pale  red  color,  and  of  soft,  juicy  consist- 
ency. Where  the  nodules  have  originated  in  a  preexisting  adenomatous 
new-growth  they  are  of  a  grayish-brown  color,  firmer,  and  not  so  juicy. 
Invasion  of  the  portal  vein  and  liver  capillaries  is  not  uncommon. 


FIG.  128 


Primary  carcinoma  of  the  liver.     (From  the  Pathological  Department  of  the  Royal 
Victoria  Hospital.     Case  of  Drs.  C.  F.  Martin  and  W.  F.  Hamilton.) 


In  the  third,  a  rare  form,  there  is  a  carcinomatous  infiltration  of  the 
Glisson's  capsule  originating  from  the  larger  bile  ducts.  The  nodules 
of  new-growth  in  the  portal  districts  are  closely  packed  and  often  fused, 
gradually  diminishing  in  size  as  one  passes  from  the  hilus  to  the  peripheral 
portions  of  the  liver.  Icterus  is  common  in  this  form,  owing  to  compres- 
sion and  obstruction  of  the  bile  ducts. 

Carcinomas  of  the  liver  originate  either  from  the  specific  parenchyma 
or  from  the  epithelium  of  the  bile  ducts.  According  to  their  histological 
structure,  we  can  divide  them  into  the  cylindrical-celled  adenocarcinoma, 
medullary,  and  scirrhous  forms.  The  carcinoma  with  cirrhosis,  accord- 
ing to  several  observers  (Hanot,  Frohmann,  Ziegler),  is  developed  in 
a  cirrhotic  liver  by  atypical  proliferation  of  the  newly-formed  liver 
cells. 

Secondary  carcinomas  of  the  liver  arise  by  direct  extension  or  from 
metastasis.  As  the  growth  in  the  liver  may  attain  to  a  considerable 


CARCINOMA 
FIG.  129 


487 


Secondary  carcinoma  of  the  liver:  medullary  form.  The  necrosis  and  softening  of  the  cancer 
masses  is  indicated  by  slight  depression  (umbilication)  of  their  centres.  (From  the  Pathological 
Museum  of  McGill  University.) 


FIG.  130 


Secondary  carcinoma  of  the  liver.     Winckel  obj.  No.  3,  without  ocular.     (From  the 
collection  of  Dr.  A.  G.  Nicholls.) 


488  THE  LIVER 

size  and  may  dominate  the  clinical  picture,  it  is  sometimes  difficult  if  not 
impossible  to  discover  the  primary  focus.  Carcinoma  of  the  gall- 
bladder and  of  the  bile  ducts  often  spreads  directly  to  the  liver,  but  cancer 
of  the  pyloric  end  of  the  stomach  only  does  so  when  there  has  been  previ- 
ous adhesion  of  the  stomach  to  the  liver.  The  occurrence  of  metastases 
in  the  liver  is  common  in  cancerous  disease,  particularly  so  when  the 
primary  growth  is  in  the  gastro-intestinal  tract,  pancreas,  oesophagus, 
uterus,  or  larynx.  They  arise  from  small  clusters  of  carcinoma  cells, 
which  have  been  broken  off  from  the  main  mass,  and  have  reached 
the  liver  as  emboli  through  the  portal  vein  or  the  general  circulation. 
Wherever  they  lodge  they  proceed  to  grow,  infiltrating  and  destroying  or 
compressing  the  liver  tissue.  At  first  they  take  the  form  of  multiple 
miliary  nodules  scattered  through  the  organ,  but  soon  fuse  into  masses 
the  size  of  the  fist  or  larger.  Where  they  reach  the  surface  they  project 
as  whitish  bosses  covered  with  congested  serosa.  Not  infrequently, 
softening  and  liquefaction  take  place  in  the  larger  nodules,  which  there- 
upon collapse  somewhat,  giving  rise  to  the  characteristic  umbilication, 
The  consistence  of  the  nodes  depends  in  general  upon  the  character  of 
the  original  growth.  The  liver,  as  a  whole,  may  be  enormously  enlarged. 
On  section  the  secondary  masses  are  whitish  in  color,  often  somewhat 
broken  down  in  the  centre.  The  larger  nodules  are  of  an  opaque  yel- 
lowish color  with,  sometimes,  radiating  striae,  the  result  of  degeneration. 
Mucinous,  cystic,  and  calcareous  degeneration  are  common,  as  well  as 
hemorrhagic  infiltration.  Where  the  liver  cells  are  pressed  upon  they 
are  reduced  to  flattened  scales  of  a  brownish  color  (brown  atrophy). 

Cysts. — Apart  from  parasitic  cysts,  blood  cysts,  retention  or  bile  cysts, 
and  congenital  cysts  should  be  mentioned.  Congenital  cystic  disease  of 
the  liver  is  often  associated  with  congenital  cysts  of  the  kidneys,  a  condi- 
tion which  it  closely  resembles. 


CHAPTEE    XXII. 

THE  BILIARY  PASSAGES. 
ALTERATIONS  IN  THE  LUMINA. 

Dilatation. — Dilatation  of  the  bile  ducts  is  due  to  obstruction  to  the 
free  outflow  of  bile.  The  causes  of  this  are  very  varied.  The  chief  that 
may  be  mentioned  are:  swelling  of  the  mucous  membrane;  gallstones, 
parasites,  and  tumors  within  the  ducts;  external  pressure  from  enlarged 
lymph-nodes  or  tumors  involving  the  neighboring  glands,  duodenum, 
liver,  or  pancreas;  inflammatory  adhesions  about  the  ducts;  the  pressure 
of  aneurisms,  a  displaced  kidney  or  liver.  Obstruction  is  one  of  the 
predisposing  causes  of  infection  and  inflammation. 

The  cystic  duct  may  become  occluded,  leading  to  dilatation  of  the 
gall-bladder.  Most  of  the  biliary  substances,  including  the  pigment, 
are  in  time  absorbed,  so  that  the  organ  is  found  to  be  greatly  distended 
with  a  clear,  colorless,  viscid  fluid  something  like  mucin  (hydrops 
vesicoe  felleoi).  Occasionally,  it  is  thin  and  limpid,  like  water.  The  wall 
of  the  bladder  is  usually  thin  and  semitransparent,  unless  thickened  by 
previous  inflammation. 

INFLAMMATIONS. 

Inflammation  of  the  bile  passages  (cholangitis),  or  of  the  gall-bladder 
(cholecystitis),  is  brought  about  by  toxic  or  infective  agents  which  may 
reach  the  liver  in  several  ways.  One  very  frequent  mode  is  for  bacteria 
to  invade  the  passages  from  the  intestine  (ascending  infection).  In  many 
cases  the  organisms  are  excreted  by  the  liver  through  the  bile  (descending 
infection),  as,  for  example,  in  typhoidal  cholangitis  and  cholecystitis. 
Infection  may  also  occur  through  the  blood  stream  (hematogenic  in- 
fection), or  by  the  extension  of  inflammation  from  neighboring  parts. 
The  presence  of  calculi  or  carcinoma  in  the  region,  or  of  parasites  in 
the  biliary  passages,  will  naturally  predispose  to  inflammation  and 
infection,  and  will  aggravate  any  such  conditions  that  previously  exist. 
The  microorganisms  usually  found  are  the  B.  coli,  the  pyogenic  cocci, 
the  Diplococcus  pneumoniae,  and  the  B.  typhi. 

The  type  of  inflammation  is  catarrhal,  purulent,  membranous,  or 
gangrenous. 

Cholangitis. — Acute  Catarrhal  Cholangitis. — Acute  catarrhal  cholan- 
gitis is  a  not  uncommon  affection.  When  secondary  to  gastroduodenitis, 
it  is  the  usual  anatomical  basis  of  the  disease  known  clinically  as  catarrhal 
jaundice.  In  some  cases,  cholelithiasis  and  hydatids  in  the  liver  or  bile 
passages  give  rise  to  this  form  of  cholangitis.  In  most,  if  not  all,  of 


490  THE  BILIARY  PASSAGES 

them  the  infection  is  of  the  ascending  type.  Acute  catarrhal  jaundice 
is  occasionally  met  with  also  in  typhoid  fever,  pneumonia,  secondary 
syphilis,  and  some  other  infectious  diseases. 

As  patients  rarely  die  while  they  are  the  subjects  of  this  condition,  it 
is  rather  difficult  to  say  what  are  the  anatomical  appearances  presented. 
The  mucous  membrane  of  the  common  bile  duct  is  said  to  be  a  little 
swollen,  but  not  particularly  reddened,  and  the  ampulla  of  Vater  is  filled 
with  a  grayish,  slimy,  mucinous  plug,  which  is  sufficient  to  obstruct  the 
free  outflow  of  bile  and  thus  to  produce  jaundice.  The  liver  is  probably 
slightly  enlarged,  the  bile  capillaries  dilated  and  full  of  bile.  Should  the 
process  become  chronic  it  is  apt  to  spread  to  all  .the  bile  passages, 
including  the  gall-bladder.  The  cystic  duct  may  in  time  become 
occluded.  The  walls  of  the  biliary  ducts  finally  become  thickened  and 
the  mucosa  presents  polypoid  outgrowths. 

Suppurative  Cholangitis. — Suppurative  cholangitis,  sometimes  also 
called  phlegmonous  cholangitis,  is  due  to  pyogenic  infection  of  the  biliary 
passages.  It  is  usually  associated  with  or  may  supervene  upon  the  catar- 
rhal form.  The  causes  are  similar  to  those  of  simple  cholangitis. 
Cholelithiasis  and  certain  of  the  infective  processes,  such  as  pyemia, 
typhoid  fever,  pneumonia,  and  influenza,  may  be  mentioned  in  particular. 
Probably  in  most  cases  the  infective  agents  travel  up  from  the  intestine. 
One  case  that  we  have  seen  appeared  to  have  originated  from  a  phleg- 
monous duodenitis.  In  some  instances,  again,  the  infection  is  probably 
hematogenic,  the  pyogenic  microorganisms  finding  in  the  damaged  bile 
passages  a  favorable  situation  for  their  growth. 

Cholecystitis. — Suppurative  Cholecystitis. — The  Suppurative  process 
is  not  uncommonly  restricted  to  the  gall-bladder  (suppurative  chole- 
cystitis). The  wall  of  the  gall-bladder  is  cedematous,  infiltrated  with 
inflammatory  products,  and  more  or  less  distended  with  mucopus  mixed 
with  bile  (empyema  of  the  gall-bladder).  The  organ  is  not  infrequently 
covered  externally  with  a  layer  of  fibrinous  exudate,  and  may  be  adherent 
to  the  neighboring  viscera.  Such  a  condition  may  be  the  starting 
point  of  a  septic  peritonitis.  Especially  is  this  likely  to  occur  if  perfora- 
tion of  the  gall-bladder  have  taken  place.  Fistulous  communications 
with  the  hollow  viscera  or  with  the  exterior  sometimes  result.  Other 
complications  are  multiple  abscesses  in  the  liver  and  generalized 
septicemia. 

The  occurrence  of  suppurative  cholecystitis  in  the  course  of  typhoid 
fever  has  received  considerable  attention.  Chiari  noted  that  the  typhoid 
bacillus  sometimes  persists  in  the  bile  passages  for  months  after  the 
apparent  cure  of  the  typhoidal  attack,  and  recent  observations  have 
proved  that  it  may  remain  even  for  years.  This  fact  is  of  importance 
in  connection  with  the  etiology  of  cholangitis,  cholecystitis,  abscess  of 
the  liver,  and  gallstones.  It  may  possibly  also  explain  some  cases  of 
reinfection. 

Membranous  or  fibrinous  cholecystitis  is  exceedingly  rare.1 

1  Rolleston,  Trans.  Path.  Soc.,  London,  53:1902:405. 


TUMORS  491 

Perichole cystitis  and  Pericholangitis. — Pericholecystitis  and  pericho- 
langitis,  or  inflammation  around  the  gall-bladder  and  bile  passages, 
is  usually  to  be  traced  to  inflammation  of  the  wall  of  these  structures. 
It  may  be  simple,  suppurative,  or  productive. 

Cholelithiasis. — The  term  cholelithiasis  is  applied  to  the  condition 
in  which  calculi  are  found  within  the  bile  passages,  together  with  the 
results  that  spring  from  them.  Biliary  calculi  are  found  more  than 
twice  as  frequently  in  females  as  in  males,  and  usually  after  middle  life. 
The  most  important  single  etiological  factor  is  infection.  In  a  large 
proportion  of  cases  the  B.  coli,  pyogenic  cocci,  and  not  infrequently  the 
B.  typhi  can  be  demonstrated.  Stagnation  of  the  bile  also  assists  by 
leading  to  the  absorption  of  the  alkaline  substances  and  the  production 
of  an  acid  bile,  which  favors  the  growth  of  the  microorganisms  concerned. 
A  slight  catarrh  is  the  result,  leading  to  an  outpouring  of  mucus  in 
which  the  various  pigments  and  salts  are  precipitated  (see  vol.  i,  p.  872). 

The  most  common  sites  for  calculi,  in  order  of  frequency,  are  the  gall- 
bladder, cystic  duct,  cystic  and  common  ducts,  common  duct,  and  hepatic 
duct.  They  are  rare  in  the  intrahepatic  ducts. 

Calculi  in  the  gall-bladder  may  lead  to  catarrhal  or  suppurative  chole- 
cystitis, with  necrosis  and  even  perforation.  In  this  situation  they  are 
often  single,  and  may  be  large  enough  to  fill  up  the  whole  cavity.  Some- 
times they  give  rise  to  little  or  no  disturbance,  and  at  death  the  gall- 
bladder is  found  to  be  thickened  and  contracted  about  the  calculus,  with, 
perhaps,  a  few  adhesions  about  it.  Fistulous  communications  at  times 
occur.  According  to  Courvoisier,  communication  with  the  exterior  is 
the  commonest  event.  Strumpell  and  Murchison,  however,  state  that 
the  cholecysticoduodenal  fistula  is  the  most  frequent.  An  extremely 
rare  form  is  the  cholecysticogastric,  an  example  of  which  one  of  us 
(A.  G.  N.)  has  had  an  opportunity  of  recording.1 

Calculi  in  the  common  or  hepatic  ducts  may  lead  to  complete  obstruc- 
tion, the  production  of  jaundice,  dilatation  of  the  ducts,  inflammatory 
and  productive  changes  in  and  about  the  ducts,  with  also  inflammatory 
and  cirrhotic  changes  in  the  liver  and  pancreas.  Occasionally,  large 
gallstones  may  ulcerate  through  and  become  impacted  in  the  intestine. 
Carcinoma,  usually  of  the  cylindrical-celled  variety,  affecting  the  gall- 
bladder, may  result  from  the  irritation  of  a  calculus.  It  often  spreads 
to  the  liver  by  contiguity. 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — Tumors  of  the  bile  passages  are  most  common  in  the 
vicinity  of  the  ampulla  of  Vater.  Papillomas  and  cystadenomas  are 
described. 

Primary  carcinoma  occurs  most  frequently  near  the  ampulla  or  at 
the  junction  of  the  common  and  cystic  ducts.  It  is  usually  cylin- 

1  Montreal  Med.  Jour.,  27:1898:826. 


492  .  THE  BILIARY  PASSAGES 

drical-celled  in  type.  Secondary  carcinoma  is  due  to  direct  extension  of 
carcinoma  of  the  duodenum,  pancreas,  stomach,  liver,  gall-bladder, 
or  the  neighboring  lymph-nodes.  Duval1  has  reported  an  apparently 
unique  case  of  melanoma  of  Vater's  diverticulum. 

Primary  connective-tissue  tumors  of  the  gall-bladder  are  rare. 
Fibroma,  lipoma,  myxoma,  and  sarcoma  have  been  met  with.  Villous 
papilloma  is  occasionally  found.  The  most  frequent  new-growth  in  this 
situation  is  carcinoma.  It  is  of  the  cylindrical-celled  variety  and  usually 
associated  with  gallstones. 

1  Journ.  of  Exper.  Med.,  10:1908:4. 


CHAPTER    XXIII. 

THE  PANCREAS 
CONGENITAL  ANOMALIES. 

COMPLETE  absence  of  the  pancreas  occurs,  but  only  in  fetuses  that 
present  other  serious  defects.  A  portion  of  the  head  may  be  separate 
from  the  rest,  constituting  a  pancreas  minus,  which  lies  upon  the  anterior 
surface  of  the  duodenum.  The  organ  may  also  be  divided  into  two  equal 
or  unequal  parts  connected  by  the  pancreatic  duct.  The  tail  may  be 
attached  to  the  head  by  a  fibrous  band  containing  the  duct  and  vessels. 
Or,  again,  the  pancreas  may  be  bifid  or  lobulated.  The  duct  of  Wirsung 
may  discharge  into  the  stomach  or  into  some  unusual  part  of  the  intestine. 
Not  infrequently  it  does  not  form  a  junction  with  the  common  bile  duct 
at  the  bile  papilla.  An  interesting,  though  rare,  anomaly  is  the  so-called 
pancreas  annulare,  forming  a  more  or  less  complete  ring  about  the  duo- 
denum, which  may  thereby  be  constricted.  The  most  common  anomaly 
is  the  presence  of  an  accessory  or  aberrant  pancreas.  This  is  usually 
found  in  the  duodenum  or  jejunum,1  less  often  in  other  parts  of  the  small 
intestine  or  in  the  stomach.  Wright  has  recorded  one  case  occurring  in 
the  abdominal  wall  in  the  region  of  the  umbilicus.2  Occasionally,  the 
aberrant  pancreas  is  found  at  the  tip  of  an  intestinal  diverticulum. 


CIRCULATORY  DISTURBANCES. 

These  are  comparatively  unimportant.  Active  hyperemia  is  met  with 
during  digestion  and  in  the  early  stages  of  inflammation.  Passive 
hyperemia  occurs  in  conditions  of  general  passive  congestion.  Hyper- 
emia of  the  pancreas  is  said  also  to  be  present  in  cases  of  pernicious 
anemia. 

Small  hemorrhages  may  take  place  into  the  substance  of  the  pancreas 
in  passive  congestion  of  the  organ,  in  scorbutus  and  other  hemorrhagic 
diatheses,  and  during  the  course  of  infective  fevers.  Massive  hemor- 
rhages also  occur  as  a  result  of  traumatism  and  in  the  affection  known 
as  hemorrhagic  pancreatitis.  This  condition  will  be  referred  to  later. 

Anemia  is  present  in  conditions  of  systemic  anemia. 

1  Nicholls,  Montreal  Medical  Journal,  29: 1900:903. 

2  James  H.  Wright,  Jour.  Boston  Soc.  Med.  Sci.,  5:  1901:  497. 


494  THE  PANCREAS 


INFLAMMATIONS. 

Pancreatitis. — Inflammation  of  the  pancreas — pancreatitis — is  not 
very  common.  In  210  autopsies  of  which  we  have  notes  acute  inflam- 
mation was  found  in  6,  in  only  1  of  which  was  it  the  cause  of  death. 

Acute  pancreatitis  is  usually  due  to  infection  with  microorganisms, 
but  may  on  occasion  result  from  the  action  of  toxic  substances  reaching 
the  gland,  the  irritation  of  the  pancreatic  ferment  or  of  hemorrhagic 
extravasation.  The  infective  forms  arise  in  three  ways — through  the 
blood  stream,  through  the  excretory  duct,  and  by  extension. 

The  hematogenic  type  is  simply  a  manifestation  of  general  septicemia, 
and  is  not  common  as  compared  with  the  frequency  of  general  sepsis. 
By  far  the  commonest  cause  of  pancreatitis  is  the  passage  into  the  gland 
of  bacteria,  bile,  or  acid  stomach  secretions  by  means  of  the  duct  of 
Wirsung. 

Ulcers  of  the  stomach  and  duodenum,  suppurative  processes  in  and 
about  the  spleen  and  left  kidney,  occasionally  extend  to  the  pancreas. 

Pancreatitis  is  simple,  suppurative,  or  specific,  and  may  affect  the  ducts, 
the  lobules,  or  the  interstitial  tissue. 

FIG.  131 


Acute  sialodochitis  with  obstruction  of  the  ducts  leading  to  dilatation  of  the  acini.     Leitz 
obj.  No.  3,  without  ocular.     (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

Sialodochitis  Pancreatica. — Catarrh  of  the  ducts — sialodochitis  pan- 
creatica — which  may  be  simple  or  purulent,  is  due  to  the  action  of  in- 
fective microorganisms  or  toxic  agents  which  have  passed  up  the  duct. 
Duodenitis  seems  to  be  the  important  predisposing  cause.  In  one  case 


SUPPURATIVE  PANCREATITIS 


495 


that  we  have  seen,  the  duct  and  its  chief  branches  were  packed  with 
inflammatory  leukocytes  and  the  lining  epithelium  was  in  places  desqua- 
mating. Owing  to  obstruction,  the  finer  lumina  were  dilated  and  the  acini 
converted  into  small  cystic  cavities,  lined  with  flattened  cells,  which 
also  in  places  were  catarrhal.  The  condition  suggested  the  dilatation 
of  the  tubules  so  often  seen  in  the  kidney  of  chronic  nephritis.  A  certain 
amount  of  interstitial  infiltration  also  had  occurred,  but  it  hardly  amounted 
to  abscess  formation.  In  such  cases  the  main  duct  is  not  necessarily 
always  occluded. 

FIG.  132 


' '  "-'^^Sum  '.-  • 

Sialodochitis  pancreatica.  Section  shows  the  extraordinary  dilatation  of  the  acini  with  a  cer- 
tain amount  o}  catarrh.  The  specimen  is  from  the  same  case  as  the  last,  but  under  higher 
magnification.  Leitz  obj.  No.  7,  without  ocular.  (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

Degenerative  Parenchymatous  Pancreatitis. — Orth  describes  a  degener- 
ative parenchymatous  pancreatitis,  which  may  possibly  be  the  same  con- 
dition spoken  of  by  other  writers  as  cloudy  swelling.  The  pancreas 
is  swollen  and  at  first  reddened,  later  assuming  a  whitish  or  grayish- 
yellow  color.  Histologically,  the  secreting  cells  of  the  acini  present 
cloudy  swelling,  fatty  degeneration,  and  necrosis.  In  a  case  reported 
by  E.  Fraenkel  the  organ  was  dotted  throughout  with  a  fatty  granular 
material  derived  from  the  degenerating  cells,  while  in  the  interstitial 
tissue  there  was  a  round-celled  infiltration.  This  form  of  pancreatitis 
occurs  in  various  infective  processes,  such  as  typhoid  fever,  pyemia, 
and  variola. 

Suppurative  Pancreatitis. — Suppurative  pancreatitis  is  by  far  the  most 
important  form  of  pancreatitis.  It  may  be  acute,  even  gangrenous, 
subacute,  or  chronic.  Diffuse,  purulent  infiltration  may  occur,  or 


496  THE  PANCREAS 

definite  abscesses  may  be  formed.  Abscesses  are  often  multiple  and  of 
small  size,  but  large  single  abscesses  occur  in  some  cases  formed  by  the 
confluence  of  smaller  foci.  The  multiple  pancreatic  abscesses  some- 
times met  with  in  generalized  septicemia  are  hematogenic  in  origin, 
due  to  the  lodgement  of  infective  emboli.  Suppurative  processes  in 
structures  adjacent  to  the  pancreas  may  extend  into  it.  We  have  met 
with  one  case  in  which  an  extensive  perinephritic  abscess  had  burst 
into  the  peritoneal  cavity,  eventually  discharging  into  the  stomach. 
Incidentally  it  caused  gangrenous  inflammation  of  the  lower  end  of  the 
spleen  and  the  tail  of  the  pancreas.  More  frequent  are  those  cases  due 
to  the  invasion  of  the  pancreas  by  way  of  the  duct  of  Wirsung.  Since 
the  common  bile  duct  and  the  pancreatic  duct  usually  empty  by  a  common 
orifice  into  the  intestine,  it  can  readily  be  understood  that  inflammatory 
processes  in  the  duodenum  and  bile  passages  are  apt  to  extend  into  the 
pancreas.  In  such  cases  the  symptoms  are  usually  acute  and  are  prac- 
tically identical  with  those  of  the  so-called  hemorrhagic  pancreatitis 
(q.  v.).  The  cases  occur  generally  in  young  adult  males  and  commonly 
terminate  fatally  in  from  two  to  four  days.  There  is  usually  an  acute 
duodenitis,  which  may  also  be  associated  with  cholangitis,  cholelithiasis, 
and  even  acute  interstitial  hepatitis.  Exceptionally,  the  affection 
becomes  chronic  and  life  may  be  prolonged  for  some  months  or  for  a 
year  or  more. 

Pancreatic  abscesses  may  rupture  into  the  peritoneal  cavity,  giving 
rise  to  a  septic  peritonitis,  or  may  be  discharged  into  the  stomach  or 
intestine.  In  a  case  recorded  by  Chiari,  a  sequestrum  of  the  pancreas 
was  passed  per  anum  with  complete  recovery.  Small  abscesses  may 
possibly  become  encapsulated  or  inspissated.  Fibrosis  is  a  constant 
accompaniment  of  chronic  suppurative  pancreatitis. 

Acute  Hemorrhagic  Pancreatitis. — Closely  allied  to  the  last-mentioned 
form  is  the  affection  known  as  acute  hemorrhagic  pancreatitis,  so-called 
from  the  fact  that  the  pancreas  is  more  or  less  completely  infiltrated  with 
blood.  The  pathogenesis  of  the  condition  is  not  entirely  clear.  Hemor- 
rhage into  the  substance  of  the  pancreas  may  result  from  trauma  and 
disease  of  the  vessels,  as,  for  example,  arteriosclerosis,  fatty  degener- 
ation of  their  walls,  and  embolism,  quite  apart  from  inflammation. 
Of  course,  such  extravasations  of  blood,  if  not  quickly  fatal  and  if  not 
so  extensive  as  to  destroy  the  pancreas,  are  followed  by  a  certain  amount 
of  reactive  inflammation.  The  general  consensus  of  opinion,  however, 
seems  to  be  that  in  most  cases  the  inflammation  is  the  cause  of  the 
hemorrhage.  Quite  often,  fat-necrosis  of  the  pancreas,  peripancreatic 
fat,  the  fat  of  the  omentum  and  mesentery,  is  present  also,  and  it  may  be 
that  the  blood  extravasation  is  to  be  explained  on  the  ground  of  erosion 
of  the  vessel  walls  by  the  liberated  pancreatic  ferment  or  the  action  of 
suppurative  inflammation. 

Hemorrhagic  pancreatitis  is  found  most  often  in  alcoholics  and  obese 
persons,  usually  in  those  over  thirty.  The  disease  is  ushered  in  suddenly 
with  intense  pain  in  the  epigastrium,  nausea,  and  vomiting.  The 
abdomen  becomes  rigid  and  hypertympanitic.  The  temperature  may 


CHRONIC  PANCREATITIS  497 

be  moderately  elevated.  Constipation  is  the  rule.  The  patient  fre- 
quently dies  collapsed  in  from  two  to  four  days.  The  disease  may, 
however,  become  subacute  or  even  chronic.  Cases  are  often  mistaken 
for  gastric  ulcer,  biliary  colic,  or  acute  intestinal  obstruction.  Patients 
usually  give  a  history  of  dyspepsia  or  of  symptoms  pointing  to  chole- 
lithiasis. Fitz1  has  written  an  excellent  monograph  on  the  subject. 

The  pancreas  is  found  to  be  enlarged,  firm,  and  densely  infiltrated 
with  blood,  often  in  large  clots.  The  adjacent  cellular  tissue,  the  omen- 
turn,  the  root  of  the  mesentery,  and  the  lesser  peritoneal  cavity  may  con- 
tain blood.  There  may  also  be  a  small  amount  of  blood-stained  fluid  in 
the  general  abdominal  cavity.  More  or  less  extensive  fat-necrosis  is 
usually  present.  The  inflammation,  no  doubt  resulting  from  infection, 
may  be  intense,  leading  to  suppuration  or  even  gangrene  of  the  organ. 
Death  takes  place  probably  from  pressure  upon  the  cceliac  axis,  as  sug- 
gested by  v.  Zenker,  from  shock,  or  from  sepsis.  The  nerve  cells  of  the 
semilunar  ganglia  have  been  found  to  be  degenerated  with  an  interstitial 
leukocytic  infiltration.  Degenerative  changes  have  also  been  observed 
in  the  peripancreatic  Paccinian  corpuscles.2  The  common  association 
of  the  condition  with  cholangitis  and  cholelithiasis,  suggests  that  bac- 
terial infection  is  an  important  factor.  The  impaction  of  a  gallstone 
in  the  ampulla  of  Vater  may  lead  to  the  entrance  of  bile  under  pressure 
into  the  pancreatic  duct.  Fat-necrosis  and  hemorrhagic  pancreatitis 
have  been  produced  experimentally  by  the  injection  of  bile,  hydrochloric 
acid,  and  other  irritating  substances  into  the  duct,  or  even  by  simple 
ligation  of  the  duct.  Alcohol  and  dietetic  errors  may  act  by  setting  up 
a  duodenitis,  which  thereupon  extends  to  the  pancreas.  Possibly,  in 
some  cases,  the  fatal  attack  is  the  last  of  the  series  of  unobtrusive 
hemorrhages,  for  not  infrequently  microscopic  examination  shows  pig- 
ment from  old  hemorrhages  along  with  recent  clots.  Should  the  patient 
survive  for  any  length  of  time,  necrotic  portions  of  the  gland  may  become 
sequestrated  and  may  lie  in  the  centre  of  abscess  cavities,  which  later 
may  discharge  into  the  abdominal  cavity,  the  stomach,  or  intestine. 

Chronic  Pancreatitis. — ^Chronic  pancreatitis,  like  the  acute  form, 
may  be  simple,  suppurative,  and  specific.  There  is  always  a  more  or 
less  widespread  production  of  fibrous  tissue,  which  leads  to  induration 
of  the  organ  (productive  pancreatitis;  sclerosis  or  fibrosis  of  the  pan- 
creas; indurative  pancreatitis).  Associated  with  this  is  usually  a  certain 
amount  of  atrophy  of  the  secreting  structure.  The  whole  organ,  there- 
fore, becomes  harder  than  normal.  The  most  frequent  causes  are  per- 
sistent inflammation  of  the  ducts,  obstruction  to  the  free  discharge  of 
the  excretion,  and  congenital  syphilis. 

In  these  cases  the  pancreas  may  be  somewhat  enlarged,  but  is  usually 
considerably  diminished  in  size.  On  section,  it  is  hard  and  resists 
cutting,  occasionally  containing  gritty  material. 

Histologically,  there  is  a  notable  increase  in  the  amount  of  connec- 

1  Boston  Med.  and  Surg.  Jour.,  120:  1889:  181. 

2  Warthin,  Reference  Handbook  of  the  Medical  Sciences,  6: 1903:  456. 
32 


498  THE  PANCREAS 

tive  tissue,  with  more  or  less  atrophy  of  the  glandular  elements.  In 
the  obstructive  cases  the  newly-formed  tissue  is  most  marked  about  the 
ducts.  Opie  distinguishes  two  forms  of  fibrosis,  a  chronic  interlobular 
pancreatitis,  in  which  the  connective  tissue  is  most  conspicuous  between 
and  around  the  lobules,  the  islands  of  Langerhans  being  involved  only 
late  or  not  at  all,  and  a  chronic  interacinar  pancreatitis,  in  which  the 
fibrosis  about  the  lobules  is  less  pronounced,  but  connective-tissue  pro- 
liferation is  extreme  round  about  the  islands  and  even  within  them. 
Pearce  has  pointed  out  that  in  the  lesions  of  congenital  syphilis  the 
islands  of  Langerhans  escape. 

FIG.  133 


Syphilitic  pancreatitis.     Reichert  obj.  No.  7,  without  ocular.      (Dr.  H.  D.  Rolleston's  case.) 

Calculi. — The  presence  of  calculi  is  one  of  the  most  potent  causes  of 
chronic  interstitial  pancreatitis  and  pancreatic  abscess.  Like  biliary 
calculi,  they  are  originally  brought  about  by  catarrh  of  the  duct.  When 
large  they  may  bring  about  obstruction,  with  dilatation  and  even 
cyst  formation.  Pancreatic  calculi  are  single  or  multiple,  round, 
oval,  or  irregular  in  shape,  and  of  a  whitish  or  grayish-brown  color. 
Chemically,  they  are  composed  of  carbonate  or  phosphate  of  lime. 
They  may  be  minute  or  quite  large.  One  reported  by  Schupmann 
was  6  cm.  long  by  1  cm.  broad.  Owing  to  the  proliferative  inflammation 
resulting,  a  large  portion  of  the  secreting  structure  of  the  pancreas 
may  be  destroyed  and  glycosuria  result. 

Tuberculosis. — This  disease  is  rare  in  the  pancreas.  One  case  of 
primary  caseous  tuberculosis  is  reported  by  Sendler,  but  this  seems  to  be 
unique.  Secondary  tuberculosis  is  also  rare.  Disseminated  miliary 


SIMPLE  ATROPHY  499 

tuberculosis  is  recorded  as  affecting  the  pancreas,  but  the  foci  are  small 
and  few  in  number.  Nearly  all  the  cases  are  due  to  the  extension  of 
tuberculous  disease  from  the  lymph-nodes  and  other  neighboring  struc- 
tures. It  is  the  lymphoid  structures  within  the  pancreas  which  become 
involved.  The  organ  seems  to  have  special  powers  of  self-defence,  for 
it  is  surprising  how  often  it  escapes  even  when  completely  surrounded 
by  tuberculous  disease. 

Syphilis. — This  disease  is  also  rare  in  the  pancreas.  Chronic  indurative 
pancreatitis  is  observed  in  cases  of  the  congenital  affection  (Fig.  133). 
Either  the  whole  organ  or  the  head  only  may  be  involved.  Gummas 
are  occasionally  observed.  When  near  the  point  of  exit  of  the  duct, 
jaundice  may  result  from  the  pressure.  Syphilitic  endarteritis  may 
also  occur,  leading  to  fibrosis  of  the  organ.  Reuter1  has  found  the 
Spirocheta  pallida  in  the  interstitial  tissue  in  a  case  of  chronic  syphilitic 
pancreatitis. 

RETROGRESSIVE     METAMORPHOSES. 

Simple  Atrophy. — Simple  atrophy  occurs  in  old  age  and  in  the 
marasmus  of  infective  or  chronic  disease.  The  pancreas  is  small, 

FIG.  134 


Pancreas.    Diabetes  mellitus.    Section  shows  round-celled  infiltration  in  the  interacinar  connective 
tissue.     Zeiss  obj.  DD,  without  ocular.     (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

shrunken,  and  cylindrical  on  section.     The  surrounding  fat  is  diminished. 
The  organ  cuts  with  somewhat  increased  resistance,  the  lobules  are 

1  Zeit.  f.  Hyg.  u.  Infect.,  54: 1906:49. 


500  THE  PANCREAS 

small,  and  the  interstitial  connective  tissue  is  relatively  increased.  When 
atrophy  is  extreme,  fatty  globules  and  crystals  make  their  appearance 
in  the  feces.  According  to  Demme  and  Biedert,  the  fatty  diarrhoeas  of 
children  are  associated  with  atrophy  and  fibrosis  of  the  pancreas.  Atrophy 
of  the  lobules  may  also  be  secondary  to  interstitial  fibrosis.  In  many 
cases  of  diabetes  the  pancreas  is  found  to  be  shrunken,  but  differs  some- 
what in  appearance  from  the  pancreas  of  simple  atrophy.  The  organ  is 
small,  flabby,  and  relaxed.  It  is  brownish  in  color  and  rather  flat  on 
transverse  section..  There  may  be  a  compensatory  increase  of  the  fat 
in  the  neighborhood.  Histologically,  the  secreting  cells  are  wasted 
while  the  interlobular  connective  tissue  is  distinctly  increased.  There 
is,  in  addition,  an  interstitial  infiltration  with  round  cells  which  extends 
from  the  periphery  for  some  little  distance  into  the  lobule  (Fig.  134). 
Opie1  has  shown  that  in  a  considerable  proportion  of  cases  the  islands 
of  Langerhans  are  in  a  state  of  hyaline  degeneration.  This  he  thinks  is 
the  cause  of  pancreatic  diabetes,  the  degeneration  interfering  with  the 
production  of  the  internal  secretion  of  the  organ. 

Fatty  Degeneration. — Fatty  degeneration  of  the  secreting  cells  is  com- 
mon. It  occurs  in  pancreatitis,  passive  congestion,  in  the  course  of 
infective  fevers,  and  in  poisoning  from  mineral  salts. 

Fatty  Infiltration. — Fatty  infiltration  occurs  in  general  obesity,  and  in 
jases  where  the  intra-abdominal  circulation  is  interfered  with.  It  may 
also  be  compensatory  to  atrophy  or  fibrosis  of  the  pancreas.  On  section, 
the  organ  is  found  to  contain  more  or  less  numerous  pads  of  fat,  separating 
widely  the  lobules,  which,  as  a  result,  may  be  markedly  atrophic.  The 
condition  predisposes  to  hemorrhage  into  the  substance  of  the  pancreas, 
possibly  of  the  nature  of  an  infarction. 

Fat  Necrosis.— This  curious  affection  is  characterized  by  the  formation 
of  areas  of  degeneration,  varying  in  size  from  that  of  a  pin-head  to  that  of 
a  pea,  or  even  larger,  in  the  pancreas,  omentum,  or,  in  fact,  in  any  of  the 
fatty  structures  within  the  abdomen.  The  areas  in  question  are  of  a 
dead  white  appearance,  sometimes,  but  not  invariably,  surrounded  by  a 
hemorrhagic  or  inflammatory  zone.  They  are  soft,  or  have  gritty 
centres.  Occasionally  the  contents  liquefy,  forming  small  cysts.  When 
in  the  pancreas  the  foci  are  situated  in  the  interstitial  stroma.  Cases  are 
on  record,  however,  in  which  fat-necrosis  affected  the  omentum,  while 
the  pancreas  itself  was  free.  The  condition  is  usually  associated  with 
acute  or  chronic  inflammation  of  the  organ,  tumors,  or  obstruction. 
The  researches  of  Hildebrand  and  Flexner  have  proved  that  fat-necrosis 
is  due  to  a  liberation  of  the  fat-splitting  ferment  of  the  pancreatic  secre- 
tion, which  acts  upon  the  fatty  substance  of  the  pancreas  and  adjacent 
parts,  converting  the  fats  into  fatty  acids  and  subsequently  into  salts 
formed  by  the  combination  of  the  fatty  acids  with  calcium.  Severe 
cases  are  sometimes  associated  with  extensive  hemorrhage  into  the  pan- 
creas, pancreatitis,  or  the  sequestration  of  large  portions  of  the  organ. 
Death,  therefore,  is  not  an  uncommon  result. 

1  Opie,  Jour.  Exper.  MecL,  5:  1901 :  397. 


TUMORS  501 

Calcification. — Calcification  is  observed  in  connection  with  fat 
necrosis,  intrapancreatic  extravasations  of  blood,  pancreatitis,  and  fatty 
infiltration. 

Self-digestion. — A  condition  to  which  more  than  a  passing  reference 
should  be  made  is  the  so-called  self-digestion.  A  pancreas  thus  affected 
has  a  peculiar  dead  white,  sometimes  slightly  glazed,  appearance. 
Under  the  microscope,  in  the  milder  grades  of  the  affection,  here  and 
there  through  the  pancreas  can  be  seen  lobules  or  portions  of  lobules  in 
which  the  nuclei  are  undergoing  fragmentation,  appearing  like  small 
particles  of  pigment,  while  the  outline  of  the  cells  is  lost.  In  the  most 
advanced  condition,  the  whole  substance  of  the  pancreas  presents  a 
diffuse,  opaque  appearance,  somewhat  resembling  ground  glass,  staining 
strongly  writh  eosin.  No  nuclei  are  to  be  seen,  and  only  the  rough  out- 
lines of  the  lobules  and  ducts  remain  to  indicate  the  character  of  the  organ. 
The  condition  was  first  adequately  described  by  Chiari,  who  attributed 
it  to  the  action  of  the  digestive  ferment  on  the  pancreas  itself.  It  is 
frequently  found,  being  present  in  63  per  cent,  of  autopsies.  It  is  not 
unlikely,  however,  that  in  some  cases  it  occurs  as  an  agonal  change,  or 
even  some  little  time  before  death.  In  general,  the  longer  the  time  that 
has  elapsed  after  death  the  more  advanced  the  condition.  But  time  is 
not  the  only  factor.  We  have  noticed  that  extensive  self-digestion  may 
be  present  as  early  as  three  hours  after  death,  while  in  other  cases  the 
condition  may  be  scarcely  noticeable  even  at  the  end  of  from  thirty  to 
forty-eight  hours.  This  suggests  that  the  physiological  condition  of  the 
gland  at  the  time  of  death  may  be  of  importance.  If  the  acini  be 
loaded  with  ferment,  we  would  expect  to  get  rapid  self-digestion.  On 
the  other  hand,  if  the  ferment  has  been  discharged,  the  action  would 
be  lacking  or  retarded.  We  should  remember  in  this  connection  that 
death  does  not  at  once  arrest  secretory  action  on  the  part  of  the  glands, 
so  that  if  at  the  moment  of  death  the  pancreas  were  in  a  state  of  rest,  it 
might  again  secrete  some  hours  later.  Possibly,  too,  the  condition  of 
acidity  or  alkalinity  of  the  blood  and  tissue-fluids,  depending  in  some 
measure  on  the  nature  of  the  bacterial  flora  in  the  body,  may  have  a 
bearing  on  the  question.  Bile  and  other  fluids  may  also  pass  up  the 
pancreatic  duct  and  modify  the  condition  therein.  Lastly,  the  amount 
of  bodily  heat  and  the  degree  of  the  external  temperature  would,  no 
doubt,  have  some  influence. 


PROGRESSIVE   METAMORPHOSES. 

Tumors. — Primary  tumors  of  the  pancreas-  are  somewhat  rare. 
Remo  Segie,1  in  11,500  autopsies  at  Milan,  found  132  cases,  divided  as 
follows:  carcinomas,  127;  sarcomas,  2;  cysts,  2;  syphiloma,  1.  In  1514 
post  mortems  at  the  Montreal  General  and  Royal  Victoria  Hospitals, 
primary  carcinoma  occurred  6  times  and  adenoma  once.  It  appears 

1  Ann.  univ.  di  med.  e  chir.,  283: 1885:  5. 


502 


THE  PANCREAS 


FIG.  135 


Adenoma  of  the  pancreas,  arising  from  an  island  of  Langerhans.  Winckel  No.  3,  without 
ocular.  The  tumor  lies  to  the  right  and  is  separated  from  the  normal  pancreatic  tissue  by  a 
thin  connective-tissue  capsule.  (From  the  collection  of  Dr.  A.  G.  Nicholls.) 


.4**^ 

+**• 

i  * .  * 


FIG.  136 


" 


Adenoma  of  the  pancreas  arising  from  an  island  of  Langerhans.     Leitz  obj.  No.  7,  without 
ocular.     (From  the  collection  of  Dr.  A.  G.  Nicholls.) 


CYSTS  503 

from  this  that  carcinoma  is  by  far  the  most  common  primary  new-growth. 
Benign  growths  are  excessively  rare.  Adenoma  of  a  tubular  character 
and  fibroadenoma  are  described  (Cesaris-Demel;  Biondi).  One  of  us 
(A.  G.  N.)  has  recorded  for  the  first  time  a  case  of  adenoma  arising 
from  an  island  of  Langerhans.1  A  similar  one  has  been  reported  recently 
by  Helmholz.2  Some  of  the  so-called  pancreatic  cysts  are  to  be  classed  as 
cystadenomas,  of  which  there  appear  to  be  two  varieties,  multilocular 
cystomas  and  papillomatous  cystomas.3 

Sarcoma. — Primary  sarcoma  is  but  little  less  rare.  It  is  usually  situ- 
ated in  the  head  or  tail  of  the  pancreas.4 

Lymphosarcoma. — Lymphosarcoma  probably  occurs.  In  the  only 
case  we  have  seen  that  seemed  to  come  under  this  category,  the  pancreas 
was  much  enlarged  and.  nodules  of  new-growth,  having  a  soft,  white 
appearance,  were  found  between  the  lobules.  Microscopically,  there 
was  a  diffuse  infiltration  of  the  organ  with  lymphoid  cells,  which,  in  parts, 
were  aggregated  together  into  definite  clumps. 

Carcinoma. — Carcinoma  is  ordinarily  met  with  in  the  head  of  the  organ. 
It  is  usually  of  the  scirrhous  type,  but  medullary  and  colloid  cancers  are 
occasionally  found.  Carcinoma  of  the  body  or  tail  is,  however,  quite 
common.  We  have  observed  two  such  cases  lately  at  the  Royal  Victoria 
Hospital.  Pressure  upon  the  common  bile  duct  from  a  growth  of  this 
kind  results  in  icterus;  pressure  upon  the  duct  of  Wirsung,  in  dilatation 
and  even  cyst  formation.  In  not  a  few  cases,  if  the  pancreas  be  exten- 
sively infiltrated,  glycosuria  makes  its  appearance. 

Histologically,  these  growths  are  adenocarcinomatous  or,  occasionally, 
cylindrical-celled  in  type. 

Secondary  carcinomas  usually  arise  by  the  extension  of  a  primary 
growth  in  the  stomach,  duodenum,  ampulla  of  Vater,  or  the  biliary 
passages.  Metastases  by  the  blood  or  lymph-stream  are  not  nearly  so 
common. 

Cysts. — Apart  from  the  cystadenomas  above  mentioned,  cysts  of  the 
pancreas  may  be  due  to  obstruction  of  the  ducts  (ranula  pancreatica) 
or  of  its  intralobular  radicles  (acne  pancreatica'),  to  hydatid  disease,  or 
to  congenital  peculiarities. 

Pseudopancreatic  cysts  are  developed  in  structures  in  the  neighborhood 
of  the  pancreas,  with  disease  of  which  they  are  occasionally  connected. 
They  may  also  be  due  to  traumatism,  colliquative  necrosis,  and  effusions 
of  fluid  in  the  lesser  peritoneal  sac. 

1  Jour,  of  Med.  Research  (N.  S.,  3) :  1902:  385. 

2  Johns  Hopkins  Hospital  Bull.,  18:1907: 185. 

3  See  Ransohoff,  Amer.  Med.,  2: 1901: 138. 

4  For  literature,  see  Geo.  A.  Boyd,  Trans.  Chicago  Path.  Soc.,  4:  1899-1901:  191. 


CHAPTEE    XXIV. 

THE  PERITONEUM. 

THE  peritoneum  is  a  delicate,  connective-tissue  membrane,  containing 
elastic  fibrils,  and  covered  with  a  layer  of  flattened  endothelial  cells. 
The  deeper  parts  contain  the  bloodvessels  and  lymphatics,  the  latter  of 
which  are  in  functional  communication  with  the  abdominal  cavity,  which 
is  thus  to  be  regarded  as  a  large  lymph-space.  The  peritoneal  membrane 
invests  the  diaphragm,  abdominal  wall,  and  the  various  organs  contained 
within  the  abdominal  cavity.  Furthermore,  the  lymph-channels  are  in 
direct  communication  with  those  of  the  pleural  sacs  and  anterior  medi- 
astinum. Consequently,  the  great  majority  of  the  disorders  of  the  peri- 
toneum are  secondary  to  disease  of  the  underlying  viscera  or  contiguous 
serous  sacs.  In  this  connection,  the  most  important  factors  are  various 
infective  conditions,  notably  of  the  digestive  tract,  less  often  of  the  liver 
and  portal  system,  and  of  the  female  genital  apparatus.  The  character 
and  intensity  of  these  infective  processes  are  dependent  largely  on  the 
nature  of  the  invading  microorganisms.  The  extension  of  the  lesion  is  in 
many  cases  assisted  by  the  movements  of  the  intestines,  and  modified  or 
localized  through  the  agency  of  the  great  omentum  and  of  adhesions. 

CONGENITAL  ANOMALIES. 

Lawson  Tait1  has  recorded  a  case  in  which  the  mesentery  was  com- 
pletely absent,  the  peritoneal  investment  covering  only  a  small  portion 
of  the  circumference  of  the  intestines  and  passing  directly  from  one  coil 
to  another. 

The  mesentery  may  be  abnormally  long  or  short,  or  may  present 
defects  in  its  substance.  The  latter  condition  is  of  practical  importance, 
since  coils  of  the  bowel  may  become  prolapsed  through  the  opening, 
resulting  in  obstruction  or  strangulation. 

Pocket-like  diverticula,  abnormal  folds  and  duplications,  and  prolonga- 
tion of  the  membrane  into  persistent  inguinal  canals  are  not  uncommon. 

The  great  omentum  varies  much  in  size,  being  long  or  short,  or,  again, 
almost  completely  absent.  Bifurcations  and  partial  defects  are  more 
common. 

CIRCULATORY  DISTURBANCES. 

Being  in  intimate  relationship  with  so  many  of  the  viscera,  and  being 
itself  vascular,  local  circulatory  disturbances  of  the  peritoneum  are 
easily  brought  about.  In  this  connection  such  conditions  as  inflam- 

1  Dublin  Quart.  Jour,  of  Med.  Sci.,  47: 1869:85. 


PERITONITIS  505 

mation  of  the  gastrointestinal  tract,  hernias,  obstruction,  and  tumors 
are  of  importance. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  is  met  with  in  the 
first  stages  of  inflammation  and  as  a  result  of  the  sudden  diminution  of 
intra-abdominal  pressure,  such  as  is  caused  by  the  removal  of  ascitic 
fluid  or  large  tumors.  Inflammatory  hyperemia  may  be  generalized 
throughout  the  peritoneal  membrane  or  may  be  localized  to  special  areas. 

Passive  Hyperemia. — Passive  hyperemia  results  from  obstruction  to 
the  portal  circulation,  either  directly  from  disorders  of  the  liver  or  indi- 
rectly from  the  heart  or  lungs.  The  vessels  of  the  great  omentum, 
stomach,  and  intestines  become  greatly  distended,  and  in  long-standing 
cases  there  is  a  transudation  of  clear,  watery,  slightly  yellowish  fluid 
into  the  abdominal  cavity  (ascites;  hy  drops  peritonei),  occasionally 
tinged  with  bile  or  blood-pigment.  Not  infrequently,  soft  gelatinous 
clots  of  fibrin  or  shreds  of  endothelium  can  be  detected  upon  the  surface 
of  the  intestines. 

Interesting  forms  of  intra-abdominal  effusion  are  chylous,  chyliform, 
and  pseudochylous  ascites.  The  distinctions  between  these  three  have 
already  been  discussed  (p.  111). 

Ascitic  fluid  may  be  free  in  the  abdominal  cavity,  when  it  tends  to 
collect  in  the  pelvis  and  flanks,  if  the  patient  be  in  a  recumbent  position, 
but  when  excessive  may  fill  the  greater  portion  of  the  abdomen.  In 
children  the  fluid  often  collects  between  the  layers  of  the  great  omentum 
(hy drops  omenti).  The  presence  of  ascitic  fluid  leads  to  more  or  less 
distension  of  the  abdomen,  with  pressure  upon  the  contained  viscera, 
and  upward  dislocation  of  the  diaphragm.  The  thoracic  organs  may  be 
interfered  with  and  serious  disturbance  of  circulation  and  respiration 
result.  In  long-standing  cases  the  peritoneal  membrane  becomes 
thickened  and  presents  a  somewhat  pearly  appearance. 

Hemorrhage. — Extravasations  of  blood  occur  into  the  abdominal 
cavity  and  into  the  substance  of  the  peritoneum.  The  more  extensive 
effusions  occur,  as  a  rule,  from  traumatic  rupture  of  various  organs,  the 
bursting  of  an  aneurism  or  of  the  sac  in  a  tubal  gestation,  or,  again,  as 
the  result  of  operations.  Operations  about  the  liver  and  bile  passages, 
particularly  where  there  is  jaundice,  are  not  infrequently  followed  by 
serious  intra-abdominal  hemorrhage.  Large  effusions  often  lead  to 
death,  while  smaller  ones  may  be  absorbed,  leaving  blackish-brown 
stains  on  the  peritoneal  surface,  the  result  of  the  chemical  interaction 
of  the  sulphuretted  hydrogen  from  the  intestines  upon  the  iron  contained 
in  the  blood-pigment. 

Petechia?  or  small  ecchymoses  occur  in  all  forms  of  active  and  passive 
hyperemia,  in  sepsis  and  other  infective  processes,  and  in  the  hemor- 
rhagic  diatheses. 

INFLAMMATIONS. 

Peritonitis. — The  inflammations  of  the  peritoneum — peritonitis — 
are,  generally  speaking,  not  unlike  those  of  the  other  serous  membranes. 


506  THE  PERITONEUM 

On  the  whole,  however,  they  tend  to  be  purulent  or  fibrinopurulent 
rather  than  fibrinous  and  serofibrinous. 

We  may,  perhaps,  with  Grawitz,  divide  peritonitis  into  primary  and 
secondary  forms.  Primary  peritonitis  is  also  called  "idiopathic"  or 
"rheumatic/'  owing  to  the  difficulty  of  assigning  the  condition  to  a 
definite  cause.  It  is  theoretically  possible  that  toxic  agents  of  a  chemical 
nature  circulating  in  the  blood  might  reach  the  peritoneum  and  set 
up  inflammation  in  the  absence  of  any  other  localization,  but  little  or 
nothing  is  known  in  regard  to  this  occurrence.  The  overwhelming 
number  of  peritonitides  are  secondary  to  disease  elsewhere,  usually  of 
the  abdominal  viscera  or  of  the  adjacent  serous  sacs.  Less  often  the 
inflammation  is  hematogenic  in  nature.  It  is,  moreover,  practically 
certain  that  all  forms  of  peritoneal  inflammation  are  due  to  the  activity 
of  pathogenic  microorganisms.  Trauma,  by  lessening  the  resisting 
power  of  the  membrane,  or  by  damaging  the  structure  or  continuity  of 
the  hollow  viscera,  is  an  important  etiological  factor. 

Flexner1  defines  primary  peritonitis  to  be  "a  condition  in  which  the 
inflammation,  usually  diffuse,  of  the  serous  cavity  takes  place  without 
the  mediation  of  any  of  the  contained  organs,  and  independently  of  any 
surgical  operation  upon  these  parts."  This  form  is  due  to  microorgan- 
isms which  reach  the  membrane  through  the  blood  stream  or  the  lymph 
channels,  or  again,  through  the  lumen  of  the  Fallopian  tubes.  (See  vol.  i, 
p.  287.)  It  is  conceivable  that  bacteria  may  pass  through  an  intact 
intestinal  wall  and  set  up  peritonitis,  but  whether  this  ever  occurs  is  not 
easily  proved.  At  all  events,  bacteria  may  on  occasion  pass  through  in 
the  absence  of  any  gross  lesion  of  the  intestine  or  any  solution  of  its 
continuity,  as,  for  example,  in  those  forms  which  complicate  ascites. 

Of  106  cases  of  acute  peritonitis  (not  including  tuberculous  forms) 
studied  by  Flexner,  12  were  primary.  Microorganisms  were  found  in 
10  of  these,  in  9  of  which  there  was  but  a  single  form  of  germ.  In  these 
cases,  an  inflammatory  focus  in  almost  any  part  of  the  body  may  provide 
the  infecting  agent. 

Flexner  also  divides  the  secondary  peritonitides  into  exogenous  and 
endogenous.  The  former  class  are  examples  of  wound-infection,  the 
bacteria  having  been  imported  from  without.  In  the  endogenous  form 
the  infective  microorganisms  come  from  the  intestinal  tract.  Of  34 
cases  of  the  former  type,  25  were  single  and  9  were  multiple  infections. 
The  organism  most  frequently  found  was  the  Staphylococcus  aureus, 
and  next  to  that  the  Streptococcus  pyogenes.  The  B.  coli,  when  present, 
was  generally  associated  with  these  other  forms.  In  the  latter  type  of 
peritonitis  the  infection  is  usually  multiple.  Here  the  B.  coli  was  most 
frequently  found;  next,  the  Streptococcus  pyogenes;  and  then  both 
combined.  As  Flexner  correctly  points  out,  the  few  cases  of  primary 
peritonitis  that  do  occur  are  examples  of  terminal  infection. 

The  most  common  cause  of  peritonitis,  then,  is  some  form  of  gastro- 
intestinal disease,  usually  ulceration  and  perforation.  In  this  connection 

1  Phila.  Med.  Jour.,  2: 1898: 1019. 


PERITONITIS  507 

appendicitis,  intestinal  obstruction,  gastric  ulcer,  strangulated  hernia, 
and  dysentery  are  of  importance.  Among  other  etiological  factors  may 
be  mentioned  cholecystitis,  cholangitis,  hepatitis,  splenitis,  mesenteric 
thrombophlebitis,  infected  mesenteric  glands,  and  inflammation  of  the 
pelvic  viscera.  Occasionally,  the  infection  spreads  from  the  pleural 
cavities,  and  the  condition  forms  part  of  a  generalized  multiserositis. 
Some  few  cases  are  hematogenic,  the  primary  focus  of  infection  being  in 
some  other  part  of  the  body. 

According  to  the  character  of  the  exudation,  we  can  classify  peritonitis 
as  fibrinous,  serofibrinous,  fibrinopurulent,  suppurative,  and  hemorrhagic. 
The  character  of  the  inflammation  depends  largely  on  the  nature  of  the 
infecting  microorganisms. 

The  acute  purulent  exudates  are  commonly  due  to  the  B.  coli,  Strepto- 
coccus, Staphylococcus,  and  the  Diplococcus  pneumonias.  The  B.  tuber- 
culosis and  the  Gonococcus  are  more  apt  to  excite  subchronic  and  chronic 
inflammation. 

With  regard  to  the  distribution  of  the  lesions  in  peritonitis,  surgeons 
generally  recognize  localized  and  diffuse  forms.  Whether  an  inflammation 
of  the  peritoneal  membrane  will  spread  and  become  generalized  depends 
a  good  deal  on  the  nature  of  the  infecting  microorganism  and  the  resisting 
power  of  the  individual.  Where  the  B.  coli,  B.  tuberculosis,  and  Gono- 
coccus are  at  work,  plastic  or  fibrous  adhesions  are  apt  to  form  about 
the  primary  focus  of  irritation,  which  more  or  less  perfectly  wall  off  the 
infective  agents  and  prevent  their  extension.  In  typhoid  fever,  on  the 
other  hand,  peritonitis  resulting  from  perforation  is  almost  invariably 
fatal,  owing  to  the  lack  of  such  reactive  inflammation.  The  omentum 
plays  an  important  part,  also,  in  peritoneal  inflammations,  as  one  of  us 
(J.  G.  A.1),  has  pointed  out.  Wherever  there  are  inflamed  areas  in  the 
abdominal  cavity  the  omentum  appears  to  search  them  out  and  applies 
'itself  to  them  with  great  rapidity,  becoming  adherent  by  plastic  and  later 
by  fibrous  adhesions.  It  is  well-known  that  the  omentum  in  this  way 
very  often  saves  the  situation  and  prevents  a  general  peritonitis.  The 
occurrence  is,  in  part,  attributable  to  the  fact  that  leukocytes  are  present 
in  great  numbers  upon  the  surface  of  the  omentum  which  are  the  source 
of  the  fibrinous  deposit.  In  this  connection,  Durham2  has  shown  in 
the  case  of  experimental  animals  that  at  the  end  of  one  hour  after  the 
intraperitoneal  injection  of  bacteria  the  peritoneal  fluid  contains  rela- 
tively few  cells.  This  leukopenia  is  due  in  the  main  to  the  attachment  of 
the  free  cells  to  the  surface  of  the  great  omentum.  In  animals  that  died 
within  twenty-four  to  forty-eight  hours  after  inoculation  with  an  efficient 
but  not  too  great  a  number  of  bacteria,  the  peritoneal  fluid  was  found 
to  give  no  growth  on  culture  media,  though  vigorous  growths  could  be 
obtained  from  the  omentum.  This  probably  explains  how  it  is  that  the 
fluid  removed  from  the  peritoneal  cavity  in  operation  cases  so  frequently 
is  sterile.  The  omentum  thus  may  become  attached  to  any  of  the 

1  The  Great  Omentum,  Phila.  Med.  Jour.,  1 : 1898 : 373. 

2  On  the  Mechanism  of  Peritoneal  Infections.  Jour,  of  Path,  and  Bact.,  4:  1897: 
338. 


508 


THE  PERITONEUM 


abdominal  viscera  that  are  inflamed,  often  forming  a  complete  barrier  to 
the  progress  of  the  inflammation,  and  in  the  same  way  may  close  in  per- 
forations of  the  hollow  viscera  or  abdominal  wall.  The  protective  power 
of  the  omentum  is  often  well  seen  in  cases  of  perforative  appendicitis, 
and  it  is  curious  how  it  will  search  out  and  wrap  itself  about  the  diseased 
structure.  In  some  cases,  the  protection  is  but  temporary,  however, 
the  omental  vessels  becoming  thrombosed  and  the  organ  itself  gangrenous. 
The  morbid  anatomy  of  acute  peritonitis  varies  according  to  the  nature 
and  intensity  of  the  infection.  In  cases  of  low  virulence,  such  as  are 
occasionally  met  with  in  ascites  and  passive  congestion  of  the  abdominal 


FIG.  137 


CL 


Exogenous  perforation  of  the  lower  end  of  the  ascending  colon.  The  illustration,  which  is 
natural  size,  shows  well  the  curious  raised  and  perforated  condition  of  the  mucous  membrane, 
and,  at  a,  the  opening  through  the  muscle  wall.  (Adami.) 

viscera,  the  serous  surface  of  the  intestines  presents  little  more  than  a 
slight  loss  of  lustre,  with  here  and  there  a  trifling  deposit  of  delicate 
fibrin.  In  the  ordinary  form,  during  the  first  stage,  there  is  marked 
congestion  of  the  serous  membrane,  especially  where  the  coils  of  intestine 
come  into  contact.  A  little  later  the  serous  surface  becomes  dull  and 
lustreless  and  the  contiguous  surfaces  become  slightly  adherent  through 
the  deposition  of  threads  or  yellowish-white  flakes  of  fibrin.  In  some 
instances  there  is  but  little  effusion  of  fluid,  but  not  infrequently  there 
is  a  moderate  exudation  of  somewhat  turbid  fluid  containing  soft  pulta- 
ceotis  masses  and  flakes  of  a  yellowish  color.  This  exudate  tends  to 
gravitate  to  the  most  dependent  parts,  namely,  the  pelvis  and  the  flanks. 


CHRONIC  PERITONITIS  509 

In  other  cases  the  exudation  is  more  turbid  and  definitely  purulent. 
In  cases  which  have  lasted  some  little  time,  we  may  have  relatively  little 
fluid  poured  out,  but  we  find  small  pockets  of  thick,  yellowish-green  pus 
between  the  coils  of  intestine,  which  are  somewhat  firmly  adherent, 
or  about  the  liver  and  spleen.  It  is  not  very  uncommon' for  the  pus  to 
burrow  through  the  intestinal  wall  and  find  vent  in  that  way  (Fig.  137). 
Or,  the  more  fluid  part  of  the  exudation  is  absorbed,  the  pus  deposits 
thus  becoming  inspissated  or  even  calcified,  while  they  become  walled 
off  by  dense  fibrous  adhesions. 

The  peritonitis  that  supervenes  upon  perforation  of  the  intestine  is 
particularly  virulent,  and  the  exudation  is  feculent  as  well  as  purulent. 
The  abdomen  usually  contains  a  considerable  quantity  of  dirty,  brown, 
turbid  fluid  having  an  offensive  fecal  odor.  The  intestines  in  peritonitis 
are  usually  much  distended,  owing  to  paralysis. 

A  peculiar  form  of  peritonitis — the  acute  hemorrhagic — should  be 
mentioned.  Here  there  appears  to  be  a  subacute  type  of  inflammation, 
in  which  a  membranous  deposit  containing  abundant,  newly-formed 
capillaries  is  laid  down.  These  capillaries  readily  rupture  and  there 
is  usually  a  considerable  outpouring  of  blood  into  the  abdominal  cavity. 
The  condition  is  analogous  to  internal  hemorrhagic  pachymeningitis.1 

Chronic  Peritonitis. — Chronic  peritonitis  may  result  from  the  acute 
form,  which  has  undergone  a  series  of  relapses  or  a  gradual  amelioration 
in  its  severity,  or  may  develop  insidiously  from  the  first.  Like  acute 
peritonitis,  it  is  usually  at  first  local  and  is  in  almost  every  case  secondary 
to  disease  of  one  of  the  abdominal  viscera.  Exceptionally,  it  may  arise 
by  extension  from  one  of  the  other  serous  membranes. 

We  may  conveniently  divide  chronic  peritonitis,  when  well-developed, 
into  (1)  chronic  exudative  peritonitis,  in  which  there  is  a  considerable  out- 
pouring of  serous,  serofibrinous,  or  fibrinopurulent  exudation,  with  loose, 
plastic  adhesions,  fibrous  bands  being  few  or  absent;  (2)  chronic  exudative 
and  adhesive,  presenting  less  fluid  exudation,  but  with  somewhat  numerous 
and  firm  organizing  adhesions,  often  leading  to  sacculation  of  the  cavity; 
and  (3)  chronic  hyperplastic  peritonitis,  where  there  is  a  serofibrinous  or 
fibrinopurulent  exudation  of  greater  or  less  extent,  the  most  striking 
feature,  however,  being  the  production  of  isolated  plaques  or  continuous 
sheets  of  hyaline  material,  of  whitish  color  and  cartilaginous  appear- 
ance (hyaloserositis),  though  ordinary  dense  fibrous  adhesions  are  also 
numerous.2 

The  hyperplastic  form  is  much  rarer  than  the  other  two,  but  is  of 
considerable  interest.  The  lesions  may  be  confined  to  the  abdominal 
cavity  or  may  form  part  and  parcel  of  a  generalized  multiserositis.  The 
exudate,  at  first  fibrinous  or  fibrinopurulent,  gradually  undergoes  organ- 

1  For  a  review  of  the  literature  on  Peritonitis  the  reader  may  consult  von  Brunn, 
Centralbl.  f.  allg.  path.  Anat.,  12 : 1901 : 1.     For  bibliography:  Eichorst,  Eulenberg's 
Real  Encyclopedic. 

2  For  literature,  see  Hale  White,  Guy's  Hosp.  Rep.,  49  :  1895  :  1;  A.  O.  J.  Kelly, 
Trans.  Coll.  of  Phys.,  Philadelphia,  1902;    Nicholls,  Some  Rare  Forms  of  Chronic 
Peritonitis,  Jour.  Amer.  Med.  Assoc.,  40: 1903:696. 


510  THE  PERITONEUM 

ization  and  hyaline  degeneration,  so  that  the  viscera,  but  especially 
the  liver  and  spleen,  are  covered  with  a  thick,  pearly  white,  cartilaginous- 
looking  membrane,  which  has  been  compared  to  porcelain  or  the  icing 
of  a  cake  (Zuckergussorgane).  The  serosa  of  the  intestines  and  mesen- 
tery may  also  be  involved,  though  usually  to  a  less  extent.  The  omen- 
turn  is  commonly  found  to  be  thickened  and  converted  into  a  dense 
fibrous  cord  traversing  the  upper  part  of  the  abdomen.  The  mesentery 
may  also  be  shortened,  so  that  the  intestines  lie  in  a  ball  close  to  the 
vertebral  column.  Scattered  adhesions  may  be  found  here  and  there. 
There  may  be  a  relatively  large  effusion  of  fluid,  resembling  that  in 
ascites,  but  at  times  it  is  fibrinopurulent.  Occasionally  there  is  no  fluid 

FIG.  138 


Chronic  perihepatitis  with  hyaline  transformation  of  the  exudate  (hyaloserositis).  The 
Glisson's  capsule  is  thrown  into  folds  from  atrophy  of  the  liver  parenchyma.  Leitz  obj.  No.  7, 
without  ocular.  (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

exudate,  and  the  whole  abdominal  cavity  is  obliterated  by  the  adhesion 
of  the  visceral  and  parietal  layers  of  the  peritoneal  membrane.  Where 
the  capsule  of  the  liver  is  chiefly  involved  (perihepatitis),  the  physical 
signs  are  very  similar  to  although  not  identical  with  those  of  atrophic 
cirrhosis  of  the  liver.  Peritonitis  of  this  type  may  be  secondary  to  chole- 
lithiasis and  pericholecystitis  (Nicholls;  Hubler),  trauma  (Henoch), 
and  passive  congestion  (v.  Wunscheim).  Occasionally,  it  is  found  as  a 
result  of  tuberculosis  or  carcinosis  of  the  peritoneum.  Probably  in  all 
cases  infection  of  a  low  grade  of  virulence  is  the  direct  cause. 

Tuberculosis. — A  common  form  of  tuberculosis  of  the  peritoneum 
is  that  in  which  small  isolated  tubercles  are  found  scattered  over  the 
membrane,  chiefly  along  the  bloodvessels.  This  is  usually  a  manifesta- 


ABNORMAL  CONTENTS  OF  THE  PERITONEAL  CAVITY        51 1 

tion  of  a  generalized  miliary  infection.  The  tubercles  may  be  capped 
by  a  small  amount  of  fibrin,  and  there  maybe  slight  congestion  in  their 
neighborhood.  Local  peritonitis  is  also  met  with  in  connection  with 
tuberculous  ulceration  of  the  bowels.  Fluid  exudation  may  be  scanty. 
In  a  few  instances,  the  inflammation  assumes  the  hyperplastic  type, 
owing  to  the  production  of  a  serofibrinous  exudate  which  undergoes 
organization  and  hyaline  transformation  (Strajesko;1  Herrick;2  Nicholls3). 
A  few  scattered  areas  of  caseation  here  and  there  will  often  give  the 
clue  to  the  nature  of  the  affection.  A  third  variety  is  that  which  remains 
strictly  localized,  usually  to  the  region  of  the  appendix  and  cecum. 
There  may  be  simply  a  diffuse  thickening  of  the  intestinal  wall  at  this 
point  without  caseation,  or  there  may  be  numerous  fibrous  adhesions 
about  the  affected  part.  Sometimes,  also,  the  omentum  and  mesentery 
are  thickened  and  contracted.  The  tissue  hyperplasia  about  the 
cecum  may  be  so  great  that  cases  have  been  mistaken  for  carcinoma. 
Lartigau4  has  studied  this  form. 

In  tuberculosis  of  the  peritoneum  the  infection  may  be  hematogenous 
or  lymphogenous,  or,  again,  may  arise  by  extension  from  the  intestines, 
Fallopian  tubes,  or  pleural  cavities. 

Syphilis. — Little  or  nothing  is  definitely  known  with  regard  to 
syphilis  of  the  peritoneum.  Lancereaux5  believed  in  the  existence  of  a 
chronic  adhesive  and  membranous  peritonitis  due  to  syphilis,  occurring 
in  children  and  occasionally  in  adults.  Herringham6  records  two 
cases  of  chronic  peritonitis  with  perihepatitis,  which  probably  started 
from  a  gummatous  liver.  Probably,  all  such  cases  are  secondary  to 
syphilitic  disease  of  the  viscera.  Primary  gummas  of  the  peritoneum 
are  unknown. 


ABNORMAL  CONTENTS  OF  THE  PERITONEAL  CAVITY. 

The  abdominal  cavity  may  contain  serous  fluid,  inflammatory  exudates, 
blood,  bile,  or  gas. 

Pneumoperitoneum,  or  gas  in  the  peritoneal  cavity,  usually  results  from 
the  rupture  of  some  viscus  in  communication  with  the  outer  air,  such  as 
the  stomach  or  intestine;  less  often  from  the  action  of  gas-producing 
microorganisms,  notably  the  B.  Welchii  and  the  B.  coli. 

Besides  this,  the  contents  of  the  stomach  or  bowels,  gallstones,  urine, 
feces,  and  parasites  may  escape  into  the  peritoneal  cavity.  The  so-called 
"free  bodies"  are  derived  from  a  variety  of  sources,  appendices  epiploicse 
which  have  become  detached,  subserous  fibroids  of  the  uterus,  ovaries, 
and  mummified  or  calcified  embryos.  Swabs  and  gauze  pads  have 
occasionally  been  left  in  the  abdominal  cavity  by  mistake  in  the  course 
of  operations  in  this  region. 

1  Allg.  Wien.  med.  Zeit.,  4:  1902. 

2  Trans.  Chicago  Path.  Soc.,  April,  1902. 

3  Jour.  Amer.  Med.  Assoc.,  40:  1903:  696.       4  Jour.  Exper.  Med.,  6: 1901-05 : 23. 
5  Trans.  New  Syd.  Soc.,  1:  326.  6  St.  Earth.  Hosp.  Rep.,  1893:  1. 


512  THE  PERITONEUM 


PROGRESSIVE    METAMORPHOSES. 

Opaline  Plaques. — Small,  opaline  plaques  are  occasionally  found  on 
the  peritoneal  surface  as  a  result  of  chronic  irritation  or  slight  inflam- 
mation. They  occur  chiefly  on  the  liver  at  points  of  pressure  and  upon 
the  spleen.  The  spleen  may  be  covered  with  a  thin,  pearly  sheet,  or 
with  numerous  round,  elevated  spots,  looking  like  drops  of  white  wax 
or  paraffin.  Passive  congestion  of  the  spleen  seems  to  be  associated 
with  the  condition  in  many  cases.  Histologically,  the  patches  are 
composed  of  hyperplastic  connective  tissue  having  a  tendency  to  undergo 
hyaline  transformation. 

Tumors. — Apart  from  the  new-growths  originating  in  the  various  ab- 
dominal viscera  that  encroach  upon  the  cavity,  tumors  of  the  peritoneum 
are  most  often  situated  in  the  great  omentum,  the  retroperitoneal  con- 
nective tissue,  the  radix  mesenterii,  less  often  in  other  parts.  Among  the 
benign  growths  may  be  mentioned  lipoma,  fibroma,  myxoma,  chondroma, 
osteoma,  hemangioma,  and  lymphangioma.  Dermoids  and  teratomas 
also  occur.  '  . 

One  of  the  most  important  and  interesting  new-growths  is  the  retro- 
peritoneal  lipoma.  This  is  rarely  pure,  but  is  usually  formed  of  an  ad- 
mixture of  fatty,  fibrous,  and  mucinous  constituents.  The  growth  begins 
retroperitoneally,  generally  in  the  neighborhood  of  the  left  kidney, 
or  in  the  root  of  the  mesentery.  It  is  difficult  to  diagnosticate  and  gradu- 
ally increases  in  size,  leading  to  more  or  less  distension  of  the  abdomen. 
It  may  become  quite  enormous.  Waldeyer  has  recorded  one  sixty-three 
pounds  in  weight.  When  the  tumor  is  of  large  size,  the  abdomen  is 
swollen,  soft,  and  fluctuating,  giving  the  sensation  of  being  filled  with 
fluid.  A  dry  tap,  however,  would  exclude  ascites  and  should  always 
lead  one  to  suspect  retroperitoneal  lipoma.  A  coil  of  intestine  can  often 
be  made  out  on  percussion  crossing  obliquely  over  the  front  of  the  tumor. 
Cases  are  generally  inoperable  when  discovered  and  invariably  end  fatally, 
partly  owing  to  the  enormous  pressure  and  partly  to  a  sarcomatous  trans- 
formation to  which  this  form  of  tumor  seems  particularly  prone.  One 
of  us  (J.  G.  A.1)  has  collected  forty  examples  from  the  literature  and  has 
recorded  two  other  cases,  in  one  of  which  the  tumor  weighed  forty-five 
pounds.  These  growths  probably  originate  from  cells  of  connective-tissue 
origin  which  have  become  nipped  in  between  the  renculi  in  the  process 
of  development. 

Another  important  primary  growth,  although  not  at  all  common,  is  the 
endothelioma.  This  occurs  in  the  form  of  multiple  small  plaques  or 
warty  excrescences,  or,  again,  diffusely  in  more  or  less  extensive  sheets. 
It  begins  in  a  proliferation  of  the  lining  endothelium  of  the  cavity  or, 
possibly,  from  the  perivascular  lymphatics.  The  condition  is  some- 
times associated  with  the  effusion  of  fluid,  occasionally  bloody,  together 
with  the  deposit  of  fibrin,  so  that  it  simulates  a  chronic  productive 

,  x  Montreal  Med.  Jour.,  25:1897:529  and  620. 


CYSTS 


513 


inflammation.  Metastases  are  not  common.  Histologically,  the  growth 
resembles  an  alveolar  carcinoma. 

Matas1  has  recorded  a  curious  case  of  primary  myxosarcoma  of  the  great 
omentum,  which  produced  extensive  secondary  growths  throughout  the 
peritoneum  with  a  mucoid  ascites. 

Secondary  malignant  growths  are  common,  and  here,  again,  the  omen- 
turn  is  most  likely  to  be  involved.  It  may  be  converted  into  a  short, 
thick,  hard  cord,  running  transversely  across  the  upper  part  of  the  abdo- 


FIG.  139 


Semidiagrammatic  cross-section  through  a  perirenal  lipoma  at  the  level  of  the  renal  vessels, 
seen  from  above.  The  perirenal  and  retrorenal  fascia  unite  to  form  the  transversal  is  fascia. 
The  whole  intestinal  tract  lies  in  front  of  the  perirenal  fascia,  a,  descending  colon;  b,  perirena 
fascia;  c,  peritoneum;  d,  retrorenal  fascia;  e,  small  intestine;  /,  superior  mesenteric  artery;  g, 
duodenum;  h,  ascending  colon.  (Reynolds  and  Wadsworth.) 

men.  In  the  case  of  carcinoma  the  primary  growth  is  usually  situated 
in  the  stomach,  gall-bladder,  pancreas,  intestine,  uterus,  ovaries,  and 
prostate.  Multiple  melanotic  sarcomas  are  also  found,  arising  by  metas- 
tasis from  melanotic  sarcomas  of  the  choroid  of  the  eye  or  of  the  skin. 

Cysts. — The  lymphangioma  is  said  sometimes  to  assume  the  form  of 
a  cystoma.  Other  cysts  are  the  serous,  chylous,  hemorrhagic,  dermoid, 
and  parasitic. 

1  Phila.  Monthly  Med.  Jour.,  December,  1899, 


33 


SECTION   IV. 
THE  NERVOUS  SYSTEM. 


CHAPTER    XXV. 

THE  STRUCTURE  OF  THE  NERVOUS  SYSTEM  AND  ITS  BEARING  UPON 

DISEASE. 

THE  nervous  system  in  man  is  composed  of  nerve  cells  and  fibers  sup- 
ported by  a  connective-tissue  stroma  of  glia  cells  and  fibers.  These 
nerve  cells,  lying  in  the  gray  matter  of  the  cerebrum  and  cerebellum, 
mid-brain,  the  medulla,  and  cord,  form  centres  from  which  run  fibers 
connecting  the  centres  with  each  other  and  with  the  periphery  of  the 
body.  The  fibers  are  simply  conductors,  centripetal  or  centrifugal, 
of  sensory  or  motor  excitations.  The  centres  are  cells,  or  aggregations 
of  many  cells,  in  which  force  is  stored  up  or  transformed,  so  that  a  sensory 
stimulus  is  able  to  initiate  a  motor  excitation.  Thus,  the  entire  nervous 
system  is  a  reproduction  on  a  large  scale  of  its  constituent  elements, 
the  neurones. 

The  neurone  is  formed  of  a  cell  with  its  prolongations,  the  dendrites, 
and  the  axis-cylinder  processes.  Each  neurone  is  anatomically  inde- 
pendent. The  cells  are  variously  formed  masses  of  protoplasm,  with 
a  large,  round  nucleus  containing  a  large  nucleolus.  By  means  of  special 
methods  of  staining  (Nissl),  the  cell  protoplasm  of  the  majority  of  nerve 
cells  (Nissl's  somatochromes)  may  be  seen  to  be  studded  with  irregularly 
shaped  granules  (chromophilic  or  tigroid  bodies;  Nissl  bodies),  which 
are  found  also  in  the  protoplasmic  dendrites,  but  are  not  present  in  the 
cone  of  origin  of  the  axis-cylinder,  nor  in  the  axis-cylinder  itself.  Other 
cells,  as  some  in  the  olfactory  system,  contain  none  of  these  granules 
(Xissl's  caryochromes).  Certain  facts,  such  as  its  diminution  under 
overactivity  and  fatigue  and  its  action  under  certain  pathological  condi- 
tions, make  it  probable  that  this  chromophilic  substance  represents  a 
nutritive  element  of  the  neurone  derived,  it  may  be,  as  a  product  of 
nuclear  activity,  its  staining  reactions  being  not  dissimilar  to  those  of  the 
nuclear  chromatin. 

By  means  of  other  special  stains  (Apathy,  Bethe,  Beelchowski, 
Cajal)  the  spaces  between  these  tigroid  bodies  may  be  seen  to  be  occupied 
by  fine  fibrils,  which  form  a  network  in  the  body  of  the  cell  surrounding 
the  nucleus,  and  can  be  followed  out  into  the  dendrites  on  the  one  hand, 
and  the  axis-cylinder  on  the  other. 


516  THE  STRUCTURE  OF  THE  NERVOUS  SYSTEM 

The  dendrites  are  protoplasmic  processes,  with,  in  many  cases,  very 
rich  arborizations,  large  at  their  origin  from  the  cell,  gradually  becoming 
thinner  like  the  branches  of  a  tree.  They  are  simply  an  expansion  of 
the  cell-body,  serving  to  increase  and  facilitate  its  connections  with 
neighboring  cells.  The  axis-cylinder  process  is  usually  single  and  of 
regular  caliber  from  its  origin  to  its  termination,  although  some  cells, 
e.  g.,  those  of  the  posterior  ganglia,  have  two  axis-cylinders.  It  may 
give  off  collaterals  at  right  angles  which  have  the  same  characters  as  it 
has  itself.  On  leaving  the  gray  matter  of  the  centre  the  axis-cylinder  or 
axone  becomes  invested  with  a  myelin  sheath,  and  it  is  collections  of 
these  axis-cylinders  with  their  sheaths  which  form  the  white  matter  of 
the  brain  and  cord.  Clothed  in  the  sheath  of  Schwann,  they  form  the 
peripheral  nerves.  Some  nerves  (sympathetic  system)  have  the  sheath 
of  Schwann  without  the  myelin  covering. 

The  axone  is  made  up  of  a  bundle  of  fibrils,  and  on  reaching  its 
destination  splits  up  into  a  terminal  arborization  about  a  second  cell, 
terminating  in  small,  button-like  bodies  (end  feet),  which  lie  in  contact 
with  the  cell  walls. 

The  neurone  in  this  simple  form,  as  an  independent  unit,  is  not  without 
detractors,  among  whom  may  be  numbered  such  authorities  as  Nissl, 
Apathy,  Bethe,  Durante,  etc.  Apathy,  by  means  of  his  special  method 
of  staining,  has  studied  the  neurofibrils  very  closely,  and  asserts  that  they 
act  in  two  ways.  Certain  ones  penetrate  into  the  ganglion  cell  and  are 
then  resolved  into  the  elementary  fibrils,  anastomosing  freely  among 
themselves  and  thus  forming  an  intracellular  network,  then  reuniting  to 
form  the  primitive  fibrils  of  the  axone.  The  second  class,  in  entering 
the  ganglion,  do  not  penetrate  into  the  cells  of  the  ganglion,  but  resolve 
themselves  directly  into  elementary  fibrils  and  form  an  extracellular 
network.  Later,  they  reunite  into  primitive  fibers  and  enter  the  cells 
of  the  ganglion. 

Apathy  asserts  that  the  motor  fibers  do  not  commence  in  the  cells  of 
the  ganglion ;  they  are  nothing  but  the  continuation  of  the  sensory  fibrils 
after  the  interposition  of  the  nervous  network,  the  ganglion  cells  being 
intercalated  in  the  course  of  the  conducting  fibers  like  the  battery  in  a 
network  of  telegraph  wires.  In  other  words,  the  fibrils  do  not  commence 
nor  terminate  in  any  place,  neither  the  sensory  nor  motor,  neither  at  the 
periphery  nor  in  the  centres,  but  form  a  great  system  of  continuous 
conduction  paths,  just  as  in  the  circulation  the  arteries  through  the 
capillary  network  are  continuous  with  the  veins.  Bethe  agrees  with 
Apathy  in  these  statements,  but  gives  to  the  extra-cellular  network  a 
greater  emphasis.  He  states  that  the  greater  number  of  fibrils  go  from 
one  neurone  to  another;  the  sensory  become  motor  without  passing 
through  the  cells  of  the  ganglion. 

Apparently,  however,  the  death  blow  was  given  to  the  neurone  theory 
by  the  discovery  by  Bethe,  and  later  by  Raiman,  etc.,  of  what  is  considered 
by  them  as  autogenous  regeneration  in  the  peripheral  part  of  a  cut  nerve. 
These  observers  maintain  that,  developmentally,  the  peripheral  nerve 
fibers,  including  their  axones,  arise  in  the  embryo  by  a  fusion  of  long 


THE  STRUCTURE  OF  THE  NERVOUS  SYSTEM  517 

chains  of  cells  placed  end  to  end.  This  pericellular  or  catenary  theory 
of  the  origin  of  peripheral  nerves  has  been  extended  by  some  even  to  the 
dendrites  and  axones  of  the  cells  of  the  central  nervous  system. 

Wilhelm  His  (1886)  was  the  first  to  maintain  from  his  embryological 
studies  that  the  neurone  was  an  independent  unit,  every  nerve  fiber 
being  a  process  from  an  embryonic  nerve  cell  (neuroblast).  The  re- 
searches of  Golgi  supported  this  view,  and  the  recent  work  of  R.  G. 
Harrison1  has  most  satisfactorily  confirmed  it.  He  found  that  by  cutting 
away  a  thin  strip  on  the  back  of  an  embryo  tadpole  he  got  some  to 
grow  which  at  the  end  of  a  week  had  no  sensory  ganglia  nor  sensory 
nerves,  although  they  had  motor  nerves,  but  these,  instead  of  showing  cell 
chains  along  their  course,  as  under  normal  conditions,  appeared  as 
naked,  non-nucleated  fibers,  which  could  be  traced  as  such  all  the  way 
from  the  spinal  cord  to  the  extreme  ventral  part  of  the  musculature, 
showing  that  the  peripheral  spinal  nerves  may  develop  in  the  absence  of 
these  sheath  nerves. 

As  for  the  question  of  autogenous  regeneration  in  the  distal  part  of 
a  sectioned  nerve,  this  is  by  no  means  proved,  and  since  Harrison's 
work,  above  quoted,  we  would  not  expect  it. 

Langley  and  Anderson2  assert  that  if  anastomosis  with  other  nerves 
in  the  limb  and  all  possibility  of  outgrowth  from  the  central  stump  be 
prevented  no  autoregeneration  will  occur.  Lugaro3  also  has  recently 
published  the  results  of  a  most  careful  series  of  experiments,  with  the 
following  conclusions : 

1.  In  young  dogs,  in  wrhom  the  lumbosacral  cord  and  the  corresponding 
posterior  ganglia  had  been  extirpated,  no  autogenous  regeneration  of  the 
nerve  fibers  in  the  corresponding  peripheral  nerves  could  be  made  out. 

2.  Some  non-myelinated  fibers  were  found  in  the  peripheral  nerves 
which  were  proved  to  come  from  the  sympathetic  ganglia. 

Finally,  much  doubt  exists  in  the  minds  of  many  competent  observers 
as  to  the  existence  of  the  extracellular  neurofibrillary  network  that 
Bethe  lays  so  much  stress  on,  in  spite  of  his  brilliant  experimental  evidence 
to  the  contrary.  This  experimental  evidence,  it  must  be  remembered, 
has  not  been  confirmed.  Cajal's  stain  and  the  new  method  of  Donaggio, 
which  show  the  neurofibrils  very  beautifully  but  do  not  stain  the  neurog- 
lia,  demonstrate  the  intracellular  network  perfectly,  but  give  no  evidence 
of  an  extracellular  one.  Certainly,  the  dependence  of  one  neurone  on 
another  is  not  complete.  Clinically,  a  lesion  is  often  limited  to  one  system, 
so  that  if  there  be  a  histological  continuity  between  the  neurones  of  the 
various  orders,  there  is  certainly  a  physiological  and  even  a  pathological- 
anatomical  independence  from  the  point  of  view  of  propagation  of  the 
lesions;  and,  as  we  have  seen,  there  is  an  embryological  independence. 

These  neurones,  then,  are  gathered  together  into  groups  to  form 
systems,  which  subserve  different  functions,  each  of  these  different 

1  Reprint  from  Sitz,  d.  Neiderrhein  Gesell.  Nat.  u.  Heilk.,  Bonn,  1904;  Nerv. 
Versuche  u.  Beobactungen  ueber  die  Entwickelung  der  peripheren  Nerven. 

2  Autogenic  Regeneration  in  the  Nerves  of  Limbs,  Jour,  of  Phys.,  31: 1904:418. 

3  Neurolog.  Centralbl.,  September,   1906:786. 


518  THE  STRUCTURE  OF  THE  NERVOUS  SYSTEM 

systems  being  made  up  of  several  relays  of  groups  or  neurones.  Barker 
has  classified  them  as  follows: 

1.  Neurones  connecting  the  sense  organs  of  the  body  with  the  central 
nervous  system  (peripheral  centripetal  neurones;  sensory  neurones  of 
the  first  order;  sensory  protoneurones). 

2.  Neurones  within  the  central  nervous  system,  connecting  the  end- 
stations  of  the  axones  of  the  peripheral  centripetal  neurones  with  other 
portions  of  the  central  nervous  system;  and  neurones  which,  in  turn, 
connect  the  end-stations  of   the  latter  with  still  higher  portions  of  the 
central  system  (sensory  neurones  of  the  second  order  and  of  higher  orders). 

3.  Neurones  connecting  the  central  nervous  system  with  the  volun- 
tary muscles  of  the  body  (lower  motor  neurones). 

4.  Neurones  within    the  central    nervous    system  which   enter  into 
conduction  relation  with  the  lower  motor  neurones  and  throw  the  latter 

under  the  influence  of  other  centres. 

FlG-  14°  Neurones  connecting  the  pallium, 

cerebellum,  etc.,  with  the  lower 
motor  neurones. 

5.  Projection,  commissural  and 
association  neurones  of  the  telen- 
cephalon. 

Neuroglia.— Neuroglia  is  the  con- 
nective tissue  of  the  central  nervous 
system  filling  in  the  spaces  between 
the  nerve  elements  and  forming  a 
support  for  these  and  the  vessels. 
It  takes  the  place  of  the  parenchy- 
matous  tissue,  if  this  be  destroyed, 

Glial  cells  with  multiple  processes,  from  a  ag  doeg  the  £brous  tjssue  Jn  other 
case  of  congenital  multiple  ghomatosis  of  the  ,  . 

brain.    (Stertz.)  organs.      In   the   adult,  two  main 

types  of  neuroglia  cells  are  found, 

viz.,  spider  cells,  having  a  round  nucleus  and  straight  unbranching 
processes,  and  other  cells  with  thicker  processes  profusely  branching;  the 
former  are  found  chiefly  in  the  white  matter,  the  latter  in  the  gray  matter 
about  the  bloodvessels. 

It  has  been  asserted  by  Metchnikoff  and  others  that  in  inflammatory 
and  degenerative  conditions,  the  neuroglial  cells  have  a  phagocytic  action 
on  the  degenerated  tissue.  Marinesco  maintained  that  whenever  the 
achromatic  substance  of  the  nerve  cell  suffers  injury  there  is  a  stimula- 
tion of  the  surrounding  neuroglia  cells  to  phagocytic  activity.  Cer- 
tainly, where  there  has  been  a  severe  lesion  of  the  nervous  system,  the 
neuroglia  cells  hypertrophy  and  proliferate  secondarily  and  are  fre- 
quently found  increased  in  number  about  the  degenerating  nerve  cells. 
But  it  is  doubtful  if  this  is  evidence  of  a  phagocytic  action.  Cerletti  in 
his  experiments  could  not  satisfy  himself  of  their  phagocytic  character. 
In  a  more  recent  communication  Marinesco1  limits  the  phagocytic  action 

1  La  Semaine  Med.,  27:1907:145. 


NEUROGLIA  519 

of  the  "satellite"  (glial)  cells  to  the  removal  of  dead  neurones  and  desig- 
nates it  necrophagy  rather  than  neuronophagy. 

There  are  not  a  great  variety  of  pathological  changes  seen  in  the  neu- 
rone. Under  the  action  of  destructive  agencies  affecting  the  cell  or  its 
axis-cylinder,  a  series  of  changes  in  structure  may  occur,  which  may  go 
on  to  absolute  degeneration  and  necrosis.  In  the  early  stages  of  these 
retrogressive  changes,  which  are  confined  to  the  finer  histological  ele- 
ments of  the  cell  and  do  not  affect  the  nucleus,  regeneration  of  these 
elements  and  return  to  normal  appearance  and  function  are  quite  possi- 
ble. It  is  only  when  the  disease  has  advanced  far  enough  to  affect  the 
nucleus  that  the  cell  is  doomed.  Our  knowledge  of  these  degenerative 
changes  is  confined  to  what  occurs  in  the  large  somatochrome  cells  of  the 
motor  nuclei,  the  spinal  ganglia,  etc.  These  changes  may  be  summed 
up  as  follows: 

1.  A  swelling  of  the  cell-body  with  structural  changes  in  the  chromatin 
and  fibrillary  elements. 

2.  Ectopia  nuclei;  the  nucleus  no  longer  remains  central,  but  wanders 
toward  the  periphery. 

3.  Vacuolization  of  the  cell-body  and  granular  degeneration  of  fibrils. 

4.  Total  necrosis. 

In  the  first  stage  there  is  a  homogeneous  swelling  of  the  cell-body,  the 
Nissl  bodies  become  irregular  in  shape  and  size,  and,  later,  are  seen  to 
be  broken  up  into  fine  dust-like  particles.  This  change  commences  in 
some  cases  in  the  perinuclear  part  of  the  cell;  in  others  it  begins  about 
the  periphery,  or  it  may  appear  as  a  diffuse  chromatolysis. 

Simultaneously  with  these  changes,  or  probably  a  little  later,  definite 
changes  occur  in  the  fibrillary  network  of  the  cell.  It  becomes  less  dis- 
tinct and  the  fibrils  themselves  less  regular.  Up  to  this  stage  regeneration 
is  possible,  but  if  the  process  advance,  the  nucleus  no  longer  holds  its 
central  position,  but  becomes  eccentric;  at  the  same  time,  the  fibrils 
undergo  a  process  of  granular  disintegration  and  fragmentation,  vacuoles 
appear  in  the  body  of  the  cell,  and  the  protoplasm  has  a  granular  appear- 
ance. The  nucleus  bulges  out  the  periphery  of  the  cell  and  is  finally 
extruded,  and  the  cell  with  its  axone  breaks  up  and  is  quickly 
absorbed. 

Chromatolysis  appears  to  be  the  primary  stage  of  the  retrogressive 
process.  It  occurs  as  an  effect  of  overactivity  and  fatigue,  in  toxic  blood 
conditions,  febrile  conditions,  after  section  of  the  axone,  etc.,  but,  under 
favorable  circumstances,  the  process  may  stop  here  and  a  more  or  less 
rapid  return  to  the  normal  occur. 

Specific  changes  in  the  cell  dependent  on  the  etiological  factor  have 
been  described.  Thus,  Marinesco  described  (1)  alterations  due  to 
indirect  traumatism;  (2)  primary  alterations  in  the  cell  due  to  direct 
action,  mostly  toxic,  or  of  nutritive  disturbances.  The  former,  e.  g., 
after  section  of  the  axone,  consists  in  central  chromatolysis  with  displace- 
ment of  the  nucleus  to  the  periphery.  The  latter,  while  they  may  involve 
the  chromatic  substance  of  the  whole  cell  diffusely,  are  most  marked 
in  the  more  peripheral  zone,  and  are,  therefore,  represented,  at  least 


520  THE  STRUCTURE  OF  THE  NERVOUS  SYSTEM 

temporarily,  by  a  peripheral  chromatolysis  without  displacement  of  the 
nucleus. 

But  these  characteristics  are  by  no  means  always  distinct.  Other 
observers  have  described  specific  alterations  in  the  cells,  dependent  on 
the  nature  of  the  poison,  maintaining  that  in  the  earlier  stages,  at  least, 
of  acute  arsenical  poisoning,  the  picture  is  quite  different  from  that 
which  is  seen  in  acute  phosphorus  poisoning.  In  the  later  stages,  and 
in  chronic  cases,  these  specific  peculiarities  are  lost. 

The  structural  changes  in  the  nerve  cell  following  solution  of  continuity 
in  the  nerve  fibers  are  practically  the  same  as  those  which  follow  the 
action  of  poisons,  and  may  go  on  to  total  destruction  of  the  cell.  The 
intensity  of  the  reaction  depends  on  the  severity  of  the  lesion,  being 
greater  if  the  nerve  is  cut  than  when  merely  compressed,  while  a  still 
more  intense  reaction  follows  if  the  nerve  be  evulsed. 

After  section,  or,  better  still,  evulsion,  of  a  motor  nerve,  the  cells  first 
show  an  increase  in  volume;  they  may  become  almost  double  in  size  and 
rounder,  and  the  fibrillary  network  shows  certain  indefinite  structural 
changes.  The  tigroid  bodies  break  up  into  fine,  dust-like  particles, 
first,  perhaps,  in  the  region  of  the  nucleus,  later  about  the  margin; 
then  the  nucleus  may  wander  toward  the  periphery. 

Waller  was  the  first  to  point  out  that  the  interruption  in  continuity 
of  the  nerve  fibers,  whatever  be  the  cause,  whether  an  incised  wound  or 
a  vascular  lesion,  is  followed  by  structural  changes  of  a  degenerative 
character  in  the  fiber  distal  to  the  lesion;  that  is,  in  the  part  separated 
from  its  parent  cell,  or  more  exactly,  as  Cameron1  maintains,  from  its 
parent  nucleus,  since  the  nucleus  is  the  nutritive  centre  for  the  nerve  cell. 
This  is  termed  secondary  degeneration.  Thus,  if  we  have  a  lesion  of  the 
motor  fibers  in  the  internal  capsule  of  the  brain,  we  get  secondary  degener- 
ation in  the  direct  and  crossed  pyramidal  tracts,  a  descending  degenera- 
tion. If  the  lesion  be  in  the  posterior  roots  between  the  ganglia  and  the 
cord,  or  in  the  course  of  these  fibers  in  the  posterior  columns,  we  have  an 
ascending  degeneration  in  the  posterior  columns  of  the  cord  up  to  their 
terminal  arborization  about  the  cells  of  the  second  relay  in  the  nuclei 
of  Goll  and  Burdach. 

These  retrogressive  changes  begin  immediately  and  apparently  simul- 
taneously in  the  whole  extent  of  the  peripheral  fiber,  progressing  more 
rapidly  the  farther  away  from  the  nutritive  centre  the  lesion  is.  First, 
the  myelin  sheath  swells,  then  it  breaks  up  into  large  and  small  globules, 
giving  the  fibers  a  varicose  appearance.  The  globules  are  of  a  fatty 
nature  and  stain  black  with  osmic  acid  (Marchi's  method).  These  fat 
particles  are  carried  away  by  leukocytes  and  probably  other  migrating 
cells  which  originate  from  the  vascular  connective-tissue  elements. 

Simultaneously  with  these  changes  in  the  myelin  there  is  swelling  of 
the  axis-cylinders  with  a  varicose  appearance  of  the  neurofibrils,  which 
later  become  finely  granular.  They  soon  break  up  and  are  absorbed, 
their  place  being  taken  by  proliferated  glial  tissue. 

1  Brain,  1906:  332. 


ABIOTROPHY  AND  EXHAUSTION  CONDITIONS  521 

Secondary  Atrophy  of  the  Second  Order. — Secondary  atrophy  of 
a  nerve  fiber  may  occur  as  a  phase  in  secondary  degeneration  following 
separation  from  the  parent  cell.  It  may  occur  also  in  the  central  stump 
which  still  remains  in  connection  with  the  cell,  i.  e.,  a  cellulopetal  atrophy. 
Besides  these  forms,  a  definite  reduction  in  volume  of  the  greater  number 
of  the  fibers  in  a  tract  may  occur  even  when  there  is  no  interruption  in 
their  continuity,  when  they  are  in  close  anatomical  and  physiological 
connection  with  centres  which  have  undergone  secondary  degeneration. 
This  has  been  termed  by  v.  Monakow,  secondary  atrophy  of  the  second 
order.  We  would  prefer  to  term  it  the  atrophy  of  physiological 
inactivity  (vol.  i,  p.  802),  or  disuse  atrophy.  Thus,  we  have  changes  of 
the  neurone  of  the  second  order  following  destruction  of  the  neurone  of 
the  first  order,  and  vice  versa. 

Regeneration,  in  the  sense  of  the  formation  of  new  nerve-elements, 
probably  never  occurs  in  the  central  nervous  system.  The  regeneration 
of  the  chromatophilic  granules  has  been  mentioned.  In  the  peripheral 
nerves,  degeneration  always  occurs  in  the  part  distal  to  the  section  from 
the  parent  cell  before  regenerative  processes  begin.  This  consists  in 
the  growth  of  axis-cylinder  processes  out  from  the  central  end  which  push 
across  the  cicatrix  and  penetrate  into  the  peripheral  segment.  Cajal 
describes  the  growing  extremity  of  the  young  fiber  as  possessing  a 
11  terminal  ball,"  which,  if  it  becomes  impacted  in  one  of  the  tissue  inter- 
stices, may  become  enormously  swollen  and  enlarged.  (Vol.  i,  p.  575.) 

Simple  atrophy  of  the  neurone  may  occur  (1)  in  chronic  conditions  where 
a  slowly  acting  toxin  circulating  in  the  system  causes  an  impairment  of 
nutrition  in  the  cell,  not  sufficient,  perhaps,  to  cause,  in  the  first  place, 
very  definite  structural  changes  in  it,  but  just  sufficient  to  prevent  it 
maintaining  its  long  nerve-processes,  which,  therefore  degenerate; 
this  degeneration  commences  at  the  extreme  periphery  of  the  axis-cylinder; 
later  on  the  cell  itself  atrophies.  (2)  The  same  thing  occurs  in  condi- 
tions in  which  there  is  an  inherent  congenital  lack  of  vitality  in  the 
neurone,  in  conditions  of  abiotrophy. 

Abiotrophy  and  "Exhaustion"  Conditions. — As  we  have  already 
seen,  fatigue  and  exhaustion  of  a  neurone  are  characterized  anatomically 
by  a  disappearance  of  the  chromatolytic  bodies  in  the  nerve  cell  and 
degenerative  changes  in  the  myelinated  fibers.  According  to  the  well- 
known  hypothesis  of  Weigert  and  Roux,  the  tissues  of  an  organ  are 
normally  in  a  state  of  equilibrium,  and  when  one  degenerates  its  place 
is  taken  by  the  surrounding  tissue;  if  one  becomes  weakened,  the  energy 
of  growth  of  its  neighbors  tends  to  crush  it  out. 

This  condition  may  be  brought  about  either  by  increased  consumption 
of  the  nutritive  elements  of  the  cell,  or  by  a  diminution  of  the  reparative 
power  of  the  cell,  and  is  anatomically  recognizable  by  the  disappearance 
of  cell  and  fibers  and  the  secondary  overgrowth  of  glial  tissue  filling  in 
the  space. 

In  such  affections  as  occupation  palsies,  we  have  this  state  of  an 
abnormal  demand  on  the  normal  tracts  or  on  the  normal  reparative  power 
of  the  cells.  In  other  cases,  we  may  have  a  reparative  power,  relatively 


522  THE  STRUCTURE  OF  THE  NERVOUS  SYSTEM 

insufficient  for  the  normal  functioning  of  the  cell.  This  is  usually  due 
to  the  action  of  toxins  in  the  blood,  alcohol,  lead,  syphilis,  etc.,  and 
the  result  varies,  depending  upon  whether  the  toxin  has  a  selective 
action  on  any  particular  part  of  the  nervous  system  and,  again,  on  the 
relative  activity  of  the  various  systems  of  neurones.  Among  diseases 
of  this  nature  Edinger  has  classed  peripheral  neuritis,  tabes  dorsalis, 
lead  palsy,  subacute  combined  sclerosis,  etc. 

Or,  again,  individual  systems  are  congenitally  too  delicate  to  carry 
out  their  normal  function  during  the  usual  span  of  life.  There  is  ap- 
parently an  inherent  lack  of  vital  nutrition  in  certain  systems  of  cells, 
so  that  they  gradually  die — very  much  like  a  plant  when  the  nutrition 
of  its  roots  is  interfered  with,  the  distal  parts  fading  first — and  their  place 
is  taken  by  glial  tissue.  To  this  condition  Sir  William  Gowers1  has 
given  the  name  "abiotrophy."  (Vide  vol.  i,  p.  809.)  It  is  seen  in  several 
members  of  a  family  being  affected  similarly  without  any  apparent 
sufficient  cause.  The  various  types  of  Friedreich's  ataxia,  the  family 
form  of  primary  optic  atrophy,  peroneal  type  of  muscular  atrophy, 
amyotrophic  lateral  sclerosis,  etc.,  are  all  included  under  this  category. 

1  Lancet,  London,  1:1902:1003. 


CHAPTER    XXVI. 

THE  BRAIN. 
CONGENITAL  ANOMALIES. 

THE  central  nervous  system  is  formed  by  the  invagination  of  the 
superior  germinal  layer  of  the  embryo.  Thus,  there  is  produced  a  shallow 
furrow,  the  medullary  groove,  running  axially  along  the  dorsal  aspect. 
The  cells  along  the  margin  of  this  proliferate,  so  that  two  elongated 
ridges,  the  medullary  folds,  arise,  which  gradually  meet  and  coalesce, 
forming  the  medullary  canal.  Later,  the  medullary  tube  separates  from 
the  superficial  ectoderm  and  becomes  an  independent  structure. 

The  primitive  brain  consists  in  a  series  of  vesicles  or  dilatations  of 
the  anterior  end  of  the  medullary  canal,  while  the  rest  becomes  the  cord. 
The  lumen  of  the  primitive  medullary  tube  persists  throughout  life  as 
the  ventricles  of  the  brain  and  the  central  canal  of  the  cord.  It  is  lined 
by  a  membrane,  the  ependyma,  which  is  composed  of  a  single  layer  of 
ciliated  epithelial  cells,  and  a  supporting  structure  of  modified  glia. 

There  are  three  chief  vesicles  from  which  the  brain  is  derived,  forming, 
respectively,  the  fore-brain,  the  mid-brain,  and  the  hind-brain.  The 
first  is  subdivided  into  two  parts,  the  prosencephalon  proper  and  the 
thalamencephalon.  From  the  former  arise  the  lateral  ventricles,  the 
cerebral  hemispheres,  the  olfactory  bulbs,  and  the  corpus  callosum. 
The  second  vesicle  forms  the  third  ventricle,  the  aqueduct  of  Sylvius, 
the  nervous  part  of  the  eye,  the  epiphysis,  and  hypophysis,  the  optic 
thalami,  the  corpora  quadrigemina,  and  the  crura.  The  third  primary 
vesicle  is  divided  into  two  parts,  the  epencephalon,  which  goes  to  form 
the  pons  and  cerebellum,  and  the  mesencephalon,  forming  the  medulla 
oblongata.  The  fourth  ventricle  is  derived  from  both  portions. 

In  the  production  of  anomalies  of  development  it  is  a  general  rule  that 
those  structures  which  go  to  form  the  most  highly  differentiated  portions 
of  the  brain  are  most  likely  to  be  involved.  Therefore,  we  find  anomalies 
most  often  and  most  extreme  in  the  fore-brain,  less  often  in  the  hind- 
brain,  and  very  exceptionally  in  the  mid-brain. 

Anomalies  of  development  may  be  conveniently  and  naturally  classi- 
fied under  the  headings  of  (1)  Agenesy,  (2)  Hypoplasia,  (3)  Hyper- 
plasia,  and  (4)  Heteroplasia. 

Agenesy. — By  the  term  agenesy  we  understand  such  conditions  as 
are  brought  about  by  failure  of  the  medullary  canal  to  close,  or  the  lack 
of  formation  of  certain  parts  of  the  nervous  system.  It  is  not  always 
possible  to  draw  the  line  between  hypoplasia,  in  which  the  parts  are 
formed  but  lag  behind  in  growth  and  development,  and  agenesy,  for 


524 


THE  BRAIN 


the  two  conditions  are  often  associated.  It  is  to  be  observed  that  we  have 
to  do  with  two  main  types  in  agenesy,  one  in  which  the  defect  involves 
the  bony  structures  as  well  as  the  nervous  elements,  and  the  other,  in 
which  the  abnormality  is  confined  to  the  nervous  system. 

Perhaps  the  most  interesting  and  important  anomaly  is  that  in  which 
the  medullary  groove  fails  to  close  posteriorly.  Here,  the  vertebral 
arches,  the  muscles,  and  integument  of  the  back  are  not  formed,  so  that 
there  is  a  broad  fissure  along  the  dorsal  aspect  of  the  head  and  trunk. 
On  the  exposed  surface  there  may  be  recognized  rudimentary  nerve 
substance,  covered  with  cylindrical  epithelium,  which  corresponds  to 
that  ordinarily  lining  the  ventricles  and  neural  canal,  and  the  anterior 
portion  of  a  highly  vascularized  pia-arachnoid  and  dura.  The  anterior 
portion  of  the  brain  is  usually  fairly  well  developed,  but  the  base  of  the 


FIG.  141 


FIG.  142 


Spina  bifida. 


(From  the  Surgical  Clinic  of  the  Montreal 
General  Hospital.) 


Anencephaly. 


skull  and  the  spinal  column  are  abnormally  curved.  This  condition 
is  called  craniorhachischisis.  Frequently,  the  malformation  is  not  so 
extensive,  affecting  only  the  head,  cranioschisis,  or  the  cord,  rhachischisis 
(spina  bifida,  vide  vol.  i,  p.  246). 

In  cranioschisis  (hemicephaly ;  acrania)  the  vault  of  the  skull  is  com- 
pletely or  partially  defective,  giving  to  the  head  of  the  foetus  a  character- 
istic cat-like  or  toad-like  appearance.  The  brain  substance  may  be 
absent  (anencephaly),  or  present  in  rudimentary  form.  Several  varieties 
of  this  malformation  may  occur. 

In  some  cases  the  defect  of  the  skull  is  not  so  extensive,  but  is  limited 
to  particular  localities,  while  the  integument  is  almost,  if  not  entirely, 
closed  in.  In  this  way  is  formed  a  pouch  or  sac,  which  contains,  accord- 
ing to  circumstances,  a  diverticulum  of  the  membranes  together  with 
fluid  (meningocele),  a  portion  of  the  brain  (encephalocele),  or  both 


CONGENITAL  ANOMALIES 


525 


(meningo-encephalocele ;  hernia  cerebri).  The  most  common  situation 
is  in  the  median  line,  and  usually  in  the  occipital  region  (meningo- 
encephalocele  occipitalis),  or  at  the  glabella  (meningo-encephalocele  sin- 
cipitalis,  etc.).  Occasionally,  the  defects  are  found  at  the  vertex  or  the 
lateral  and  inferior  portions  of 
the  cranium.  Some  of  these 
cases  probably  differ  etiologic- 
ally  from  those  just  mentioned. 
They  are  believed  to  be  due  to 
traction  of  an  adherent  amnion 
on  the  skull. 

In  encephalocele  the  sac  contains 
membranes  and  brain-substance, 
but  no  fluid.  Sometimes  an  oc- 
cipital encephalocele  contains 
the  greater  part  of  the  brain  and 
forms  a  relatively  enormous 
pouch  hanging  down  the  back 
(notencephaly). 

Analogous  to  the  malforma- 
tions of  the  brain  just  referred 
to,  we  have  a  corresponding 
series  confined  to  the  cord  and 
spinal  column.  These  have 
already  been  discussed  (vol.  i, 
p.  246.) 

Allied  to  the  conditions  we 
have  just  described  is  dilatation 
of  the  central  neural  canal.  The 
ventricles  of  the  brain  may  be 
dilated  and  filled  with  fluid 

(hydrocephalus  internus  congenitus),  or  there  may  be  a  diffuse  dilatation 
of  the  central  canal  of  the  cord  (hydro mye loce le ;  syringomyelocele ; 
myelocystocele ;  hydrorrhachis  interna). 

In  congenital  internal  hydrocephalus,  there  is  either  a  uniform  or  a 
cystic  dilatation  of  the  ventricles.  The  brain  is  distended,  and  its  sub- 
stance may  be  reduced  to  a  thin  sheet.  The  lining  epithelium  of  the 
neural  canal  can  usually  be  recognized  here  and  there  on  the  inner  wall 
of  the  sac.  The  bones  of  the  cranium  are  often  separated  one  from  the 
other,  so  that  the  head  is  enlarged,  and  defects  of  ossification  may  also 
be  found.  In  rare  cases,  rupture  of  the  sac  takes  place  at  birth  or  dur- 
ing intra-uterine  life,  thus  producing  a  secondary  cranioschisis.  Virchow1 
has  described  local  dilatations  of  the  ventricles,  analogous  to  the  cir- 
cumscribed dilatation  of  the  cord  known  as  myelocystocele.  Of  these 
may  be  mentioned,  hydrops  of  the  fourth  and  fifth  ventricles,  and  hydrops 
cysticus  cornu  posterioris. 


Iniencephaly.     (McGill  Medical  Museum.) 


Die  Krankhaften  Geschwiilste,  1 :  1863,  Berlin;  also  Virch,  Archiv,  27 : 1863 : 575, 


526  THE  BRAIN 

The  local  dilatations  of  the  cord — myelocystocele — are  associated  with 
defects  in  the  fusion  of  the  arches  or  bodies  of  the  vertebrae,  and  are  due 
to  accumulation  of  fluid  within  the  central  canal  at  such  points.  The 
wall  of  the  sac  is  composed  of  spinal  substance  and  the  meninges.  The 
cavity  is  lined  with  epithelium  continuous  with  that  of  the  central  canal. 

Anencephaly. — Complete  absence  of  the  brain,  anencephaly,  is  usually 
associated  with  acrania,  and  occasionally  with  absence  of  the  spinal  cord 
(amyelia).  At  times  there  is  an  abortive  attempt  at  the  formation  of  a 
pons,  medulla,  and  cord. 

Cyclencephaly. — One  of  the  most  curious  forms  of  agenesy  is  cyclen- 
cephaly,  of  which  various  degrees  exist.  In  this,  the  anterior  portion  of 
the  prosencephalon  is  the  part  involved.  The  normal  division  into 
two  hemispheres  does  not  take  place  and  the  cerebrum  appears  as  a 
single  cyst-wall  enclosing  a  more  or  less  enlarged  ventricle.  In  some 
cases  the  eyes  are  fused  into  a  single  organ,  situated  in  the  middle  of  the 
forehead  (cyclopia)  and  provided  with  a  single  optic  nerve.  The  nose 
may  be  rudimentary  or  absent.  The  lower  jaw  and  the  bones  of  the  face 
are  absent,  and  the  ears  may  be  situated  lower  down  than  normal.  In 
the  less  extensive  deformity,  the  two  eyes  may  be  separate,  although 
closely  approximated  and  lying  in  a  single  orbit,  or  partially  fused 
(synophthalmia).  (Vide  vol.  i,  p.  241.) 

Arhinencephaly. — Defect  of  the  olfactory  bulbs — arhinencephaly— 
has  been  described.  A  great  variety  of  malformations  are  frequently 
associated  with  this  condition,  such  as  rudimentary  formation  of  the 
nose,  harelip,  cleft  palate,  and  absence  of  the  olfactory  nerves,  synotia, 
accessory  auricles,  anomalies  of  the  heart  and  great  vessels,  umbilical 
hernia,  defects  of  the  diaphragm,  and  supernumerary  digits. 

Agyria. — Not  uncommonly,  defective  development  of  the  prosenceph- 
alon manifests  itself  in  partial  or  complete  absence  of  the  convolutions 
(agyria),  or  of  larger  portions  of  the  brain  substance,  or,  again,  of  the 
commissures. 

Most  frequently  the  corpus  callosum,  fornix,  the  soft  commissure  of 
the  third  ventricle,  and  the  corpora  candicatia  are  lacking.  In  absence 
of  the  corpus  callosum,  there  is  usually  defect  of  the  gyms  fornicatus 
and  gyrus  hippocampi,  as  well  as  other  anomalies  in  the  convolutions. 
Where  considerable  amounts  of  the  external  portion  of  the  cerebrum 
fail  to  develop,  we  get  fissures  or  deep  excavations,  usually  in  the  central 
or  lateral  aspects  of  the  brain,  which  are  bridged  over  by  the  arachnoid, 
while  the  pia  dips  down  and  covers  over  the  base.  In  such  cases  fluid 
accumulates  in  the  cavity  in  the  subarachnoid  space,  or  sometimes  in 
the  meshes  of  the  pia  and  in  the  subdural  space.  Defects  of  this  kind, 
due  to  primary  errors  of  development,  constitute  one  form  of  what  is 
known  as  porencephaly.  Lesions  not  unlike  them,  however,  are  occa- 
sionally produced  by  trauma,  vascular  disturbances,  or  inflammation 
(secondary  porencephaly). 

Extensive  defects  of  the  brain,  such  as  acrania  and  anencephaly, 
lead  to  imperfect  development  of  the  cord  (atelomyelia).  In  such  cases 
it  may  be  abnormally  short.  Again,  when  portions  of  the  brain  are  lack- 


CONGENITAL  ANOMALIES  527 

ing,  the  neurones  of  which  are  ordinarily  continued  into  the  cord,  we 
get  symmetrical  or  asymmetrical  aplasia  of  the  corresponding  spinal 
tracts.  The  spinal  cord  may  be  entirely  absent  (amyelia).  In  this  case 
it  is  said  that  the  posterior  ganglia  and  the  sensory  nerves  may  be  per- 
fectly developed  (Lionowa). 

Hypoplasia. — The  weight  of  the  adult  human  brain  varies  within 
wide  limits.  The  average  may  be  struck  at  1400  grams  in  the  male, 
and  about  1300  in  the  female.  As  examples  of  extreme  limits  might 
be  mentioned  the  brain  of  a  Bushwoman  (871  grams)  and  that  of 
v.  Turgenieff  (2012  grams).  Under  pathological  conditions,  however, 
these  figures  may  be  widely  exceeded.  Thus,  Ziegler1  figures  the  brain 
of  a  microcephalic  idiot,  Helene  Becker,  which  weighed  only  219  grams, 
and  Van  Walsem2  describes  that  of  an  epileptic  idiot  which  reached  the 
almost  incredible  weight  of  2850  grams.  In  determining  the  proper 
weight  of  the  brain  in  any  given  case,  age,  sex,  race,  and  body  weight 
should  be  considered.  The  brain  takes  on  its  most  rapid  growth  during 
the  first  year  of  life,  and  reaches,  practically,  its  maximum  at  the  age  of 
seven  or  eight.  After  about  fifty  it  begins  to  decrease.  The  white 
races  have  larger  brains  than  the  black;  the  male  than  the  female.  In 
healthy  human  beings,  according  to  Quain,  the  proportionate  weight 
of  the  brain  as  compared  with  the  body  as  a  whole  is  1  to  45. 

Hypoplasia  might  be  defined  as  a  condition  of  the  brain  in  which, 
while  the  parts  present  the  normal  configuration,  the  size  and  weight  of 
the  brain  fall  notably  short  of  the  normal  average  for  the  particular 
individual  concerned,  laying,  of  course,  due  stress  upon  the  consider- 
ations mentioned  above.  As  a  matter  of  fact,  however,  hypoplasia  is 
usually  associated  with  other  peculiarities  of  development,  such  as 
local  agenesy,  imperfect  or  irregular  formation  of  the  convolutions,  and 
structural  changes  in  the  nervous  substance. 

Microcephaly. — Hypoplasia  of  the  brain,  as  a  whole,  occurs,  and  may 
be  associated  with  a  corresponding  smallness  of  the  cranium  (micro- 
cephaly).  The  condition  is  often  apparent  at  birth,  but  becomes  more 
obtrusive  as  the  general  somatic  development  goes  on.  In  some  cases, 
the  adult  brain  does  not  reach  the  size  of  that  of  the  newborn  child. 
As  a  rule,  the  skull  presents  premature  synostosis  of  certain  of  the  sutures 
with  corresponding  asymmetry,  and  other  peculiarities  of  ossification, 
such  as  Wormian  bones.  The  brain  itself  is  not  only  small,  but  the  scheme 
of  the  convolutions  is  much  less  complex  than  in  the  case  of  normal 
brains. 

Micrencephaly. — In  another  class  of  cases  the  brain  is  dispropor- 
tionately small,  as  compared  with  the  size  of  the  cranium  (micrencephaly). 
This  is  generally  associated  with  an  abnormal  accumulation  of  fluid  in 
the  subarachnoid  space  (hydrocephalus  meningeus  sive  externus;  menin- 
geal  hy  drops),  in  the  ventricles  (hy  drops  ventricularis  sive  internus),  or 
both.  Some  authorities  have  attributed  the  hypoplasia  of  the  brain  to 

1  Lehrbuch  der  speciellen  pathologischen  Anatomie,  Jena,  1895:321, 

2  Neurol.  CentralbL,  13  :  1899  :  578, 


528  THE  BRAIN 

the  presence  of  this  fluid,  but  while  this  may  be  true  in  some  cases,  it  is 
more  likely  that  the  hydrops  is  secondary  (hydrops  ex  vacuo)  in  most 
instances.  Undoubtedly,  hydrops  is  frequently  associated  with  anomalies 
of  development  both  of  the  brain  and  skull,  such  as  arhinencephaly  and 
cyclopia.  When  the  brain  is  small  we  can  speak  of  hydrocephalic  micren- 
cephaly.  All  the  ventricles  may  be  dilated  or  only  certain  portions  of 
them. 

Partial  Hypoplasia. — Partial  hypoplasia  involves  usually  the  hemi- 
spheres of  the  cerebrum  and  cerebellum,  less  commonly  the  corpus 
callosum  and  the  structures  at  the  base.  In  the  case  of  the  cerebrum, 
it  leads  to  asymmetry  and  is  generally  associated  with  microgyria  or 
even  agenesy  of  certain  convolutions. 

Hypoplasia  of  the  brain  is  most  probably  due  to  a  primary  vitium  of 
development,  but  in  some  cases  may  be  the  result  of  pathological  processes 
acting  during  intra-uterine  life.  Premature  synostosis  of  the  cranial 
sutures  is  at  work  in  some  instances. 

Hypoplasia  of  the  cerebellum  may  affect  the  organ  as  a  whole,  but 
is  generally  unilateral.  The  condition  is  associated  with  hypoplasia 
of  the  olivary  bodies,  pons,  and  medulla.  The  transverse  fibers  in  the 
pyramidal  tracts  of  the  pons  are  absent. 

As  might  be  expected,  microscopic  study  of  the  tissues  involved  in 
hypoplasia  shows  marked  deviations  from  the  normal.  Certain  of  the 
ganglion  cells  of  the  cortex  are  lacking,  and  corresponding  with  this  is 
an  absence  of  neuraxones  belonging  to  them.  There  may  be  a  relative 
or  compensatory  increase  (macrencephaly)  in  other  parts. 
,  Microgyria. — Occasionally,  however,  the  convolutions  are  particularly 
numerous  but  diminutive  (microgyria).  Or,  again,  owing  to  defect  of 
the  nervous  substance,  the  convolutions  are  represented  only  by  mem- 
brane. While  the  hypoplasia  is  most  marked  in  the  cerebrum,  the  cere- 
bellum and  basal  structures  are  to  some  extent  involved.  As  a  conse- 
quence we  find  lack  of  formation  or  defect  in  the  medullation  of  certain 
fibers  in  the  pons,  medulla,  and  cord,  particularly  those  that  become 
medullated  somewhat  late,  such  as  the  pyramidal  tracts  and  columns  of 
Goll,  less  often  of  the  anterior  columns  and  cerebellar  tracts  (micro- 
myelia;  atelomyelia). 

Hydrocephahis. — In  not  a  few  cases  the  accumulation  of  the  cerebro- 
spinal  fluid,  with  dilatation  of  the  ventricles,  comes  on  after  the  brain  is 
fairly  well  formed.  The  condition  may  arise  during  intra-uterine  exist- 
ence and  the  hydrocephalic  head  prove  an  obstruction  during  parturition. 
The  enlargement  of  the  head  is  not  always  marked  at  the  first,  but 
gradually  increases  and  may  become  extreme.  The  cranium  enlarges, 
the  skin  is  stretched,  the  subcutaneous  veins  are  prominent,  and  the 
fontanelles  are  enlarged.  Finally,  the  sutures  give  way,  and  the  cranial 
bones  become  separated.  The  meninges  are  distended  and  tense.  The 
dilatation  of  the  ventricles  may  be  so  extreme  that  the  substance  of  the 
hemispheres  is  transformed  into  a  thin  sac,  little  being  left  but  the  pia- 
arachnoid.  The  sulci  are  obliterated  and  the  convolutions  disappear. 
The  basal  ganglia  are  flattened,  but  the  cerebellum  usually  is  unaffected. 


CONGENITAL  ANOMALIES  529 

The  fourth  ventricle  is  not,  as  a  rule,  dilated.  The  hydrops  may  be 
diffuse  and  symmetrical,  or  only  one  ventricle,  or  a  portion  of  one  ven- 
tricle may  be  involved.  Dilatation  of  the  fourth  ventricle  leads  to  pressure 
on  the  cerebellum,  pons,  and  cord.  The  fluid  in  the  ventricles  is  clear, 
colorless,  or  slightly  yellowish. 

The  causes  of  hydrocephalus  are  not  well  understood.  It  has  been 
attributed  to  inflammation  of  the  ependyma  and  to  interference  with  the 
return  venous  circulation,  but  the  evidences  of  this  are  hardly  convincing. 
The  pia  at  the  transverse  fissure  has  been  found  to  be  indurated,  and 
thus  might  conceivably  press  upon  the  veins  of  Galen.  The  normal 
channels  of  communication  which  exist  at  the  transverse  fissure  between 


FIG.  144 


Hydrocephalus;  child  aged  about  four  years.     Mental  condition  good.     Diplegia  with  talipes. 
Circumference  of  the  head  about  28  inches.     (Dr.  A.  E.  Vipond's  case.) 

the  ventricles  and  the  subarachnoid  space  are  occasionally  obliterated. 
In  unilateral  hydrocephalus  the  foramen  of  Munroe  may  be  found  to  be 
occluded.  Probably  obstruction  of  the  lymphatics  and  transudation  of 
lymph  has  a  good  deal  to  do  with  it. 

Corresponding  in  some  particulars  to  hydrops  of  the  brain  and  its 
membranes,  we  have  accumulations  of  fluid  in  and  about  the  cord. 
A  collection  of  fluid  within  the  spinal  meninges — hydrorrhachis  externa — 
sometimes  leads  to  atrophy  or  hypoplasia  of  the  cord. 

Hydro myelia. — Fluid  within  the  central  canal  is  called  hydrorrhachis 
interna  or  hydromyelia.  It  leads  to  a  diffuse  or  saccular  dilatation  of 
the  central  canal  with  more  or  less  encroachment  upon  the  substance  of 
34 


530  THE  BRAIN 

the  cord.  The  condition  may  be  merely  microscopic  or  may  involve 
the  greater  part  of  the  thickness  of  the  cord.  The  cavity  is  circular 
or  irregular  and  situated  in  the  centre  of  the  cord,  or  it  may  extend 
into  the  posterior  horns  and  columns. 

The  changes  in  the  cord  are  referable  to  the  effects  of  pressure,  being 
chiefly  atrophy,  thickening  of  the  vessels,  and  slight  peri-ependymal 
gliosis.  The  cause  is  unknown.  Some  have  suggested  a  secretory 
function  on  the  part  of  the  ependymal  cells.  Others  think  it  due  to 
vascular  disturbances. 

Hyperplasia. — Hyperplasia  of  the  brain  is  usually  associated  with 
a  corresponding  developmental  enlargement  of  the  cranium  (macro- 
cephaly).  Two  forms  may  be  differentiated. 

In  the  first,  there  is  a  true  hyperplasia  of  all  the  elements  composing 
the  brain,  which  differs,  therefore,  in  no  respect  save  that  of  size  from 
the  normal  brain.  The  enlargement  of  the  brain  due  to  hydrocephalus 
should  not  be  confounded  with  true  macrencephaly.  The  weight  of  the 
brain  in  this  condition  may  vary  from  1500  to  2200  grams.  Individuals 
possessing  abnormally  large  brains  have  occasionally  been  noted  for 
intellectual  vigor,  but  this  is  by  no  means  an  invariable  rule.  Idiots  and 
epileptics,  on  the  other  hand,  occasionally  have  large  brains.  In  moder- 
ate grades  the  membranes  are  put  on  the  stretch,  and  the  epidural  space 
and  the  cavity  of  the  ventricles  are  encroached  upon. 

In  the  second  class,  the  enlargement  is  due  to  a  relative  increase  in 
the  amount  of  glia  (gliosis).  This  may  be  generalized  or  confined  to 
certain  districts,  convolutions,  or  parts  of  convolutions.  The  condition 
is  referable  to  a  primary  peculiarity  of  development. 

An  interesting  and  somewhat  obscure  affection  of  the  spinal  cord 
associated  with  hyperplasia  of  the  glia  tissue  is  known  as  syringomyelia. 
This  term  simply  means  cavity  formation  in  the  cord  (ffuptfz,  a  flute), 
and  therefore  might  be  taken  to  include  such  conditions  as  hydromyelia, 
hematomyelia,  pyomyelia,  and  hemorrhagic,  degenerative,  and  inflam- 
matory softening.  It  is  generally  taken  to  mean,  however,  a  condition, 
distinct  from  all  these,  which  is  attended  by  a  somewhat  variable,  but 
still  characteristic,  train  of  symptoms.  These  are,  in  a  typical  case, 
muscular  wasting  of  the  Aran-Duchenne  type,  loss  of  thermic  and  painful 
sensations,  with  preservation  of  tactile  sensibility.  Irregular  forms  are 
also  met  with  occasionally  which  recall  amyotrophic  lateral  sclerosis, 
tabes  dorsalis,  and  Friedreich's  ataxia.  The  lesions  are  generally 
found  in  the  cervical  and  upper  dorsal  portions  of  the  cord,  but  may 
involve  its  whole  length,  and  even  extend  into  the  medulla,  pons,  and 
internal  capsule.1 

To  gross  appearance  the  cord  may  present  little  change,  although  this 
is  unusual.  The  dura  is  normal,  the  pia-arachnoid  normal  or  but  slightly 
thickened.  The  cord  itself  may  present  a  natural  configuration  or  may 
collapse  on  removal  into  a  ribbon-like  band,  according  to  the  extent  of 
the  pathological  change.  Sometimes  there  is  slight  enlargement,  and 

1  Spiller,  Brit.  Med.  Jour.,  2:  1906:  1017. 


HYPERPLASIA  531 

there  may  be  fluctuation  on  palpation.  The  cord  not  infrequently  shows 
atrophy  in  certain  tracts.  On  section,  it  is  found  to  contain  one  or 
or  more  cavities,  usually  one,  situated  somewhat  posteriorly  to  the  central 
canal.  The  cavity  has  a  predilection  for  the  gray  matter,  usually  of 
the  posterior  part  and  the  dorsal  horns,  but  not  infrequently  extends 
more  or  less  widely  into  the  white  matter.  It  is  round,  oval,  triangular, 
slit-like,  or  irregular  in  shape,  and  may  be  confined  to  one  side  of  the 
cord.  Its  distinguishing  features  are  asymmetry  and  irregularity. 
Occasionally,  more  than  one  cavity  may  be  found  occupying  different 
sections  of  the  cord  without  any  attempt  at  communication.  In  a  typical 
case,  the  cavity  is  distinct  from  the  central  canal,  but  in  some  instances 
is  found  to  communicate  with  it  at  several  points.  The  space  is  usually 
filled  with  the  detritus  from  colliquative  necrosis,  cerebrospinal  fluid, 
or,  rarely,  blood. 

FIG.  145 


Syringomyelia.  Dorsal  cord,  showing  central  cavitation.     (From  collection  of  Dr.  Colin  K.  Russel.) 

Microscopically,  the  space  is  bounded  by  a  zone  of  delicate  neuroglia. 
Rarely,  it  has  been  lined  by  what  appeared  to  be  connective  tissue,  sup- 
posed to  be  derived  from  inclusion  of  the  pia.  Occasionally,  it  may  be 
lined  here  and  there  with  ependymal  epithelium.  The  extent  of  the 
glial  hyperplasia  varies  within  wide  limits.  The  central  canal  may  be 
normal  in  size  and  appearance,  or  may  be  dilated  (hydromyelia).  The 
rest  of  the  cord  may  show  little  or  no  change,  but,  as  a  rule,  there  is  con- 
siderable ascending  and  descending  degeneration.  The  nerve  roots  are 
often  involved,  and  there  is  a  descending  degeneration  of  the  peripheral 
part  of  the  anterior  motor  neurones.  The  vessels  commonly  show 
hyaline  degeneration  and  thickening  of  the  intima.  Diapedesis  of  cells, 
thrombosis,  and  rupture  of  the  capillaries  may  take  place. 

These  appearances  have  been  variously  interrupted.  The  generally 
accepted  view  at  the  present  time  is  that  syringomyelia  is  due  to  a  primary 
hyperplasia  of  the  neuroglia,  followed  by  degenerative  softening  and 
cavity  formation.  The  supporting  structure  of  the  central  nervous 
system,  or  glia,  is  of  ectodermal  origin  and  is  produced  by  the  pro- 


532 


THE  CORD 

FIG.  146 


Syringomyelia,  with  extensive  cavitation  of  the  posterior  horn.     (From  collection 
of  Dr.  Colin  K.  Russel.) 


FIG.  147 


Syringomyelia.     Lumbar  cord,  showing  cavitation  of  both  posterior  cornua  and  descending 
degeneration  in  the  pyramidal  tracts.     (From  collection  of  Dr.  Colin  K.  Russel.) 


HETEROTOPIA  533 

liferation  of  the  ependymal  cells  of  the  central  neural  canal.  The 
original  ependyma  is  represented  in  the  human  cord  by  the  cuboidal 
cells  forming  the  lining  epithelium  of  the  central  canal,  and  small  groups 
of  cells  in  its  immediate  neighborhood.  Syringomyelia  is  thus  to  be 
regarded  as  a  central  gliosis  caused  by  the  proliferation  of  the  ependymal 
cells,  the  vascular  changes  which  accompany  it,  such  as  thickening  of 
the  vessels,  thrombosis,  embolism,  and  hemorrhage,  being  responsible 
for  the  softening,  which  is  largely  of  the  nature  of  a  necrosis  from  lack 
of  nutrition.  Virchow  believed  that  syringomyelia  was  consecutive  to 
congenital  hydromyelia,  and  thought  that  the  gliosis  was  due  to  the 
proliferation  of  inclusions  of  embryonic  glia  cells  about  the  central 
canal.  Such  embryonic  cells  may  take  the  form  of  diverticula  from 
the  neural  canal,  solid  masses,  or  isolated  cavities.  The  pressure  of  the 
fluid  within  the  central  canal  is  here  supposed  to  play  an  important  part 
in  the  production  of  the  softening. 

Another  theory,  held  by  certain  French  observers  (Hallopeau;  Joffroy), 
is  that  the  process  is  essentially  an  inflammatory  one,  which  directly 
and  indirectly,  owing  to  vascular  changes,  leads  to  softening  and  cavity 
formation  (my elite  cavitaire). 

Another  view  is  that  obstruction  to  the  blood  or  lymph-circulation, 
due,  for  example,  to  the  pressure  of  tumors  in  the  posterior  fossa  of  the 
skull,  or  of  the  cord  itself,  and  meningitis,  lead  to  hydrops  of  the  canal 
and  secondary  dilatation. 

None  of  the  theories  propounded  is  entirely  satisfactory,  for  cases 
have  occasionally  been  met  with  which  cannot  be  explained  on  any  of 
the  hypotheses.  On  the  whole,  the  preponderance  of  evidence  goes  to 
show  that  errors  of  development  are  the  most  important  factor.1 

Diastematomyelia. — What  might  be  called  a  numerical  hyperplasia  is 
seen  in  the  cord,  in  the  condition  known  as  diastematomyelia,  or  redu- 
plication of  the  cord.  The  reduplication  occurs  in  the  lumbar  region. 
The  two  divisions  are  contained  within  the  one  vertebral  canal,  or  are 
separated  by  a  bony  septum. 

Diplomyelia. — Diplomyelia,  or  the  formation  of  two  cords,  is  met  with 
in  certain  double  monsters. 

The  cord  may  also  be  abnormally  long.  The  spinal  roots  may  be 
abnormally  numerous,  or,  again,  defective. 

Heterotopia. — Heterotopia  may  be  defined  as  the  presence  of  normal 
constituents  of  the  cord  in  abnormal  situations.  Thus,  portions  of  the 
gray  matter  may  be  found  in  the  white  substance.  Many  of  these  ap- 
pearances are  due  to  artefacts  produced  in  sectioning  the  cord,  but  there 
are  undoubtedly  cases  in  which  the  condition  is  due  to  an  error  of  develop- 
ment. Of  this  nature  are  some  forms  of  asymmetry  of  the  cord.  One 
pyramidal  tract,  for  instance,  may  be  small  or  even  absent,  while  the 
other  is  correspondingly  large.  This  is  due  to  the  failure  of  certain  of 
the  motor  fibers  to  decussate. 

1  For  one  of  the  best  monographs  on  Syringomyelia,  with  full  bibliography,  see 
Schlesinger,  Die  Syringomyelie,  \Vien,  1895. 


534  THE  CEREBRAL  MENINGES 


THE  CEREBRAL  MENINGES. 

The  covering  membranes  of  the  brain  are  three  in  number:  an  outer, 
the  dura;  a  middle,  the  arachnoid;  and  an  inner,  the  pia.  The  last 
two  are  so  intimately  associated,  anatomically  and  pathologically,  that 
they  practically  form  one  structure.  The  membranes,  owing  to  their 
relations,  are  liable  to  be  involved  in  pathological  processes  originating 
both  in  the  brain  and  in  the  cranial  bones;  and,  further,  bring  highly 
vascular  and  bounding  spaces  which  are  practically  large  lymph-channels, 
infective  agents  can  readily  reach  them  from  distant  or  adjacent  parts, 
and  may  set  up  rapidly  extending  inflammation.  It  should  be  remarked 
that,  while  it  is  convenient  for  descriptive  purposes  to  adopt  the  regional 
method  of  classification,  it  is  almost  impossible  for  one  membrane  to  be 
diseased  without  to  some  extent  involving  the  others  and  even  the  brain 
itself. 

The  Cerebral  Dura  Mater. 

The  dura  mater  is  a  tough,  inelastic,  connective-tissue  membrane, 
and  is  lined  on  its  inner  surface  by  a  layer  of  endothelium  of  a  grayish- 
white,  glistening  appearance,  which  serves  the  double  purpose  of  a  peri- 
osteum for  the  cranial  bones  and  a  protective  covering  for  the  brain.  In 
adults  it  is  only  loosely  adherent  over  the  vault,  while  at  the  base  of  the 
skull  it  is  much  more  closely  attached.  It  sends  three  processes  into 
the  intracranial  cavity  for  support  and  pro  tection  of  the  brain:  first 
the  falx  cerebri,  running  longitudinally  between  the  two  cerebral  hemi- 
spheres; second,  the  tentorium  cerebelli,  on  which  rest  the  occipital  lobes 
separating  them  from  the  hemispheres  o.f  the  cerebellum;  and  third, 
the  falx  cerebelli,  running  vertically  between  the  two  lobes  of  the  cere- 
bellum. It  also  sends  prolongations  enclosing  the  various  cranial  nerves 
and  vessels  as  they  make  their  exit  from  the  skull.  Around  the  margin 
of  the  foramen  magnum  it  is  closely  adherent  to  the  bone  and  is  contin- 
uous with  the  spinal  dura  mater.  In  certain  situations  the  dura  splits  up 
into  two  layers  to  contain  the  various  venous  sinuses,  which  play  an 
important  part  in  connection  with  certain  infective  processes.  Thus, 
in  the  superior  border  of  the  falx  cerebri  lies  the  superior  longitudinal 
sinus,  while  the  inferior  longitudinal  sinus  is  enclosed  in  its  inferior 
border.  The  tentorium  cerebelli  encloses  the  two  lateral  sinuses  in  its 
posterior  convex  borders.  In  its  anterior  borders  it  encloses  the  superior 
petrosal  sinus,  and  along  the  middle  line  of  its  upper  surface  runs  the 
Mraight  sinus.  The  occipital  sinus  runs  in  the  attached  margin  of  the 
falx  cerebelli. 

On  the  outer  surface  of  the  dura  run  the  branches  of  the  middle 
meningeal  artery.  The  veins  of  the  dura  are  connected  with  those  of 
the  scalp  by  numerous  branches. 


CIRCULATORY  DISTURBANCES. 

Congestion.  ('on^onon  of  the  dura  is  commonly  met  \\iih  in 
association  \\  iih  inflammation  of  the  dura  or  other  membranes,  or  \\  here 
there  is  an  increase  in  intraeranial  pressure  from  am  cause,  provided, 
of  course,  that  that  pressure  l»e  not  excessive.  In  such  cases,  on  removal 
of  the  eal\  ariuin.  tlie  dtira  \veeps  blood  rather  freelv. 

Anemia.  Anemia  is  met  \\ith  in  death  by  bleeding  and  in  all  forms 
of  s\  stemic  anemia. 

Hemorrhage.  Hemorrhage  ma\  take  place  upon  the  Mirfaee  of 
the  dura  (epidural\  into  its  substance  (intradural),  or  beneath  it  (sub- 

durah.  The  commonest  cause  is  tranmatisin.  such  as  concussion  and 
fracture,  or.  au;ain.  disease  of  the  adjacent  bom  structures.  Alter  falls, 
the  bleeding  is  often  due  to  rupture  of  the  middle  mcnin^cal  arten  or 
some  of  its  branches.  The  hemorrhage  does  not  necessarily  occur 
at  the  site  of  the  Mo\\.  luit  ma\  take  place  on  the  opposite  side  .  hv 
OOnfrtfCOt'i))  or  at  some  other  part.  \\hen  the  m|iirv  is  severe,  fatal 
consequences  often  follo\\  .  o\\  IIIL;'  to  compression  of  the  brain.  It  is 
to  be  remembered  that  serious  s\  inptoms  do  not  al\\  a\  s  come  on  iminedi- 
atelv  on  receipt  of  the  in  jnr\  .  but  the  patient  ma\  \\  alk  a\\  a\  after  what  is 
regarded  as  a  truial  accident,  and  mas  die  a  te\\  hours  later,  unless 

carefully  watched  and  treated.    The  effused  blood  tends  to  gravitate 

to  the  tloor  of  the  cranial  ea\  it\  .  and  max   interfere  \\  ith  the  \  Hal  ceiiti-cs 

there,  although  sonu%times  n  remains  curiously  localized  to  the  region 

of  origin.      \\  here  \\\c  hemorrhage  has  been  sl,>\\  .  or  of  the  nature  of  an 
oo/iui;.  the  blood  is  found  clotted  or  even  disintegrated.      In  such  ca 
especialb    if    infection    has    taken    place,  a    not    uncommon    e\ent    in    the 
ease  of  fractures,  there  max    be  more  or  less   inflammation  ol    the  brain 
and  melinites,  and  c\cn  abscess-formation.      Kxtradnral  htMiiorrha; 
oecasjonalb    met    \\ith    in   the  ne\\  born   child    as  the  result   of  a   difficult 

or  instrumental  deli\er\.    This  is  due  to  laceration  of  the  vessels  from 

the  excessive  over  riding  of   tin'   cranial   hones.      If   not    immediately  or 
(|iiickl\    fatal    it    ma\    ^i\e    rise    to  a    peculiar    form    of   spastic    parab 
often  associated  \\ith  idiocx  .  knoun  as  infantile  ft  ri  l>nil  fxil.^u  or  ft  nhrnl 
iH^ln.  The     same     conditinn     m -cumui;-     i luring-     infra   uterine     life 

ma\  cause  ///  rn  (Ji/r  n  '  dlu.      1  lenu  >rrha;;c  into  the  substance 

of  the  dura  is  rare  and  iisualK   of  slight  extent.       It  is  sometimes  a    ;oci 
ated  \\ith  cxtradural  hemorrhage  m  cases  of  jraiimat  ism. 

ThrOlllhosis.  Thrombosis  of  the  venous  sinuses  of  the  dura  is  of 
not  infrequent  occurrence  and  is  ab\a\s  of  :;ra\c  import.  In  one  class 

of  cases,  the  condition  appears  to  depend  1 1 pon  general svstemic  weakness, 

\\here  particularb  tlu-re  is  deterioration  in  the  (|ualit\  of  the  bloc.!. 
This  leads  to  degeneration  of  the  \essel  \\alK  a  condition  \\hich.  \\ith 
impaired  \  i^or  of  the  circulation,  favors  clotting  \nnininfn'  ///ro/M/>av/.v). 
Anatomical  peculiariti(\s  are  of  some  importance  here  also,  particnlarls 
sudden  enlargement  of  the  vessels,  which  lead-  |,.  ;lo\\  in^  of  the  blood 
am, ami  the  une\ennessof  their  walls  due  tc.  the  ra«-chi«»nian  bodies. 


536  THE  DURA 

The  condition  is  met  with  in  marasmus,  profound  anemias,  summer 
diarrhoea  of  children,  and  in  the  aged.  The  longitudinal  sinus  is  the 
site  of  election  for  the  process,  but  the  transverse  may  be  involved. 
(Edema  and  rupture  of  the  meningeal  or  cerebral  vessels  are  sometimes 
found  as  a  result.  In  another  class  of  cases,  the  condition  has  an 
inflammatory  basis  (thrombosinusitis)  and  is  analogous  to  thrombophle- 
bitis. This  is  seen  in  meningitis  and  certain  injuries  to  the  cranial 
bones.  Perhaps  the  most  common  cause  is  suppurative  otitis  media, 
complicated  with  necrosis  of  the  petrous  bone.  Here  the  lateral  sinus 
is  apt  to  be  involved.  We  have  met  recently  with  a  case  of  thrombosis 
of  both  choroidal  sinuses  in  puerperal  septicemia. 

There  is  at  first  inflammation  of  the  wall  of  the  sinus,  followed  by 
clotting  of  the  blood.  The  vessel  is  filled  with  a  dirty,  necrotic-looking 
substance,  of  a  grayish-red  appearance,  and  its  wall  is  often  of  a  yellow- 
ish-green color.  The  process  may  extend  into  the  jugular  vein.  The 
dura  is  apt  to  be  involved  in  the  vicinity  and  there  may  be  foci  of  suppu- 
ration between  its  layers.  Supervening  upon  the  affection  we  may  get 
meningitis  and  abscess  of  the  brain.  As  the  condition  is  infective, 
metastatic  abscesses  may  be  formed  in  various  parts  and  a  general 
septicemia  induced.  When  middle  ear  disease  is  the  primary  cause,  the 
abscess  is  most  likely  to  be  found  in  the  temporosphenoidal  lobe  or  in  the 
cerebellum.  In  marantic  cases,  or  where  the  infection  is  mild,  the  throm- 
bus may  be  absorbed  or  become  organized,  provided  that  the  patient 
live  long  enough. 

INFLAMMATIONS. 

Pachymeningitis. — Inflammation  of  the  dura  is  called  pachymenin- 
gitis.  It  is  divided  into  external  and  internal  forms,  according  to  the 
surface  of  the  membrane  chiefly  involved. 

Pachymeningitis  Externa  Acuta. — Pachymeningitis  externa  acuta  is 
usually  due  to  traumatism,  such  as  fractures,  gunshot  injuries,  and 
cutting  wounds  of  the  skull,  or  to  the  extension  of  disease  from  the 
neighboring  bone,  as  in  caries  of  the  petrous  bone  and  osteomyelitis. 
Occasionally,  it  is  secondary  to  erysipelas  of  the  scalp,  owing  to  infection 
extending  through  the  veins  of  the  diploe.  The  dura  is  congested, 
swollen,  and  softened,  and  there  is  an  exudate  which  may  be  serous, 
seropurulent,  or  purulent.  The  surface  of  the  membrane  is  grayish- 
white  or  grayish-yellow,  and  in  some  cases  covered  with  extravasated 
blood.  The  process  may  spread  to  the  pia-arachnoid  or  to  the  brain 
itself.  When  not  fatal,  the  exudate  may  become  absorbed  or  encysted. 
Not  infrequently  the  membrane  remains  thickened  and  is  adherent  to 
the  skull. 

Pachymeningitis  Externa  Chronica. — Pachymeningitis  externa  chronica 
arises  from  trauma,  local  bone  disease,  and  sunstroke,  in  cases  where 
infection  does  not  take  place.  The  dura  is  thickened,  owing  to  the  pro- 
liferation of  the  connective  tissue,  and  in  some  cases  there  is  a  formation 
of  bone. 


PACHYMENINGITIS  INTERN  A  HEMORRHAGICA  537 

Pachymeningitis  Interna  Acuta. — Pachymeningitis  interna  acuta  is 
usually  dependent  on  external  pachymeningitis.  Certain  affections  of 
the  brain,  dural  sinuses,  and  pia-arachnoid,  also  lead  to  it.  The  exudate 
may  be  simple  or  purulent. 

Pachymeningitis  Interna  Hemorrhagica. — The  most  important  form,  how- 
ever, is  pachymeningitis  interna  hemorrhagica,  which  is  not  uncommonly 
met  with.  In  the  early  stages,  the  disease  is  characterized  by  the  forma- 
tion on  the  inner  surface  of  the  dura  of  a  delicate,  homogeneous,  granular 
or  fibrillar  deposit  of  fibrin,  containing  relatively  few  leukocytes.  This, 
in  time,  becomes  organized  through  the  ingrowth  of  delicate  capillaries, 
derived  from  the  vessels  of  the  dura,  together  with  young  fibroblasts. 
Thus,  a  delicate  connective-tissue  membrane  is  formed  lining  the  dura, 
containing  an  abundance  of  wide,  thin-walled  capillaries.  Owing  to 
the  tenuity  of  the  vessels,  and  probably  also  to  the  degeneration  which 
their  walls  undergo,  hemorrhage  readily  takes  place,  both  by  rhexis 
and  diapedesis,  into  the  substance  of  the  newly-formed  membrane 
and  upon  its  surface.  The  extravasation  is,  in  many  instances,  of  the 
nature  of  a  simple  oozing,  but  not  infrequently  large  effusions  take  place, 
which  dissect  the  membrane  away  from  the  dura  and  collect  in  the  circum- 
scribed cavities  thus  formed  (hematoma  duras  matris).  As  the  hemorrhages 
are  apt  to  be  repeated,  we  get  an  alternation  of  old  clots,  more  or  less 
disorganized,  containing  blood  pigment,  with  a  fresh  deposit  of  fibrin, 
giving  the  whole  a  laminated  appearance.  The  process  is  essentially 
sluggish  and  prone  to  relapse.  The  smaller  extravasations  may  be 
absorbed,  but  the  larger  ones  are  only  imperfectly  removed,  if  at  all, 
while  from  time  to  time  a  fresh  exudation  and  another  formation  of 
membrane  takes  place.  As  the  affection  progresses,  the  new  membrane 
becomes  firmer  and  more  densely  organized  and  contains  the  remains 
of  blood,  blood  pigment,  fibrin,  and  even  deposits  of  lime  salts.  The 
presence  of  so  much  foreign  and  irritating  material  excites  inflammation, 
which  tends  to  reproduce  and  perpetuate  the  condition.  If  the  larger 
collections  of  blood  are  finally  absorbed,  we  occasionally  find  traces  of 
them  in  collections  of  fluid  between  the  dura  and  the  new  membrane 
(hygroma  durce  matris;  hydrocephalus  pachymeningiticus  partialis). 
In  the  older  and  denser  portions  of  the  organizing  membrane  the  vessels 
gradually  become  more  or  less  occluded,  but  as  new  ones  are  being  formed 
in  other  places  the  process  hardly  ever  comes  to  a  stop.  As  a  rule,  the 
affection  is  confined  to  the  dura,  but  in  some  cases  the  pia-arachnoid  is 
involved,  and  vascularized  adhesions  form  between  it  and  the  dura. 

Hemorrhagic  pachymeningitis  is  most  commonly  seen  in  drunkards 
and  the  insane.  It  has  been  observed  in  general  paralysis  of  the  insane, 
senile  dementia,  Huntingdon's  chorea,  and  scurvy.  It  may  be  due  to 
traumatism.  Occasionally,  it  has  been  found  associated  with  atrophy 
of  the  brain,  apparently  without  any  cause. 

The  etiology  is  not  entirely  clear.  The  majority  of  pathologists  agree 
with  Virchow  that  the  process  is  an  inflammatory  one.  It  is  possible 
that  in  some  cases,  owing  to  fatty  and  other  degenerative  changes  in 
the  walls  of  the  dural  capillaries,  these  give  way  and  we  get  hemorrhage 


538  THE  DURA 

upon  the  surface  of  the  dura  followed  by  inflammation,  exudation,  organ- 
ization, and  membrane  formation.  Another  view  is  that  spasm  and 
contraction  of  the  vessels  of  the  brain  lead  to  diminution  of  the  intracranial 
pressure  and  consequently  hemorrhage. 

Tuberculosis. — Tuberculosis  of  the  dura  is  rather  an  uncommon 
condition.  It  is  usually  found  in  association  with  miliary  tuberculosis 
of  the  pia,  but  occasionally  is  due  to  the  extension  of  bone  tuberculosis. 
It  takes  various  forms,  either  a  discrete  miliary  eruption  of  tubercles, 
a  membranous  deposit  containing  tubercles  on  the  inner  side  of  the 
dura,  or  large  caseous  masses  or  tuberculomas. 

Syphilis. — In  syphilis,  multiple,  small,  cellular  foci  or  granulomas, 
occasionally  coalescing,  may  be  formed,  in  some  cases  enclosing  necrotic 
or  gummy  material.  Adhesions  may  take  place  between  the  dura  and 
the  pia-arachnoid.  The  condition  is  most  apt  to  occur  at  the  base  of 
the  brain,  and  may  involve  the  cranial  nerves. 

Histologically,  the  lesions  consist  in  cellular  and  highly  vascular 
granulation  tissue  which  tends  to  go  on  to  necrosis.  The  process  usually 
begins  in  the  adventitia  of  the  vessels  or  in  the  epineurium  of  the  nerves. 
The  nerve-fibers  may  eventually  undergo  atrophy. 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — Of  the  benign  tumors,  the  most  important  are  the  fibroma, 
lipoma,  chondroma,  and  osteoma. 

Fibromas  are  rare.  They  are  found  on  any  part  of  the  dura  in  the 
form  of  hard,  nodular,  spherical  growths.  Lipomas  are  still  rarer. 
Small,  gelatinous  tumors,  ecchondromas,  are  found  occasionally  near  the 
clivus.  So-called  osteomas  are  met  with  in  the  tentorium  and  falx. 
They  are  more  properly  examples  of  metaplasia. 

The  primary  malignant  tumors  are  the  sarcoma  and  the  endothelioma. 

The  sarcomas  are  generally  spindle-celled,  more  rarely  round-celled, 
mixed-celled,  or  alveolar.  Very  vascular  sarcomas,  or  angiosarcomas, 
have  been  described.  In  certain  cases,  small  spicules  or  nodular  con- 
cretions of  mineral  matter  are  found  in  these  growths.  Hence,  they  are 
called  psammosarcomas.  Sarcomas  of  the  dura  may  erode  the  bone  and 
appear  externally.  They  are  sometimes  of  large  size,  and  may  produce 
serious  pressure  upon  the  brain. 

Endotheliomas. — Endotheliomas  are  firm,  flattened,  or  nodular  tumors, 
originating  from  the  endothelial  cells  covering  the  dura,  or,  possibly, 
from  the  lining  membrane  of  the  vessels  of  the  subdural  space.  The 
growth  readily  implicates  the  pia-arachnoid,  and  may  finally  extend  to 
the  brain,  which  it  compresses  or  invades.  Irritation  of  some  kind 
seems  to  be  a  potent  factor  in  the  causation.  We  have  twice  seen, 
post  mortem,  cases  in  which  a  spur  of  bone  projecting  from  the  inner 
surface  of  the  parietal  bone  formed  the  centre  about  which  an  endo- 
thelioma developed.  Occasionally,  these  tumors  spring  from  the  outer 
surface  of  the  dura  and  erode  the  calvarium,  finally  appearing  externally. 


ENDOTHELIOMAS 
FIG.  148 


539 


- 


Osseous  metaplasia  in  the  falx  cerebri.      The  bone  is  the  dark  irregular  mass  in  the  central 
portion.     (Pathological  Museum  of  McGill  University.) 


FIG.  149 


Endothelioma  of  the  brain.  The  case  is  referred  to  in  the  text, 
tumor  resulting  from  the  exostosis  of  the  calvarium  can  be  recognized. 
Laboratory  of  the  Royal  Victoria  Hospital.) 


The   depression   in   the 
(From  the  Pathological 


540 


THE  DURA 


Microscopically,   they    present 
and    anastomosing   bands  of 


FIG.  150 


I®    I, 


Portion  of  an  endothelioma  of  the  dura 
mater,  showing  the  characteristic  whorled 
arrangement  of  the  tumor  cells  and  at 
a,  a  concentrically  arranged  calcareous 
deposit  or  psammoma  body.  (P.  Ernst.) 


the  appearance  of  richly  branching 
flattened  cells,  tending  to  be  spindle- 
shaped,  with  a  characteristic  concen- 
tric arrangement. 

It  is  not  infrequent  to  meet  with 
tumors  showing  histological  transitions 
from  the  endotheliomatous  to  the  sar- 
comatous  type  (vide  vol.  i,  p.  757).  The 
commonest  form  of  psammoma  is  a  slow- 
growing,  relatively  benign  endothelioma, 
with  necrosis  of  the  "cell-nests"  and 
deposit  of  calcareous  salts  in  the  clusters 
of  necrosed  cells. 

Carcinoma. — Carcinoma  is  occasionally 
met  with  as  a  metastatic  growth. 

Chordoma  is  a  curious  tumor  origi- 
nating at  the  base  of  the  skull  in  the 
upper  termination  of  the  notochord. 
It  is  situated  near  the  clivus  and  in- 
vades the  dura  secondarily  (see  vol.  i, 
p.  699). 

FIG.  151 


t      « 


.»»  »" 


•:'v  V--::  "%*•:; 


*    »    * 


f  .•          \    •   '     we 
*'•&?  -'*    ' 

Section  of  a  chordoma.  To  the  right  the  cells  are  of  the  benign  type,  not  unlike  in  arrange- 
ment those  of  cartilage;  to  the  left,  through  active  multiplication  the  cells  are  taking  on  a  more 
sarcomatous  type  and  the  growth  is  becoming  malignant.  (Fischer.) 


LEPTOMENINGITIS  541 

The  Pia-arachnoid. 

The  arachnoid  is  a  delicate,  connective-tissue  membrane,  devoid 
of  bloodvessels,  covering  the  brain,  and  lying  in  close  relationship 
with  the  dura.  The  space  between  the  two  is  known  as  the  subdural 
space.  The  pia  is  also  a  delicate  connective-tissue  membrane,  but 
vascular.  It  closely  follows  the  contour  of  the  cerebrum,  dipping  down 
into  the  sulci  and  sending  prolongations,  which  carry  bloodvessels  and 
lymphatics,  into  the  cortical  substance.  The  arachnoid,  on  the  con- 
trary, passes  from  top  to  top  of  the  convolutions.  The  space  between 
the  arachnoid  and  pia  contains  the  cerebrospinal  fluid,  and  is  called  the 
subarachnoid  space.  Passing  from  one  membrane  to  the  other  are 
innumerable  bands  and  strands  of  connective  tissue,  covered  with  a 
continuation  of  the  endothelium  lining  the  sac. 

At  the  base,  the  pia  and  arachnoid  are  widely  separated  from  one 
another,  in  certain  situations.  Thus,  in  the  interpeduncular  space  and 
posteriorly  between  the  posterior  surface  of  the  medulla  and  the  inferior 
surface  of  the  cerebellum  quite  large  reservoirs  are  formed  for  the 
cerebrospinal  fluid.  The  chief  bloodvessels  run  in  the  subarachnoid 
space;  the  veins  lie  superficially,  the  arteries  at  the  bottom  of  the  sulci. 

The  pia  mater  is  continued  into  the  lateral  ventricles  through  the 
transverse  fissure  in  the  form  of  the  tela  choroidea  superior,  and  into 
the  fourth  ventricle  in  the  form  of  the  tela  choroidea  inferior,  carrying 
with  it  the  choroid  plexus  of  vessels.  The  subarachnoid  space  is  con- 
tinuous with  the  ventricular  cavities  through  the  foramen  of  Majendie  in 
the  lower  end  of  the  fourth  ventricle. 


CIRCULATORY  DISTURBANCES. 

The  circulatory  disturbances  affecting  the  pia-arachnoid  will  be  more 
conveniently  discussed  when  treating  of  the  brain,  inasmuch  as  there  is 
such  an  intimate  relationship  between  the  brain  and  its  membranes  that 
the  same  causes  often  produce  analogous  and  simultaneous  results  in 
both  (see  p.  551). 

INFLAMMATIONS. 

Leptomeningitis. — Inflammation  of  the  pia-arachnoid,  leptomenin- 
gitis,  or,  as  it  is  usually  more  briefly  termed,  meningitis,  may  be  acute 
or  chronic. 

Acute  Leptomeningitis. — Acute  leptomeningitis  is,  in  the  vast  majority 
of  cases,  produced  by  microorganisms,  probably  the  only  exceptions 
being  those  forms  met  with  in  sunstroke  and  the  various  intoxications. 
The  germs  usually  at  work  are  the  Pyogenic  cocci,  the  B.  tuberculosis, 
the  Diplococcus  pneumonias,  the  Meningococcus  intracellularis,  and, 
occasionally,  the  B.  typhi,  the  B.  coli,  the  B.  Friedlanderi,  the  B.  pyo- 
cyaneus,  B.  influenzas,  B.  diphtherias,  B.  anthracis,  the  Gonococcus  and 
the  Actinomyces  bovis. 


542  THE  PIA-ARACHNOID 

The  infecting  agents  reach  the  meninges  either  through  the  blood 
(hematogenic  infection),  by  extension  from  neighboring  parts,  or  from 
the  external  air.  The  hematogenic  form  is  well  illustrated  in  those 
cases  which  complicate  croupous  pneumonia,  endocarditis,  acute  rheu- 
matism, pulmonary  tuberculosis,  typhoid  fever,  scarlatina,  pleurisy, 
and  bedsores.  The  second  variety  is  due  to  the  extension  of  an  inflam- 
matory process  from  the  brain,  dura,  or  bones  of  the  skull.  Of  especial 
importance  in  this  connection  are  affections  of  the  middle  ear,  the  mastoid 
cells,  and  the  accessory  cavities  of  the  nose.  Those  arising  from  external 
infection  are  the  result  of  traumatism,  such  as  fractures  and  penetrating 
wounds  of  the  skull. 

While  in  many  cases  the  meningitis  is  due  to  the  particular  micro- 
organism producing  the  primary  disease,  examples  of  secondary  and 
mixed  infection  are  not  uncommon. 

It  is  hardly  possible  for  the  pia-arachnoid  to  be  inflamed  without  cor- 
responding changes  in  the  brain  substance,  at  least,  in  the  superficial 
layers  of  the  cortex.  This  is,  in  part,  due  to  the  close  apposition  be- 
tween the  structures  in  question,  so  that  lesions  are  readily  produced 
by  direct  extension,  but  also  in  some  measure  to  the  dissemination  of 
infective  agents  through  the  bloodvessels  and  lymphatics  of  the  pia 
which  send  minute  branches  into  the  connective-tissue  septa  of  the 
cortex.  The  cells  of  the  gray  matter,  therefore,  commonly  suffer, 
showing  fatty  degeneration,  vacuolation,  and  changes  in  the  chromo- 
philic  bodies.  Multiple,  small  ecchymoses  are  often  to  be  seen  in  the 
cortex.  In  well-marked  cases  the  condition  would  properly  be  termed 
acute  meningo-encephalitis. 

Acute  Serous  Meningitis. — Acute  serous  meningitis  (Quincke)  is  an 
important  affection  of  the  pia-arachnoid,  often  of  the  cortex,  character- 
ized by  congestive  hyperemia,  oedema,  and  the  production  of  a  serous 
and  cellular  exudate.  It  is  met  with  most  frequently  in  children  at  the 
onset  or  during  the  course  of  certain  of  the  infective  fevers,  such  as 
scarlatina  and  measles.  It  is  found,  however,  in  adults  also,  as  a  re- 
sult of  traumatism,  and  in  those  suffering  from  obstructive  cardiac  dis- 
ease, alcoholism,  uremia,  and  other  intoxications.  The  causative  factors 
are  not  always  entirely  clear. 

The  anatomical  picture  is  not  constant.  In  certain  cases  the  inflam- 
matory products  are  so  scanty  that  the  condition  is  only  detected  on 
microscopic  examination.  In  the  severer  forms,  the  pia-arachnoid  is 
congested,  oedematous,  and  the  subarachnoid  space  contains  a  con- 
siderable quantity  of  watery  exudation,  sometimes  clear,  sometimes 
turbid  and  containing  flakes.  Along  the  vessels  the  fluid  is  apt  to  be  of 
a  yellowish  color  and  gelatinous  consistency.  The  dura  is  usually  tense 
and  injected,  somewhat  moist  and  shiny  on  the  inner  surface.  If  the 
exudate  be  at  all  extensive,  the  membranes  are  distended  and  the  convolu- 
tions flattened.  The  extent  of  the  disease  varies  and  the  lesions  are,  as 
a  rule,  irregularly  distributed,  being  most  marked  along  the  course  of 
the  main  vessels.  At  one  time  the  involvement  is  most  marked  at  the 
base,  at  another,  over  the  sides  and  convexities.  The  arachnoid  usually 


ACUTE  SEROUS  MENINGITIS  543 

peels  off  readily,  leaving  a  moist,  congested,  and  oedematous  pia.  Minute 
hemorrhages  may  also  be  seen  in  the  pia  and  the  superficial  layers  of  the 
cortex.  Occasionally,  the  disease  is  localized  to  one  part  of  the  cortex 
owing  to  the  delimitation  of  the  exudation  by  adhesions.  In  addition 
to  the  meningeal  involvement  the  disease  may  extend  to  the  lining  of 
the  ventricles  and  produce  a  considerable  accumulation  of  fluid  there 
(hy  drops  ventriculorum). 

We  may,  further,  recognize  a  seropurulent,  fibrinopurulent,  and  purulent 
meningitis,  according  to  the  nature  of  the  exudate  produced.  These 
include  the  majority  of  meningitis  cases.  The  exudate  is  more  cellular, 
consisting  of  a  turbid  serous  fluid  containing  numerous  leukocytes  and, 
sometimes,  flakes  of  fibrin,  or  it  may  be  entirely  purulent.  The  exudate 
tends  to  collect  about  the  vessels  and  in  the  sulci,  but  in  severe  cases  the 
brain  may  be  bathed  in  exudate. 

The  distribution  of  the  lesions  is  dependent  largely  upon  the  cause  and 
the  nature  of  the  infecting  agents.  Hematogenic  causes  may  affect  the 
base,  the  convexity,  and  practically  any  part  of  the  membranes.  Trau- 
matism,  caries  of  the  petrous  bone,  and  infection  of  the  accessory  cavities 
lead  to  a  local  lesion,  which  may,  however,  become  general.  Again,  the 
cranial  cavity  alone  may  be  involved,  or  the  whole  central  nervous  system, 
as  in  the  case  of  epidemic  cerebrospinal  meningitis. 

The  cerebral  cortex,  of  course,  suffers.  It  is  oedematous,  the  vessels 
are  congested,  and  there  are  frequently  multiple  minute  hemorrhages. 
The  vessel  walls  and  the  connective-tissue  septa  are  infiltrated  with 
inflammatory  products.  The  ganglion  cells  are  swollen,  vacuolated,  and 
fatty,  the  axis-cylinders  disintegrated.  In  cases  where  the  process  has 
extended  to  the  ventricles,  the  choroid  plexus  is  congested,  swollen, 
bathed  in  pus,  and  infiltrated  with  inflammatory  products.  The 
ependyma  and  the  underlying  brain  substance  are  oedematous  and 
softened.  Should  the  exudate  be  excessive,  the  ventricles  are  distended 
and  the  brain  compressed.  The  gyri  are  flattened,  the  cerebrospinal 
fluid  is  squeezed  out,  and,  as  a  result,  the  meninges,  which  were  pre- 
viously oedematous,  now  become  drier. 

As  a  rule,  purulent  meningitis  is  fatal,  but  the  milder  and  more  local- 
ized forms  are  sometimes  recovered  from.  In  such  cases  the  only  traces 
left  of  the  trouble  are  a  thickening  of  the  pia-arachnoid,  with  possibly 
adhesions  between  it  and  the  dura.  This  is  due  to  absorption  and 
the  organization  of  the  exudate  into  fibrous  tissue.  Purulent  meningitis 
is,  in  most  cases,  a  complication  of  disease  elsewhere,  and  may  usually 
be  traced  to  pneumonia,  acute  endocarditis,  infection  of  the  nasal  or  aural 
cavities,  traumatism,  or  to  terminal  infection  in  some  chronic  disease. 
Here  the  pyogenic  cocci,  the  Pneumococcus,  and  the  B.  coli  play  the 
leading  role,  less  commonly  the  B.  influenzae,  B.  typhi,1  B.  diphtheriae, 
B.  Welchii,  and  the  Gonococcus. 

Occasionally,  cases  crop  up,  either  sporadically  or  in  epidemics,  which 
run  a  somewhat  characteristic  course,  and  cannot  be  attributed  to  any 

1  T.  Henry  and  Rosenberger,  Proc.  Path.  Soc.  of  Phila.,  February,  1908:  52. 


544  THE  PIA-ARACHNOID 

of  the  causes  just  mentioned.  This  variety  is  called  acute  cerebrospinal 
meningitis,  epidemic  cerebrospinal  meningitis,  "spotted  fever,"  and 
cerebrospinal  fever.  Here  the  Diplococcus  intracellularis  meningitidis 
(Weichselbaum),  or  the  Pneumococcus,  is  found,  either  alone  or 
associated  with  other  pyogenic  germs i  At  one  time  it  was  debated 
which  of  the  two  microorganisms  above  mentioned  was  the  specific 
cause  of  the  disease.  Weichselbaum1  was  the  first  to  recognize  and 
describe  a  diplococcus  in  these  cases,  tending  to  be  intracellular,  which 
he  regarded  as  specific  and  named  the  Diplococcus  intracellularis 
meningitidis.  His  observations  were  afterward  confirmed  by  Heubner2 
in  Germany,  and  Councilman,  Mallory,  and  Wright3  in  America. 
Netter,4  however,  strongly  maintained  that  the  pneumococcus  was  the 
important  agent,  and  held,  though  it  now  seems  on  inconclusive 
evidence,  that  the  Weichselbaum  organism  is  merely  a  degenerate  form 
of  this  germ.  At  the  present  time  the  majority  of  authorities  concede 
the  specificity  of  the  Diplococcus  intracellularis  for  most  cases.  The 
agglutination  test  and  the  favorable  results  following  the  therapeutic 
use  of  a  specific  serum  (Flexner)  place  the  distinction  between  the 
bacteria  mentioned  beyond  question.  The  relative  frequency  of  the 
two  microorganisms  in  the  disease  is  not  positively  settled.  Combined 
infection  seems  to  be  a  common  event,  and  it  has  been  thought  that 
where  the  pneumococcus  has  been  found  alone  the  Diplococcus  intra- 
cellularis may  have  been  present  but  have  died  out,  as  its  tenacity  of 
life  is  known  not  to  be  great. 

In  the  form  of  cerebrospinal  meningitis  under  discussion,  the  exudate 
is  abundant  and  found  throughout  the  whole  extent  of  the  central  nervous 
system.  At  one  time  the  membranes  of  the  cerebrum  are  chiefly  in- 
volved, at  another,  those  of  the  cord.  As  a  rule,  the  exudate  tends  to 
collect  at  the  base  of  the  brain  and  along  the  posterior  aspect  of  the  cord. 
The  exudate  is  largely  serous,  but  turbid  from  the  admixture  of  leuko- 
cytes and  a  small  amount  of  fibrin.  On  removing  the  skull  cap,  the 
veins  of  the  diploe,  and  the  vessels  and  sinuses  of  the  dura  are  congested. 
The  arachnoid  is  somewhat  turbid  and  the  pial  vessels  are  injected. 
The  exudate  is  found  chiefly  along  the  course  of  the  vessels,  and  fills 
up  the  cisterns  at  the  base  of  the  brain,  and  may  even  extend  to  the 
ventricles,  which  are  often  found  to  be  distended.  The  pus  produced 
by  the  Diplococcus  pneumonise  is  somewhat  different,  being  of  a  creamy 
yellowish-green  color,  more  viscid  and  rarely  mixed  with  blood.  Micro- 
scopically, the  vessels  of  the  cortex  are  congested  and  surrounded  by 
aggregations  of  leukocytes.  There  are  multiple  small  hemorrhages  and 
large  areas  of  necrosis.  It  is  not  uncommon  to  find  lesions  in  other  parts 
of  the  body  as  well  as  in  the  meninges.  Arthritis  is  comparatively 

1  Ueber  d.  Etiologie  der  acuten  Meningitis  Cerebrospinalis,  Fortschr.  d.  Med.,  5  : 
1887  :Nos.  18  and  19. 

2  Deut.  med.  Woch.,  1897. 

3  Journ.  Boston  Soc.  Med.  Scien.,  2: 1897-8:  53. 

4  Bull,  et  Mem.  Soc.  Med.  d.  Hop.  de  Paris,  15:  1898:  407. 


TUBERCULOSIS  545 

frequent,  being  found  more  often  in  this  disease  than  in  other  forms  of 
meningitis.  The  Diplococcus  intracellularis  has  been  isolated  from  the 
joints  in  these  cases.  Multiple  abscesses,  petechial  spots,  herpes  and 
other  rashes  have  been  observed.  A  rare  complication  is  purulent  peri- 
carditis.1 These  facts  seem  to  indicate  that  cerebrospinal  meningitis  is 
really  a  systemic  infection,  the  meningeal  features  of  which  dominate  the 
clinical  picture.2  The  distinction  between  the  form  just  described  and 
that  due  to  a  frank  Diplococcus  pneumonise  infection  is  marked,  patho- 
logically speaking,  for  in  the  latter  affection  the  exudate  is  thick,  puru- 
lent, and  of  a  greenish  hue. 

Meningo-encephalitis  is  sometimes  met  with  in  epidemics  of  influenza, 
and  is  one  cause  of  hemiplegia  and  aphasia  in  young  persons. 

W.  T.  Howard,  Jr.,  has  reported  a  case  of  acute  fibrinopurulent 
cerebrospinal  meningitis  with  abscess  of  the  brain  due  to  the  B.  Welchii.3 

Chronic  Lepto meningitis. — Chronic  leptomeningitis,  etiologically  speak- 
ing, is  a  somewhat  obscure  affection.  Some  cases  result  from  a  previous 
acute  attack,  and  are  mostly  of  an  infective  nature.  They  are  met  with 
as  sequelae  of  cerebrospinal  meningitis,  typhoid  fever,  acute  rheumatism, 
erysipelas,  and  syphilis.  Others,  again,  are  insidious  in  their  develop- 
ment and  are  possibly  in  many  cases  to  be  regarded  as  degenerative 
manifestations,  inasmuch  as  they  seem  to  depend  on  vascular  disturbances 
or  circulating  toxins  rather  than  microbic  influences.  Such  are  the  latent 
meningitides  sometimes  met  with  in  chronic  alcoholism  and  Bright's 
disease.  They  are  apt  to  be  combined  with  encephalitis  (chronic  meningo- 
encephalitis).  Some  few  cases  may  possibly  be  attributed  to  excessive 
mental  strain  or  nervous  shock.  Chronic  leptomeningitis  is  at  times 
associated  with  pachymeningitis  interna  proliferans. 

The  anatomical  picture  varies  greatly  in  different  cases.  Local  or 
diffuse  thickenings  of  a  whitish  color,  in  the  form  of  streaks  or  patches, 
may  be  found  on  the  pia-arachnoid.  They  are  composed  of  hyperplastic 
connective  tissue  and  are  often  the  sole  relics  of  a  previous  acute  inflam- 
mation. Some,  however,  are  more  probably  the  result  of  circulatory 
or  nutritional  disturbances.  Proliferation  of  the  lining  endothelium  is 
also  to  be  seen  in  some  instances.  In  the  more  severe  forms  cellular 
infiltration  plays  a  more  striking  part.  This  is  met  with  in  the  neighbor- 
hood of  chronic  suppurating  foci,  syphilitic  and  tuberculous  bone  disease, 
tumors,  and  areas  of  degeneration. 

Tuberculosis. — Tuberculosis  of  the  pia-arachnoid  is  a  common  occur- 
rence, particularly  in  children  and  young  adults.  It  is  probably  never 
primary,  but  in  all  cases  is  attributable  to  tuberculous  disease  elsewhere. 
The  affection  is  brought  about  by  metastasis,  usually  from  some  focus 
in  the  lungs  or  lymphatic  glands,  or  by  direct  extension  from  the  dura 
mater  or  bones  of  the  skull. 

1  Stewart  and  Martin,  Montreal  Med.  Jour.,  27:  1898:  159. 

2  For  a  very  full  consideration  of  this  disease,  see  Osier,  on  the  Etiology  and  Diag- 
nosis of  Cerebrospinal  Fever,  Cavendish  Lecture,  1899. 

3  Johns  Hopkins  Hospital  Bulletin,  10:1899:66. 

35 


546  THE  PIA-ARACHNOID 

Anatomically,  two  forms  may  be  recognized,  the  diffuse  miliary  and 
the  solitary  tubercle.  The  first  variety  is  due  to  the  dissemination  of 
considerable  numbers  of  the  specific  bacilli  through  the  arterial  system. 
It  is  frequently,  but  by  no  means  invariably,  part  and  parcel  of  a  systemic 
miliary  tuberculosis.  Diffuse  tuberculous  meningitis,  or  miliary  tuber- 
culosis of  the  pia-arachnoid,  is  characterized  by  the  formation  of  whitish 
or  grayish-white  granules,  the  size  of  a  pin-head  or  smaller,  in  the  pia, 
which  tend  to  be  scattered  along  the  course  of  the  vessels.  They  are 
usually  most  numerous  at  the  base  of  the  brain,  about  the  chiasm,  at 
the  anterior  and  posterior  perforated  spaces,  the  circle  of  Willis,  and 
along  the  Sylvian  fissures.  The  process  frequently  spreads  by  the  Sylvian 
arteries  and  its  branches  to  the  convexity  of  the  brain.  The  condition 
is  usually  bilateral,  but  it  is  not  uncommon  to  find  one  side  more  affected 
than  the  other.  Exceptionally,  one  side  only  is  involved.  It  may,  how- 
ever, be  still  more  restricted  in  extent.  We  remember  seeing  one  case 
in  which  there  were  attacks  of  Jackson ian  epilepsy  due  to  the  presence 
of  a  cluster  of  a  dozen  or  more  milia  over  a  portion  of  the  motor  area. 
Associated  with  the  efflorescence  of  tubercles  is  a  more  or  less  abundant 
exudate  of  a  serous,  seropurulent,  or  fibrinopurulent  appearance,  which 
accumulates  not  only  in  the  meshes  of  the  pia-arachnoid,  but  also  in 
the  various  lymph-cisterns,  the  ventricles,  and  even  in  the  brain  substance. 
On  removing  the  skull  cap,  the  dura  is  congested  and  weeps  blood. 
The  pia-arachnoid  is  congested  and  oedematous,  and  along  the  course  of 
the  vessels  can  be  seen  minute  tubercles,  with  sometimes  petechial  hemor- 
rhages. The  basal  convolutions  are  often  somewhat  compressed.  The 
cortex  is  swollen  and  oedematous.  The  ventricles  contain  a  variable 
amount  of  fluid,  similar  to  the  exudation  elsewhere,  the  choroid  plexus  is 
thickened,  and  the  ependyma  has  a  turbid  granular  appearance.  In- 
ternal hydrocephalus  is  not  an  uncommon  result.  It  may  be  due  to 
involvement  of  the  ependyma  of  the  ventricles,  or  to  obstruction  of  the 
communicating  passage  between  the  ventricles  and  the  subarachnoid 
space.  In  some  few  cases  the  amount  of  exudate  may  be  considerable, 
without  obvious  tubercles,  but  these  can  usually  be  recognized  by  the 
use  of  a  hand-lens  or  on  microscopic  investigation.  Occasionally,  the 
granulations  are  the  most  marked  feature,  while  the  exudation  is  scanty 
(dry  tuberculous  meningitis). 

The  process  begins  by  a  specific  inflammation  of  the  walls  of  the  smaller 
arterioles  and  capillaries.  Small  collections  of  leukocytes  and  epithelioid 
cells  are  formed  in  the  vessel  walls,  which  increase,  gradually  extending 
into  the  lumina  and  into  the  peri  vascular  lymph-spaces.  In  the  first 
stage  there  is  a  proliferation  of  the  endothelial  lining  of  the  bloodvessels, 
leading  to  more  or  less  obliteration  of  the  lumina,  but  this  is  quickly 
masked  by  the  exudative  process.  These  changes  at  first  are  confined 
to  the  pial  vessels  but  quickly  extend  to  the  superficial  vessels  of  the 
cortex  lying  in  the  fibrous  trabeculse,  and  eventually  invade  the  nerve 
substance  (tuberculous  menin  go-encephalitis).  This  leads  to  swelling 
and  degeneration  of  the  cortical  ganglia  and  nerve  fibers.  The  main 
nerve-trunks  passing  out  from  the  base  of  the  brain  may  be  similarly 


SYPHILIS  547 

involved.  The  milia  quickly  undergo  central  caseation,  thus  becoming 
more  opaque.  Only  in  extremely  chronic  cases  do  the  granules  attain 
any  considerable  size.  Tubercle  bacilli  can  be  demonstrated  within  the 
lymph-spaces  and  in  the  granulation  tissue. 

The  disease  is  almost  invariably  fatal  in  a  few  weeks,  although  one  or 
two  authentic  cases  of  recovery  are  on  record,  in  one  at  least  of  which 
the  tubercle  bacilli  were  obtained  by  spinal  puncture. 

Should  only  a  few  bacilli  reach  the  meninges  through  a  single  arterial 
twig,  we  get  a  small  cluster  of  tubercles,  which,  in  time,  coalesce  to  form 
large  nodular  masses,  varying  in  size  from  that  of  a  walnut  to  that  of  a  hen's 
egg.  These  are  situated  in  the  pia-arachnoid,  but  frequently  encroach 
upon  the  substance  of  the  brain  or  extend  to  the  dura.  Unless  situated 
at  or  near  the  motor  area,  or  in  the  parts  connected  with  the  special 
senses,  they  may  remain  latent  and  unsuspected  for  a  long  time.  Such 
masses  are  nodular,  firmer  or  softer,  containing  in  their  centres  yellowish- 
white  caseous  detritus.  Occasionally,  owing  to  the  infiltration  of  fluid, 
the  degenerated  material  is  partially  liquefied,  so  that  the  tuberculoma 
resembles  an  abscess.  In  long-standing  cases,  the  nodule  may  become 
calcified.  As  a  rule,  the  necrotic  mass  is  surrounded  by  a  zone  of  grayish 
or  grayish-white,  semitranslucent  granulation  tissue,  or,  in  the  older  cases, 
dense  fibrous  tissue.  Where  the  affection  is  progressive,  secondary 
tubercles  may  often  be  noted  in  this  peripheral  zone.  Such  tuberculomas 
are  practically  tumors  and  produce  their  effects  by  pressure  and  by  inter- 
ference with  the  circulation  of  the  blood  and  lymph.  They  may  also 
lead  to  the  production  of  fresh  tubercles  in  the  adjacent  parts  of  the 
brain  or  to  a  disseminated  cerebral  miliary  infection.  This  result  is 
due  to  local  metastasis  through  the  lymph-stream  or  to  the  discharge 
of  infective  material  into  the  transverse  sinus.  Microscopically,  this 
form  does  not  differ  from  the  small  miliary  focus,  save  in  point  of  size 
and  the  relatively  much  greater  amount  of  caseation,  and  in  the  peripheral 
fibrosis. 

Syphilis. — The  meninges  are  a  rather  common  site  for  syphilitic 
processes.  The  parts  most  liable  to  be  involved  are  the  basal  and,  next, 
the  frontal  and  parietal  portions  of  the  membranes.  While  the  pia- 
arachnoid  is  chiefly  involved,  the  process  commonly  extends  to  the  cortex 
of  the  brain.  It  is  rare  for  the  central  regions  of  the  brain  and  cord  to 
be  invaded.  Meningitis  and  meningocephalitis  are  met  with  usually 
in  inveterate  cases  of  syphilis,  but  also,  although  rarely,  in  the  secondary 
stage. 

The  affection  is  characterized  by  two  main  features,  the  formation  of 
gummas  and  involvement  of  the  bloodvessels.  The  gummas  resemble 
other  infectious  granulomas  in  that  they  are  circumscribed  foci  of 
inflammation  which  pass  through  the  stages  of  exudation  and  infiltra- 
tion into  that  of  degeneration.  At  first  they  appear  as  small,  grayish, 
grayish-red,  semitranslucent  or  gelatinous  masses  composed  of  a  cellular 
granulation  tissue,  together  with  newly-formed  vessels.  Should  the 
process  advance,  there  is  a  gradual  formation  of  cellular  fibrous  tissue 
Accompanied  by  centra]  necrosis.  The  gummas  are  produced  first  in 


548  THE  PIA-ARACHNOID 

the  substance  of  the  pia-arachnoid  but  later  in  its  cortical  prolongations, 
and  even  in  the  nerve-substance  proper  (meningo-encephalitis  syphilitica 
gummosa).  The  bloodvessels  are  somewhat  peculiarly  involved.  All 
the  coats  of  the  vessels  are  infiltrated  with  inflammatory  products, 
which  eventually  give  place  to  fibrous  transformation.  The  striking 
feature,  however,  is  the  proliferation  of  the  endothelium,  brought  about 
by  division  of  its  cells,  followed  by  cellular  infiltration,  which  frequently 
results  in  marked  narrowing  or  even  obliteration  of  the  lumen.  Such  a 
state  of  things  naturally  predisposes  to  thrombosis,  and  this  is  an  impor- 
tant factor  in  producing  occlusion  of  the  vessels.  The  gummas  may  be 
single  or  multiple,  and  are  not  infrequently  localized  to  a  comparatively 
small  district.  It  is,  moreover,  not  uncommon  to  find  definite  large 
masses  or  diffuse  gummatous  infiltration.  Owing  to  growth  by  direct 
extension  or  by  the  coalescence  of  small,  separate  foci,  nodular  masses 
as  large  as  a  walnut  may  be  produced,  which  are  firm  in  texture  and 
on  section  present  yellowish  streaks  and  patches  due  to  degeneration. 
This  form  is  commonly  associated  with  proliferation  of  fibrous  tissue 
forming  dense  bands  about  the  gumma,  and  often  leading  to  adhesion 
with  the  overlying  dura.  In  progressive  cases  this  fibrous  tissue  in  its 
turn  undergoes  necrosis.  The  cerebral  substance,  as  one  would  expect, 
manifests  marked  change.  Advancing  gummas  lead  to  compression  and 
destruction  of  the  nervous  tissue,  while,  owing  to  the  vascular  changes, 
ischemic  necrosis  and  hemorrhages  are  not  uncommon. 

Actinomycosis. — So  far  as  is  known,  actinomycosis  is  always  second- 
ary, arising  by  extension  from  the  face  and  nasopharynx,  or  by  metas- 
tasis. The  meningitis  produced  is  either  localized  or  diffuse,  and  is 
accompanied  by  a  serous  or  fibrinous  exudate,  in  whi'ch  the  "sulphur 
grains"  of  the  actinomyces  may  be  discovered,  and  adhesion  of  the 
membranes.  There  seems  to  be  in  these  cases  a  tendency  to  invade  the 
walls  of  the  veins  and  sinuses. 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — These  are  chiefly  of  the  connective-tissue  type,  and  are 
found  not  only  in  the  external  pia-arachnoid,  but  also  in  the  telse  choroideae 
and  the  lining  membrane  of  the  ventricles. 

The  benign  forms,  fibroma,  lipoma,  myxoma,  chondroma,  and  osteoma, 
are  rare.  They  form  small,  nodular  or  lobulated  masses  which  com- 
press the  adjacent  brain  substance.  A  cystic  lymphangioma  has  also 
been  described. 

A  peculiar  growth,  the  exact  nature  of  which  is  somewhat  obscure,  is 
the  cholesteatoma  (Perlgeschwulst).  This  is  found  especially  in  the 
meninges  at  the  base  of  the  brain,  about  the  anterior  or  posterior  trans- 
verse fissures,  and,  occasionally,  in  the  substance  of  the  brain.  It  forms 
a  solitary  tumor  enclosed  in  a  fibrous  capsule,  or  else  multiple  free  nodules 
in  the  pia  or  brain.  On  section,  it  is  soft,  of  shining  white  appearance, 
with  a  silky  sheen.  Microscopically,  it  is  composed  largely  of  keratin- 


ENDOTHELIOMA  549 

ized  cells,  resembling  the  horny  epithelium  of  the  skin.  Most  authorities 
seem  to  think  that  it  is  endothelial  in  origin,  but  Ziegler  holds  that  it 
more  probably  arises  from  the  external  germinal  layer  or  misplaced 
epithelium.  This  view  is  supported  by  the  fact  that  hairs  are  sometimes 
found  within  the  growth. 

Dermoid  Cysts. — Dermoid  cysts  are  found  in  the  meninges,  but  are 
rare. 

Not  uncommonly  multiple  cysts  containing  watery  or  colloid  material 
are  met  with  in  the  choroid  plexus. 

Endothelioma. — The  most  important  of  the  malignant  growths  found 
in  the  pia-arachnoid  is  the  endothelioma,  a  tumor  of  somewhat  variable 
structure,  originating  in  the  endothelial  cells  lining  the  bloodvessels  and 
lymphatics  or  those  covering  the  arachnoid  and  lining  the  subarachnoid 
space.  Certain  of  these  growths  develop  around  vessels,  presumably 
from  the  endothelium  of  the  perivascular  lymphatics,  the  so-called 
peritheliomas,  though  it  should  be  remarked  that  it  is  not  always  easy  or 
possible  to  decide  whether  growths  presenting  this  particular  appearance 
originate  in  the  adventitia  (perithelium)  of  the  bloodvessels  or  in  the 
lining  endothelium  of  the  perivascular  lymphatics.1  No  doubt,  the 
majority  of  tumors  arising  in  the  pia  arachnoid  are  endotheliomas. 

Endotheliomas  form  circumscribed  or  diffuse,  superficial,  flattened 
masses,  of  firm  consistence,  and  of  grayish  or  grayish-red  color.  Rarely, 
they  are  melanotic.  In  general,  they  resemble  the  sarcomas,  although 
in  places  they  may  present  a  somewhat  carcinomatous  appearance. 
Where  the  connection  with  the  lymphatics  or  bloodvessels  can  be  traced, 
the  older  cells  composing  the  tumor  are  flattened  or  spindle-shaped, 
resembling  endothelial  cells,  but  in  the  newer,  more  rapidly  growing 
parts,  the  cells  lose  this  character  and  come  to  resemble  closely  the  poly- 
morphous cells  of  certain  carcinomas.  Solid  strands  or  masses  of  such 
cells  in  a  connective-tissue  stroma  often  give  the  tumor  an  alveolar 
appearance.  Not  infrequently  the  cells  are  laid  down  more  or  less 
concentrically  in  layers,  after  the  fashion  of  whorls.  Besides  the 
endothelioma,  ordinary  types  of  sarcoma  are  met  with,  myxo sarcoma, 
angiosarcoma,  angiomyxosarcoma.  It  is  not  uncommon  to  find  in  the 

1  A  word  or  two  of  explanation  and  criticism  may  not  be  out  of  place  here.  The 
term  perithelioma,  meaning  a  tumor  derived  from  perithelium,  came  into  vogue 
some  fifteen  years  ago.  It  was  applied  to  a  growth  of  a  special  histological  type, 
the  main  feature  being  the  grouping  of  the  newly-formed  tumor  cells  about  the 
bloodvessels  in  a  radial  fashion,  recalling,  to  make  a  somewhat  crude  comparison, 
the  relationship  of  the  spokes  to  the  hub  of  a  wheel.  The  word  later  came  to  be 
applied  in  many  quarters  to  any  tumor  having  this  peculiar  arrangement.  Strictly 
speaking,  before  we  should  term  a  tumor  a  perithelioma,  we  should  be  able  to  demon- 
strate the  continuity  of  the  tumor  cells  with  those  of  the  perithelium  or  adventitia. 
Such  a  growth  is  not  a  lining  membrane  tumor,  but  is  really  a  form  of  sarcoma. 
Perhaps,  to  avoid  confusion,  the  old  term  "  angiosarcoma"  would  be  preferable. 
Many  of  the  growths,  however,  presenting  this  radiate  arrangement  originate  from 
the  lining  cells  of  the  perivascular  lymphatics  and  should  be  called  "perivascular" 
or  "  periangial"  endotheliomas. 


550 


THE  PIA-ARACHNOID 


choroid  plexus  small  granular  concretions,  the  so-called  "sand  bodies." 
On  occasion,  they  are  found  in  considerable  numbers  in  tumors,  notably 
endotheliomas  and  sarcomas,  giving  them  a  hard,  gritty  character 
(psammoma,  psammosarcoma).  They  vary  in  size  and  shape,  being 
laminated,  rounded,  irregular,  or  spinous. 

Carcinomas. — Carcinomas  are  found  in  the  ventricles  and  generally 
arise  from  the  choroid  plexus  or  from  the  epithelial  layer,  more  rarely 
from  the  ependyma.  They  form  soft  growths  composed  of  a  fibrous 
stroma  in  which  are  nests  of  epithelial  cells  of  cylindrical  type.  In  some 
cases  the  stroma  is  vascular  and  proliferates,  giving  to  the  tumor  a 
papillary  appearance.  The  stroma  often  undergoes  mucinous  degen- 

FIG.  152 


Endothelioma  (psammoma) , 


Winckel  obj.  No.  3,  without  ocular.     (From  the  collection 
of  A.  G.  Nicholls.) 


eration,  so  that  the  growth  presents  a  peculiar  appearance.  Owing 
to  the  accumulation  of  the  mucin,  the  papillae  are  not  infrequently 
converted  into  cysts.  These  are  bounded  by  epithelial-cell  masses,  which 
in  time  may  form  a  new  fibrous  stroma  sometimes  containing  cell-nests, 
not  unlike  the  "pearls"  so  commonly  found  in  cutaneous  epitheliomas. 
The  neoplasm  usually  remains  localized  to  the  ventricle,  producing  its 
effects  mainly  by  pressure,  but  occasionally  secondary  nodules  are  formed 
within  the  brain  substance. 

Parasites. — Among  the  animal  parasites  may  be  mentioned  the 
Echinococcus  and  the  Cysticercus.  The  echinococcus  leads  to  the  forma- 
tion of  single  or  multiple  cysts  which  press  upon  the  brain  substance  and 
result  in  its  degeneration.  The  cysticercus  is  met  with  usually  in  the 


(EDEMA  551 

form  of  a  small  cyst  with  a  scolex,  or  as  the  so-called  cysticercus  race- 
mosus.  Here,  there  are  large,  tabulated,  and  generally  sterile  cysts, 
presenting  internally  and  externally  grape-like  masses  of  secondary  or 
daughter-cysts.  In  the  neighborhood  connective-tissue  proliferation  is 
to  be  observed.  Such  cysts  may  become  calcified. 


THE  CEREBRUM  AND  CEREBELLUM. 
CIRCULATORY  DISTURBANCES. 

The  amount  of  blood  present  in  the  brain  and  its  membranes  varies 
widely  even  under  normal  conditions  being  dependent  on  increased  or 
diminished  function,  emotion,  and  divers  peripheral  impressions. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  occurs  patho- 
logically in  excessive  action  of  the  heart  and  whenever  arterial  tension 
is  diminished.  It  may  be  general  or  local.  Dilatation  of  the  arterioles 
with  increased  blood  supply  may  be  due  to  drugs,  such  as  amyl  nitrite, 
nitroglycerin,  and  alcohol,  or  to  sunstroke. 

Passive  Hyperemia. — Passive  hyperemia  of  the  brain  and  its  membranes, 
as  of  other  organs,  is  commonly  due  to  obstruction  to  the  free  outflow  of 
blood  from  the  part.  This  is  met  with  in  chronic  cardiac  and  pul- 
monary diseases,  and  in  the  paresis  of  the  cerebral  vessels,  which  results 
from  increased  intracranial  pressure.  The  condition  is  found  in  death 
by  suffocation  and  in  those  dying  in  the  status  epilepticus.  Local 
passive  congestion  is  due  to  thrombosis  or  the  pressure  of  tumors  or 
exudates  upon  the  efferent  veins  and  sinuses,  or,  again,  in  some  cases,  to 
the  recumbent  position.  Owing  to  the  delicacy  of  the  meninges  and  the 
comparatively  unsupported  condition  of  the  vessels,  hyperemia  is  more 
readily  detected  there  post  mortem  than  in  the  case  of  the  brain.  In  the 
latter  organ,  one  sees  at  most  slight  distension  of  the  smaller  vessels  and 
capillaries  with  blood,  which  on  section  exudes  in  minute  drops. 
Sometimes  the  substance  of  the  brain  presents  a  slight  rosy  flush.  The 
appearances  are,  however,  very  inconstant,  and  congestion  may  dis- 
appear entirely  after  death. 

Anemia. — Anemia  of  the  brain  is  due  to  general  systemic  anemia,  or 
any  cause  which  interferes  with  the  proper  supply  of  blood  to  the  part. 
Endarteritis,  spasm  of  the  vessels,  cardiac  weakness,  aortic  disease, 
increased  intracranial  pressure,  as  from  exudate  into  the  subarachnoid 
space,  meningeal  hemorrhage,  hy drops  ventriculorum,  tumors,  all  play 
a  part  here.  In  other  cases  anemia  may  be  collateral,  due  to  an  excessive 
accumulation  of  blood  in  some  other  part,  the  abdominal  cavity,  for  in- 
stance. This  is  seen  in  the  case  of  an  ordinary  "faint."  Local  anemia 
occurs  also  from  partial  or  complete  obstruction  of  the  arteries,  or  from 
emboli,  thrombi,  or  pressure. 

(Edema. — Circulatory  disturbances,  particularly  changes  in  the  vessel 
walls,  lead  to  cedema  of  the  brain  in  cases  where  the  outpoured  plasma 
is  not  promptly  removed  by  the  lymph-channels.  On  section,  the  brain 


552  THE  BRAIN 

is  pale,  moist,  and  shiny.  The  condition  is  brought  about  by  heart 
weakness,  obstruction  to  the  general  circulation,  thrombosis  of  the  sinuses 
of  the  dura.  Local  oedema  is  also  found  in  the  neighborhood  of  hemor- 
rhagic  exudates,  tumors,  and  thrombosed  veins.  Toxic  causes,  too, 
sometimes  play  a  part,  as  in  chronic  nephritis.  (Edema  is  a  frequent 
accompaniment  of  inflammation  of  the  brain  and  meninges. 

As  a  result  of  acute  inflammation  and  passive  congestion,  it  is  not  un- 
common to  find  a  transudation  of  fluid  into  the  ventricles  (hydrops 
ventriculorum;  hydrocephalus  inter nus),  which  leads  to  dilatation  of  the 
ventricles  and  compression  of  the  brain. 

Microscopically,  the  glia  is  looser  and  more  reticular,  and  the  lym- 
phatic channels  are  distended  with  fluid. 

Hemorrhage. — Cerebral  hemorrhage  is  of  comparatively  frequent 
occurrence,  as  compared  with  hemorrhage  into  other  organs. '  This  is 
probably  due  to  the  facts  that  (1)  the  vessels  of  the  brain  possess  scanty 
anastomoses;  (2)  they  are  given  off  from  vessels  of  much  larger  caliber 
than  themselves,  and  are  therefore  under  higher  pressure;  (3)  the 
muscular  coat  of  the  cerebral  arterioles  is  very  slight,  so  that  they 
can  offer  only  a  weak  resistance  to  a  dilating  force;  and  (4)  the  brain 
substance  offers  less  support. 

Two  factors  are  chiefly  concerned  in  its  causation,  degeneration  of 
the  walls  of  the  bloodvessels,  and  increased  blood  pressure.  It  may 
take  the  form  of  hemorrhage  per  rhexin  or  per  diapedesin.  Rupture 
of  vessels  is  most  likely  to  occur  in  infancy,  owing  to  inherited  disease 
of  the  vessels,  and  after  middle  life,  when  the  vessels  are  apt  to  be 
sclerosed.  Another  potent  cause,  traumatism,  may,  of  course,  occur  at 
any  age. 

Small  capillary  hemorrhages  are  met  with  in  congestive  hyperemia 
and  acute  encephalitis,  as  well  as  in  certain  of  the  infective  diseases,  such 
as  malaria,  variola,  anthrax,  and  the  hemorrhagic  diatheses.  In  some 
of  the  cases  the  extravasation  of  blood  is  brought  about  by  fatty  de- 
generation of  the  vessel  walls  or  by  the  obstructive  and  necrotizing  action 
of  bacterial  emboli,  or  even  the  blockage  of  vessels  by  simple  emboli  of 
broken-down  tissue  or  blood  cells.  In  such  cases  the  hemorrhages  are 
scattered  along  the  vessels.  They  vary  in  size  from  that  of  a  millet  seed 
to  a  pea,  and  are  present  both  in  the  brain  substance  and  in  the  cortical 
prolongations, of  the  pia.  When  in  the  latter  situation,  the  hemorrhages 
have  been  regarded,  but  incorrectly  so,  as  dissecting  aneurisms.  Ob- 
struction of  vessels  by  sclerosis  may  also  lead  to  hemorrhages,  usually 
of  small  extent.  Hemorrhages  are  also  due  to  congestion  brought 
about  by  the  pressure  of  tumors  or  exudates,  and  to  trauma  of  various 
kinds. 

Of  more  importance  is  spontaneous  hemorrhage  which  is  caused  by 
rupture  of  an  artery.  In  such  cases  there  is  almost  invariably  advanced 
degeneration  of  the  vessel  walls  caused  by  sclerosis,  calcification,  or 
inflammation.  In  the  common  condition  of  arterial  sclerosis  the  lesions 
may  be  fairly  generalized,  but  in  not  a  few  cases  some  particular  organ  is 
specially  picked  out,  such  as  the  brain,  heart,  or  kidney.  Sclerosis  being 


HEMORRHAGE  553 

primarily  a  degenerative  process  leads  to  weakening  of  the  vessel  walls. 
The  proliferative  changes  in  the  intima  result  in  more  or  less  obstruction 
in  the  lumen,  which  may  indeed  be  complete,  and  this  in  its  turn  con- 
tributes to  the  dilatation  of  the  other  parts  of  the  vessel.  Miliary 
aneurisms  are  therefore  rather  common,  being  present,  it  is  said,  in 
one-third  to  one-half  the  cases.  Rupture  of  the  vessel  is  precipitated  by 
increased  arterial  tension,  such  as  may  be  caused  by  excitement,  worry, 
mental  or  physical  overwork — in  fact,  any  condition  which  puts  sudden 
strain  on  the  vessel. 

The  most  frequent  site  for  this  form  of  hemorrhage  is  in  the  basal 
ganglia.  One  of  the  penetrating  branches  of  the  Sylvian  artery,  usually 
the  lenticulostriate,  is  the  vessel  at  fault.  Less  commonly,  the  hemor- 
rhage takes  place  into  the  pons,  the  peduncles,  the  cerebellum,  the  white 
matter,  and,  most  rarely,  into  the  convexity.  This  distribution  is  ac- 
counted for  by  the  fact  that  the  blood  pressure  is  greater  in  the  Sylvian 
artery  and  its  branches,  the  latter  coming  directly  off,  and  that  arterio- 
sclerosis is  usually  commoner  and  more  extensive  about  the  base  of 
the  brain.  The  effects  produced  depend  on  the  extent  of  the  extrava- 
sation. Minute  hemorrhages  lead  simply  to  pressure  upon  and  dis- 
location of  the  adjacent  brain  substance  without  much  further  disturb- 
ance, and  may  be  absorbed,  leaving  little  or  no  trace,  although  the 
clinical  symptoms  may  be  striking,  if  temporary.  When  large  vessels 
give  way,  extravasations  varying  in  size  from  that  of  a  pea  to  that  of  a 
walnut,  or  larger,  take  place,  leading  to  destruction  of  the  brain  tissue 
in  the  neighborhood  and  pressure  on  the  peripheral  parts.  In  the  most 
extreme  cases,  the  whole  of  the  corpus  striatum  on  one  side  may  be 
torn  up  and  the  blood  may  dissect  its  way  through  the  white  substance 
and  destroy  the  greater  part  of  the  posterior  lobe,  or  the  blood  may  even 
find  its  way  into  the  ventricle  or  into  the  meninges.  In  such  cases  the 
convolutions  are  compressed  and  the  brain  substance  in  other  parts  is 
anemic. 

In  the  early  stages  one  sees  in  the  affected  area  a  variable  quantity 
of  dark,  soft,  semifluid,  or  granulated  blood,  mixed  with  detritus  from  the 
destroyed  tissue.  In  the  neighboring  brain  substance  are  numerous 
petechial  hemorrhages,  the  result  of  the  sudden  disturbance  of  the  vascu- 
lar equilibrium.  Later,  the  fibrin  separates  more  completely  and  the 
serum  is  to  some  extent  removed  by  the  lymphatics,  so  that  the  pressure 
upon  the  brain  is  to  that  extent  at  least  relieved.  The  clot  gradually 
contracts,  becomes  more  granular,  and  changes  from  a  red  to  a  brownish 
color,  owing  to  the  transformation  of  the  hemoglobin  into  hematoidin. 
The  pigment  is  gradually  diffused  into  the  neighboring  tissues,  imparting 
to  them  a  yellowish  tinge.  In  time,  the  greater  part  of  the  fibrin,  cor- 
puscles, pigment,  and  detritus  is  transformed  and  absorbed,  so  that 
a  cavity  remains,  containing  a  clear  or  slightly  tinged  fluid  (degenerative 
or  apoplectic  cyst).  Sometimes,  however,  the  tissues  collapse  to  fill  up 
the  deficiency,  and  in  such  cases  there  is  compensatory  enlargement 
of  the  ventricles  and  the  subdural  space.  When  the  extravasation  is 
not  too  extensive  the  damage  is  made  good  by  the  formation  of  a  fibrous 


554  THE  BRAIN 

scar,  either  quite  firm,  or  enclosing  the  remains  of  the  destroyed  tissue, 
together  with  pigment  and  cholesterin  crystals.  Old  cysts  eventually 
become  walled  in  by  fibrous  tissue  derived,  it  is  believed,  from  the  pro- 
liferation of  the  adventitia  of  the  vessels.  We  thus  get  a  closed,  smooth, 
fibrous  sac,  more  or  less  pigmented,  containing  either  clear  fluid  or 
fluid  with  granules  of  hemosiderin,  crystals  and  amorphous  masses  of 
hematoidin.  As  a  secondary  result,  we  find  degeneration  and  atrophy 
of  the  neuraxones  belonging  to  the  affected  area. 

Encephalomalacia. — The  arteries  of  the  brain  come  largely  under 
the  category  of  "end"  arteries.  Consequently,  obstruction  of  their 
lumina,  if  at  all  marked,  is  followed  by  most  serious  results.  These 
consist  in  a  peculiar  form  of  infarct — necrosis  and  softening  (encephalo- 
malacia) — in  certain  vascular  districts,  accompanied  by  more  or  less  inter- 
ference of  function,  and  followed  often  by  death.  The  occlusion  of  the 
vessels  is  commonly  brought  about  by  thrombosis  or  embolism.  Arterio- 
sclerosis, in  the  form  of  proliferating  endarteritis,  may  also  cause  it,  and 
in  any  event  would  predispose  to  the  conditions  just  mentioned.  The 
causes  of  embolism  and  thrombosis  are  the  same  here  as  elsewhere. 
Emboli  are  usually  vegetations  from  diseased  valves  of  the  heart,  dis- 
lodged portions  of  intracardiac,  venous,  or  arterial  thrombi,  disintegrating 
tissue,  or  microorganisms.  Thrombi  are  found  in  certain  infectious 
diseases,  marasmus,  or  form  in  diseased  vessels  or  upon  emboli. 

Emboli  generally  reach  the  brain  by  the  most  direct  path.  Usually, 
it  is  the  Sylvian  artery  or  one  of  its  branches  that  is  involved,  less  com- 
monly, the  anterior  cerebral,  and  less  commonly  still,  the  posterior 
cerebral.  The  left  side  of  the  brain  is  affected  slightly  more  often  than 
the  right. 

Arteriosclerosis,  when  present,  is  most  marked  in  the  circle  of  Willis 
and  the  Sylvian  arteries.  In  advanced  cases,  all  the  arterioles  of  the 
cortex  may  be  involved  and  look  like  small,  white  threads  upon  the  pia. 
The  sclerosis  is  commonly  of  the  nodose  variety.  When  the  blood  supply 
of  the  brain  is  gradually  cut  off,  as  in  a  slowly  progressive  arteriosclerosis, 
we  get  simple  atrophy  of  the  brain  tissue,  with,  in  time,  a  tendency  to 
fibrosis.  This  is  well  seen  in  aged  people.  The  more  frequent  event  of 
sudden  anemia,  brought  about  by  embolism  or  thrombosis,  or  the  cir- 
culatory disturbances  in  the  neighborhood  of  inflammatory  foci,  produces 
local  softening  with  rapid  disintegration  of  the  brain  substance. 

The  older  anatomists  used  to  speak  of  three  kinds  of  cerebral  soften- 
ing— white,  red,  and  yellow.  It  should  be  remarked  that  these  terms 
refer  properly  not  to  distinct  pathological  processes,  but  rather  to  special 
peculiarities  or  different  stages  of  the  one  affection.  In  white  softening 
there  is  a  pure  anemic  necrosis,  the  tissues  being  absolutely  cut  off  from 
the  circulation.  In  red  softening,  hemorrhage  takes  place  into  the 
necrosed  area,  either  by  regurgitation  or  by  rupture  of  neighboring 
vessels.  Later,  when  fatty  degeneration  of  the  cells  occurs,  with  trans- 
formation of  the  blood  and  liberated  blood  pigment,  we  have  the  so-called 
yellow  softening. 

In  the  early  stages,  extensive  softening  of  the  cerebral  substance  may 


ENCEPHALITIS  555 

be  present  with  but  few  visible  signs.  The  affected  district  is  sometimes 
a  little  cedematous  or  turbid,  but  not  infrequently  it  does  not  differ 
materially  from  the  healthy  tissue.  On  palpation,  however,  the  part  is 
found  to  be  softer  than  normal  and  pulpy.  The  other  regions  of  the 
brain  often  show  slight  hyperemia.  In  more  advanced  cases,  the 
softened  area  may  be  of  a  reddish  or  yellowish  color,  as  above  mentioned, 
and  is  of  a  pulpy,  semiliquid  consistence.  In  some  cases  there  may  be 
multiple  small  foci  of  softening  in  close  juxtaposition,  giving  the  part 
a  cribriform  appearance  (etat  crible).  In  other  cases  the  destruction  of 
tissue  is  complete,  leading  to  the  formation  of  cystic  cavities  containing 
fluid,  fatty  granules,  and  detritus  (one  form  of  porencephaly).  Not 
infrequently,  however,  the  cavity  resembles  a  sponge,  being  traversed 
by  small  bloodvessels  and  fine  strands  of  glial  tissue. 

Microscopically,  in  the  degenerated  area  we  see  what  has  been  termed 
"varicose"  atrophy  of  the  protoplasmic  processes  of  the  specific  nerve 
cells,  and  chromatophilic  changes  in  the  ganglia,  while  the  neuroglia  is 
disintegrated,  presenting  numerous  pigmented  cells,  droplets  of  fat  and 
myelin,  leukocytes,  and  corpora  amylacea. 

In  course  of  time,  the  detritus  is,  to  some  extent,  absorbed,  the  fluid 
thereby  becoming  thinner  and  clearer,  and  there  is  an  attempt  at  cicatri- 
zation. In  young  persons  and  those  with  healthy  vessels,  provided  that 
the  lesion  is  small,  there  is  a  slight  amount  of  proliferation  of  the  glia 
about  the  softened  area,  leading  to  induration.  In  many  cases,  however, 
even  this  is  wanting,  and  the  cavity  is  surrounded  by  a  more  or  less  necrotic 
zone.  When  the  cyst  lies  near  the  surface  of  the  brain,  the  superficial 
boundary  collapses,  leaving  a  depression  in  communication  with  the 
subarachnoid  space,  which  is  filled  with  fluid  (another  form  of  poren- 
cephaly). The  collapsed  tissue  is  more  opaque  than  normal,  whitish,  or 
pigmented. 

The  clinical  symptoms  produced  by  encephalomalacia  depend  upon 
the  localization  and  extent  of  the  lesion.  In  slowly  developing  cases  of 
wide  extent,  there  is  apt  to  be  gradual  degradation  of  the  intellect, 
amounting  even  to  dementia.  In  the  sudden  local  lesions  we  get  various 
motor  or  sensory  phenomena.  A  lesion  in  the  internal  capsule  will 
lead  to  hemiplegia  on  the  opposite  side;  one  in  the  cortical  motor  area 
causes  motor  paralysis  of  the  corresponding  muscles.  When  the  third 
left  frontal  convolution  is  involved  motor  aphasia  results.  Lesions  in 
the  posterior  cerebral  lobe  and  posterior  part  of  the  vertex  cause  inter- 
ference with  vision.  It  should  be  remembered  that  encephalomalacia 
accounts  for  a  certain  number  of  the  cerebral  birth  palsies. 


INFLAMMATIONS. 

Encephalitis. — Inflammation  of  the  brain  is  termed  encephalitis. 
Before  entering  on  the  discussion  of  this  subject  it  should  be  observed 
that  in  many  cases  it  is  difficult,  if  not  impossible,  to  be  sure  that  certain 
lesions  are  of  an  inflammatory  nature  or  not.  The  nervous  tissue  is 


556  THE  BRAIN 

the  most  delicate  and  highly  specialized  in  the  body,  consequently,  it 
is  comparatively  easily  put  out  of  gear,  and,  further,  its  reparative 
powers  are  slight.  Extensive  lesions  may  be  produced  by  trifling 
causes,  are  often  quickly  induced,  and  are  followed  by  far-reaching 
results.  Many  conditions,  such  as  embolic  infection,  abscess,  traumat- 
ism,  and  tuberculosis,  are  frankly  inflammatory  and  present  no  special 
difficulty.  There  are  others,  however,  which  are  somewhat  similar 
in  appearance,  due  to  intoxication  with  alcohol,  bacterial  or  mineral 
poisons,  to  sunstroke  and  concussion  of  the  brain,  that  are  not  quite 
so  clear.  The  most  obscure  of  all  are  those  chronic  conditions,  of  which 
disseminated  sclerosis,  system  degenerations,  Huntingdon's  chorea,  and 
general  paralysis  of  the  insane  may  be  cited  as  examples.  Some  of 
these  appear  to  be  dependent  on  circulating  toxins,  and  are,  therefore, 
possibly  inflammatory  or  degenerative,  while  others  are  more  probably 
manifestations  of  developmental  defects.  The  difficulty  arises  from 
the  fact  that  degeneration  and  disintegration  of  the  specific  nerve  ele- 
ments are  common  to  all  these  affections  and  are  followed  by  insidious 
regeneration  of  glial  elements.  It  is  obviously,  therefore,  impossible, 
in  the  present  state  of  our  knowledge,  to  make  a  satisfactory  classification. 
Any  that  we  adopt  must  be  based  mainly  on  clinical  grounds  and  on 
convenience. 

Several  forms  of  encephalitis  can  be  differentiated  according  to  their 
localization.  In  the  majority  of  cases  the  cortex  and  peripheral  portions 
of  the  cerebrum  and  cerebellum  are  involved,  less  frequently  the  basal 
ganglia.  In  many  instances  cortical  encephalitis  is  associated  with 
meningitis  (meningo-encephalitis).  When  the  gray  matter  is  chiefly 
involved  we  speak  of  poliencephalitis.  The  medulla  may  be  affected 
(bulbomyelitis),  or  both  brain  and  cord  (encephalomyelitis). 

Encephalitis  may  be  hematogenic,  traumatic,  or  the  result  of  the 
extension  of  inflammation  from  contiguous  parts,  as  the  meninges  and 
cranial  bones.  According  to  the  kind  of  exudation,  it  is  simple  or  sup- 
purative. 

Acute  Encephalitis. — Acute  encephalitis  of  hematogenic  origin  occurs 
as  a  complication  of  certain  infective  processes,  chief  among  which  are 
acute  endocarditis,  septicemia,  cerebrospinal  meningitis.  More  rarely, 
it  has  been  met  with  in  typhoid  fever,  acute  rheumatism,  scarlatina, 
influenza,  ulcerative  pulmonary  tuberculosis,  and  rabies.  The  condition 
may  be  due  to  the  specific  germ  producing  the  primary  disease,  but  is 
not  infrequently  brought  about  also  by  secondary  infection. 

The  lesions  are  most  commonly  met  with  in  the  cerebral  cortex,  but 
any  part  of  the  brain  may  be  involved.  In  the  more  strictly  localized 
form,  the  gray  matter  in  the  floor  of  the  third  ventricle  and  about  the 
aqueduct  of  Sylvius  is  often  involved  (superior  poliencephalitis),  or  that 
in  the  neighborhood  of  the  fourth  ventricle  (inferior  poliencephalitis). 
The  cerebellum  is  rarely  involved.  The  foci  of  inflammation  may  be 
single,  multiple,  or  evenly  scattered  throughout  the  brain. 

The  smaller  areas  of  inflammation  are  often  invisible  to  the  naked 
eye  and  may  only  be  discovered  accidentally  on  making  a  microscopic 


TRAUMATIC  ENCEPHALITIS  557 

examination.  Larger  ones  appear  as  tumid  patches  of  a  diffuse  reddish 
color  and  may  present  small  hemorrhages.  In  suppurative  cases,  the 
foci  are  yellowish-white  in  color,  with  soft  or  liquefied  centres. 

Microscopically,  the  vessels  are  congested,  surrounded  by  inflamma- 
tory leukocytes,  and  here  and  there  may  be  small  extravasations  of  blood. 
The  nerve  elements  are  softened  and  degenerated.  The  ganglion  cells  are 
also  degenerated,  and  may  disappear.  The  axis-cylinders  swell  up, 
become  nodose,  and  disintegrate,  while  the  myelin  sheaths  are  con- 
verted into  small  droplets.  The  glia  shows  also  retrogressive  changes. 

When  there  is  a  tendency  to  heal,  the  destroyed  cells  are  to  some 
extent  absorbed,  and  at  the  periphery  of  the  affected  area  there  is 
proliferation  of  the  glia.  Small  foci  may  heal  completely,  and  even 
larger  ones,  if  the  patient  survive,  with  the  formation  of  a  sclerosed 
area  or  a  fibrous  scar. 

Acute  Suppurative  Encephalitis. — Acute  suppurative  encephalitis  is 
due  to  the  action  of  various  pyogenic  microorganisms,  notably,  the 
staphylococcus,  streptococcus,  and  Pneumococcus,  and  is  met  with  in 
such  conditions  as  gangrene  of  the  lung,  infected  wounds,  and  ulcerating 
carcinomas.  The  condition  is  brought  about  by  infective  emboli  derived 
from  a  primary  focus,  and  is  really  a  manifestation  of  a  generalized 
septicemia.  The  lesions  are  usually  multiple,  and  are  found  in  the 
cerebrum  and  cerebellum,  less  commonly  in  the  basal  ganglia. 

The  process  begins  with  the  formation  in  the  brain  of  minute  ischemic 
infarcts,  with  softening  and  hemorrhagic  extravasation.  To  this  is 
quickly  added  suppuration.  Abscesses  are  thus  produced,  varying  in 
size  from  that  of  a  hemp  seed  or  pea  to  that  of  a  walnut  or  hen's  egg, 
containing  creamy,  yellowish-white  or  yellowish-green  pus.  In  excep- 
tional cases  a  large  part  of  one  hemisphere  may  be  excavated.  In  the 
acute  forms,  after  the  pus  is  evacuated,  the  wall  of  the  abscess  is  found 
to  be  covered  with  shaggy  detritus,  and  the  surrounding  tissues  are  mark- 
edly cedematous  and  present  multiple  minute  hemorrhages.  The 
abscess  may  extend  to  the  surface  and  induce  meningitis,  or  may  burst 
into  a  ventricle,  so  that  severe  and  widespread  inflammation  is  set  up. 

In  the  unlikely  event  of  the  patient  surviving,  it  is  possible  that  the 
smaller  abscesses  may  heal  with  the  production  of  scar  tissue.  Larger 
ones,  after  a  lengthy  period,  become  bounded  by  a  zone  of  condensation, 
composed  of  a  rather  dense,  fibrous  capsule,  lined  with  granulation 
tissue.  So  long  as  pus  is  being  produced  the  adjacent  brain  substance 
is  compressed  and  undergoes  degeneration,  although  in  time  the  process 
may  cease  to  extend  and  the  pus  to  some  extent  be  absorbed.  The 
encapsulation  of  the  abscess  does  not  entirely  obviate  the  possibility  of 
extension  of  the  inflammation  to  neighboring  parts.  Diffuse  meningitis 
or  pressure  on  some  important  centre  may  induce  a  fatal  termination. 
Abscesses  in  the  cerebellum,  by  pressure  on  the  veins  of  Galen  and  throm- 
bophlebitis, lead  to  chronic  hydrops  of  the  ventricles. 

Traumatic  Encephalitis. —  The  description  just  given  of  the  lesions 
found  in  acute  hematogenic  encephalitis  will  apply  fairly  well  to  the 
traumatic  form.  Here,  however,  the  process  is  infective  or  non-infective, 


558 


THE  BRAIN 


In  the  simple  form,  the  pathological  changes  are  local,  corresponding, 
as  a  rule,  to  the  point  of  injury,  and  the  resulting  inflammation  is  chiefly 
the  attempt  at  removal  of  the  destroyed  tissue  and  its  replacement.  In 
the  infective  variety,  the  process  may  extend  more  or  less  widely  into 
the  substance  of  the  brain  (abscess)  or  along  the  meninges  (meningitis). 
Encephalitis  per  Extensionem. — Encephalitis  per  extensionem  com- 
monly originates  in  meningitis.  As  a  matter  of  fact,  leptomeningitis, 
so-called,  is  practically  always  a  meningo-encephalitis.  Many  cases, 
35  per  cent.,  according  to  Collins,  are  the  result  of  suppurative  middle 
ear  disease,  which  has  led  to  necrosis  of  the  petrous  bone.  In  children, 
however,  meningo-encephalitis  may  be  due  to  an  otitis  without  bone 
disease,  owing  to  the  fact  that  certain  bony  sutures  remain  unossified 

FIG.  153 


Extensive  abscess  of  the  brain. 


(From  the  Pathological  Department  of  the  Montreal 
General  Hospital.) 


for  some  time,  thus  allowing  direct  communication  between  the  middle 
ear  and  the  cranial  cavity.  This  channel  of  infection  is  still  more 
effective  since  the  dura  frequently  sends  prolongations  downward  into 
the  fissures.  In  such  cases  the  temporal  lobe  and  the  cerebellum  are 
most  liable  to  be  involved.  In  advanced  and  severe  cases  abscesses 
of  considerable  size  may  be  produced. 

Still  other  cases  originate  in  infection  from  the  nasopharynx,  the  frontal 
and  ethmoidal  sinuses,  and  the  orbital  cavity.  Certain  traumatic 
cases  also  come  into  the  category  of  encephalitis  by  extension.  In  many 
instances  extensive  subdural  collections  of  pus  are  found,,  together  with 
meningitis  and  sinus  thrombosis, 


SYPHILIS  559 

Tuberculosis. — Tuberculosis  of  the  brain  occurs  in  the  form  of  mul- 
tiple scattered  granulomas  of  small  size  (milia),  and  as  the  large  solitary 
tubercle.  The  infection  may  be  hematogenic,  due  to  bacilli  that  have 
entered  the  circulation  from  some  distant  focus,  usually  the  peribronchial, 
mesenteric,  or  retroperitoneal  lymph-nodes.  In  many  cases,  but  not  all, 
the  condition  is  part  and  parcel  of  a  generalized  miliary  tuberculosis.  As 
a  rule,  the  meninges  are  involved  as  well — tuberculous  meningo-encepha- 
litis  (see  p.  545).  In  another  class  the  infection  is  lymphogenic,  origi- 
nating in  the  petrous  bone  or  the  nasopharynx. 

In  the  hematogenic  type,  the  tubercles  are  distributed  along  the 
smaller  arterioles  and  arterial  capillaries,  usually  in  the  cortex,  but  to 
some  extent  in  the  central  ganglia  also.  The  process  begins  with  the 
formation  of  minute  foci  of  cellular  infiltration,  often  with  hemorrhage. 
These  enlarge,  undergo  necrosis,  and  then  appear  as  small  grayish  or 
yellowish-white  nodules  with  caseous  centres,  frequently  surrounded  by 
a  hemorrhagic  zone. 

When  but  a  small  area  of  the  brain  is  implicated  and  the  patient  lives 
for  some  time,  larger  solitary  tubercles  are  produced,  varying  in  size 
from  that  of  a  pea  to  that  of  a  walnut  or  larger.  They  form  well-defined 
globular  or  nodular  masses,  of  firm  consistency,  resembling  tumors. 
This  is,  perhaps,  the  commonest  form  of  cerebral  tumor  found  in  young 
persons.  On  section,  they  are  composed  of  a  large  central  necrotic  area, 
bounded  by  a  zone  of  granulation  tissue,  in  which  can  frequently  be 
recognized  small  subsidiary  tubercles.  Occasionally,  the  caseous 
matter  softens  or  liquefies,  so  that  an  abscess-like  lesion  is  produced. 
Tuberculomas  are  found  most  commonly  in  the  cerebellum,  next  in  the 
cerebrum,  more  rarely  in  the  basal  ganglia,  pons,  and  medulla. 

Syphilis. — The  syphilitic  manifestations  in  the  brain  assume  three 
types,  meningo-encephalitis  (see  p.  547),  endarteritis,  and  the  gumma. 
Syphilitic  endarteritis  leads  often  to  obstruction  of  the  vessels  and 
encephalomalacia,  and  possibly,  also,  to  local  atrophies,  focal  and  system 
scleroses. 

Gummas  take  the  form  of  grayish  or  grayish-red,  somewhat  translu- 
cent nodules,  of  irregular  shape.  The  central  portion  commonly  shows 
degeneration  and  necrosis.  When  healing  tends  to  take  place,  dense 
fibrous  bands  delimit  and  gradually  substitute  the  mass.  Gummas 
commonly  develop  in  the  meninges  and  extend  to  the  brain.  They  may, 
however,  develop  in  any  part  of  the  brain,  but  have  a  preference  for  the 
gray  matter.  The  membranes  overlying  the  gumma  usually  become 
adherent. 

Uhle  and  Mackinney1  report  finding  the  spirocheta  pallida  in  a  gumma 
of  the  brain. 

Actinomycosis. — Primary  actinomycosis  of  the  brain  is  excessively 
rare.  One  case  has  been  recorded  by  Bollinger  in  a  woman,  in  whom 
there  was  a  granuloma  the  size  of  a  hazelnut  in  the  third  ventricle,  without 
evidence  of  actinomycosis  elsewhere.  More  commonly,  the  condition 

1  Proc.  Path.  Soc.  of  Phila.  (N,  S),  9:  1906:  195, 


560  THE  BRAIN 

arises  by  metastasis,  or  the  extension  of  actinomycosis  of  the  face,  neck, 
or  throat.  In  the  metastatic  form,  which  is  usually  only  one  phase  of 
a  general  infection,  multiple  gelatinous-looking  or  necrotic  foci  are  pro- 
duced. In  the  cases  due  to  extension,  the  actinomyces  reach  the  cranial 
cavity  through  the  various  foramina  and  fissures. 

As  a  rule,  the  meninges  are  involved  in  a  more  or  less  diffused  suppura- 
tive  inflammation,  with  the  formation  of  numerous  small  necrotic  foci 
that  tend  to  invade  the  brain  substance  and  the  various  sinuses.  In  the 
more  chronic  cases  adhesion  may  take  place  between  the  membranes. 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  brain  is  seen  particularly  well  in  old  age. 
The  brain,  as  a  whole,  is  diminished  in  size,  as  is  proved  by  the  weight 
being  below  the  normal,  but  the  wasting  is  relatively  most  marked  in  the 
frontal  and  vertical  regions.  The  convolutions  are  small  and  the  sulci 
wide.  In  cases  of  moderate  extent,  when  the  brain  is  sectioned,  little 
alteration  may  be  noticed  save  that  the  gray  matter  of  the  cortex  is  some- 
what thinned.  In  the  more  advanced  cases,  however,  besides  this,  the 
perivascular  spaces  are  enlarged,  so  that  the  vessels  lie  in  wide  channels, 
and  small  foci  of  degeneration  are  to  be  seen  (etat  crible).  It  is  not 
uncommon  as  a  concomitant  of  the  loss  of  substance  to  find  enlargement 
of  the  subarachnoid  space  and  of  the  ventricles,  which  are  filled  with 
fluid  (hydrops  meningeus  ex  vacuo;  h.  ventriculorum  ex  vacua).  The 
cerebellum,  as  a  rule,  is  not  involved  to  any  extent,  but  occasionally 
has  been  found  to  be  wasted. 

Microscopically,  the  changes  are  referable  to  atrophy  of  the  specific 
nerve  cells,  ganglia,  and  medullated  nerve-fibers  alike.  There  is  granular 
disintegration  with  chromatolysis,  and  often  pigmentation  of  the  cells. 

Senile  atrophy  may,  with  considerable  certainty,  be  attributed  to 
several  causes,  chief  among  which  are  the  normal  tendency  to  retrogres- 
sion of  all  tissues  as  advanced  life  is  approached ;  impoverished  nutrition 
of  the  body  as  a  whole;  and  the  local  effects  of  imperfect  blood  supply, 
owing  to  sclerosis  of  the  vessels.  In  accordance  with  the  general  rule  that 
the  more  highly  differentiated  structures  are  the  most  liable  to  disease  and 
degeneration,  it  is  that  portion  of  the  brain  containing  the  intellectual 
centres  which  chiefly  suffers. 

Other  causes  of  atrophy  are  prolonged  wasting  diseases,  alcoholism, 
and  chronic  lead-poisoning. 

Fatty  degeneration  of  the  specific  nerve-cells,  fragmentation  of  the  myelin 
sheaths,  disintegration  of  the  fibers,  necrosis,  and  liquefaction  are  frequently 
met  with  in  connection  with  injuries,  inflammation,  and  circulatory 
disturbances.  They  need  not  be  more  specially  dealt  with  here. 

We  come  now  to  discuss  certain  affections  of  the  brain,  which  are  dealt 
with  here  largely  as  a  matter  of  convenience,  since  their  etiology  is 
obscure.  In  these  cases  the  most  striking  feature  is  atrophy  of  the 
brain  substance.  The  difficulty  lies  in  determining  whether  this  atrophy 


GENERAL  PARALYSIS  OF  THE  INSANE  561 

is  a  primary  degeneration  or  results  from  preexisting  inflammation. 
The  fact  that  some  of  the  cases  occur  in  young  persons,  and  in  several 
members  of  the  same  family,  suggests  that  inherited  peculiarities  or 
anomalies  of  development  of  the  specific  nerve  elements  may  play  a 
contributory,  if  not  a  leading,  role.  This  need  not  imply  that  there  is 
failure  to  reach  anatomical  perfection,  but  merely  that  there  is  a  deficiency 
of  vegetative  force,  so  that  the  structures  are  after  a  time  unable  to 
meet  the  demands  made  upon  them,  or  become  unduly  susceptible  to 
deleterious  influences.  Whether  we  are  to  regard  this  condition  of 
things  as  a  disease  in  itself  or  as  merely  predisposing  to  other  morbid 
processes  must  at  present  be  left  open.  The  most  important  affections 
coming  under  this  category  are  general  paresis  of  the  insane,  Hunting- 
don's chorea,  and  disseminated  sclerosis. 

General  Paralysis  of  the  Insane  (Progressive  General  Paralysis ; 
Dementia  Paralytica). — General  paralysis  of  the  insane  is  a  chronic 
affection  of  the  nervous  system,  both  central  and  peripheral,  character- 
ized clinically  by  a  well-defined  train  of  symptoms,  chief  among  which 
may  be  mentioned,  mental  degradation  and  other  disturbances  of  the 
intellect,  delusions  of  a  peculiar  kind  (delire  de  grandeur),  muscular 
tremors  and  weakness,  sometimes  paralyses,  disturbance  of  speech, 
and  Argyll-Robertson  pupil,  with  often  special  features  referable  to 
changes  in  the  spinal  cord.  The  disease  is  usually  met  with  in  those 
who  have  led  the  strenuous  life,  or  who  have  been  subjected  to  great 
mental  stress  and  worry,  especially  if  they  have  been  alcoholics  or  have 
had  syphilis.  It  is  often  met  with  in  tabes  dorsalis,  inveterate  epilepsy, 
and  after  trauma.  Men  are  most  often  affected,  especially  those  who 
are  intellectually  active.  The  disease  is  most  common  between  the 
ages  of  thirty  and  fifty  years.  According  to  Eddinger,  dementia  para- 
lytica  is  an  exhaustive  disease  comparable  to  tabes  dorsalis,  in  which, 
owing  to  mental  strain,  shock,  or  worry,  the  cerebrum  is  primarily  and 
most  severely  affected.  As  bearing  on  the  etiology  of  the  condition  it 
may  be  remarked  that  Wassermann's  precipitation  test  is  positive  in 
this  disease  as  it  is  in  syphilis. 

The  lesions  found  are  somewhat  striking.  The  dura  is  often  adherent 
to  the  calvarium,  and  there  may  be  an  internal  hemorrhagic  pachymenin- 
gitis.  The  pia  is  turbid  and  thickened,  especially  in  the  frontal  region, 
and  is  often  firmly  attached  to  the  surface  of  the  brain,  so  that  on  attempt- 
ing to  remove  it  portions  of  the  substance  are  torn  away.  The  spinal 
pia  is  sometimes  also  adherent  posteriorly,  and  there  may  be  sclerotic 
changes  in  the  posterior  or  lateral  columns,  or  both.  The  cerebral  con- 
volutions are  less  distinct  than  normal,  the  brain  substance  is  sclerosed, 
and  the  cortical  gray  matter  is  diminished.  The  frontal  and  parietal 
lobes  are  most  markedly  affected.  Owing  to  dilatation  of  the  vessels 
and  enlargement  of  the  perivascular  lymph-spaces,  the  brain  often  pre- 
sents a  spongy  or  cribriform  appearance.  The  ventricles  are  usually 
dilated,  and  the  choroid  plexus  and  ependyma  are  granular. 

Microscopically,  the  pial  vessels  are  surrounded  by  collections  of  small 
round  cells,  plasma  cells,  and,  occasionally,  "mast"  cells,  and  there  is 
36 


562  THE  BRAIN 

proliferation  of  the  connective-tissue  trabeculae  penetrating  the  brain. 
The  specific  nerve-cells  are  swollen,  cloudy,  or  granular,  often  vacuolated, 
atrophic,  and  pigmented.  The  neuraxones  are  altered  in  size  and 
shape  and  the  dendrites  are  wasted.  The  myelin  sheaths  are  absent 
wholly  or  in  part.  The  nuclei  stain  badly.  The  glia  is  greatly  increased 
and  the  vessel  walls  are  thickened.  To  the  lesions  in  the  brain  are  often 
added  those  of  tabes  dorsalis  or  combined  sclerosis.  The  granulation 
of  the  ependyma  is  due  to  the  subepithelial  proliferation  of  the  glia. 

Disseminated  Sclerosis  (Multiple  or  Insular  Sclerosis;  Sclerose 
en  Plaques). — The  lesions  in  this  disease  are  found  scattered  irreg- 
ularly throughout  the  central  nervous  system.  At  one  time  the  cord  is 
chiefly  affected ;  at  another,  the  cerebrum.  Occasionally,  single  foci  are 
found  in  the  brain  which  are  probably  of  the  same  nature.  The  lesions 
consist,  as  the  name  implies,  in  the  formation  of  islets  of  sclerosis, 
varying  in  size  from  that  of  a  pin-head  to  patches  two  or  three  inches 
across.  The  larger  lesions  are  apt  to  be  found  in  the  neighborhood  of 
the  ventricles  and  in  the  pons  and  medulla.  When  the  foci  are  situated 
in  the  cortex  or  have  extended  through  to  the  surface  of  the  brain,  slightly 
elevated  nodules  are  to  be  seen,  to  which  the  pia  may  or  may  not  be 
adherent.  On  section,  the  patches  are  rounded  or  irregular  in  shape, 
of  a  grayish-white  or  reddish-gray  color,  distinctly  firmer  than  the  normal 
brain  substance.  They  can  often  be  localized  better  by  the  sense  of 
touch  than  by  their  appearance.  When  marked  degeneration  is  going 
on,  the  areas  appear  somewhat  variegated.  In  the  cord,  the  cervical  and 
lumbar  enlargements  are  most  likely  to  be  involved. 

Microscopically,  the  sclerotic  areas  are  found  to  consist  in  the  main 
of  proliferated  glia  cells  and  connective  tissue,  in  which  can  be  seen 
remnants  of  degenerated  nerve-cells  and  fibers.  The  axones  show 
loss  of  their  medullary  sheaths,  or  may  be  entirely  disintegrated,  often 
leaving  only  a  space  to  show  where  they  were  formerly  present.  At 
the  periphery,  these  changes  are  most  marked,  and  there  may  be  found 
drops  of  myelin  and  fat,  granule  cells,  and  amyloid  bodies.  The  vas- 
cular changes  are  not  constant,  but  there  are  usually  thickening  and 
hyaline  degeneration  of  the  vessel  walls,  with  distension  of  the  peri  vascu- 
lar lymphatics.  Ribbert  has  described  a  new  formation  of  vessels. 

The  causes  of  the  disease  are  quite  obscure.  Some  cases  seem  to  be 
dependent  on  previous  infection  or  toxic  influences,  and  are  probably 
due  to  a  primary  degeneration  of  the  specific  nervous  elements,  together 
with  an  attempt  at  repair  on  the  part  of  the  glial  cells. 

In  other  cases  where  the  disease  begins  in  early  life,  or  there  is  a  dis- 
tinctly familial  taint,  it  is  probably  due  to  defective  embryonal  develop- 
ment. The  symptoms  vary  according  to  the  distribution  of  the  lesions. 
There  is  usually  some  impairment  of  emotional  control  with  attacks  of 
giddiness.  Later,  we  get  spasticity  of  the  lower  extremities,  nystagmus, 
scanning  speech,  extension  tremor,  and  partial  optic  atrophy. 

Huntingdon's  Chorea  (Chronic  Degenerative  Chorea).— Hunting- 
don's chorea  is  a  curious  and  rare  affection,  characterized  by  irregular 
movements,  disturbance  of  speech,  and  dementia.  It  usually  occurs 


TUMORS  563 

late  in  life,  and  is  notably  a  family  and  local  disease.  This  feature, 
together  with  the  fact  that  the  convolutions  of  the  brain  frequently  show 
morphological  deviations  from  the  normal,  has  led  most  authorities  to 
refer  the  affection  to  defective  development.  As  the  disease  does  not 
usually  come  on  until  youth  is  past  it  would  seem  as  if  the  "exhaustion 
theory"  would  apply  here. 

The  brain  is  found  to  be  atrophied,  especially  over  the  frontal  and 
central  regions,  the  meninges  show  patchy  thickening  and  the  vessels 
are  sclerosed.  Rarely,  internal  hemorrhagic  pachymeningitis  has  been 
observed  also.  On  section,  the  cervical  gray  matter  is  diminished. 

Microscopically,  there  is  atrophy  and  degeneration  of  the  ganglion 
cells,  especially  in  the  large  and  small  pyramidal  layers.  The  cell 
bodies  are  granular,  the  nuclei  enlarged  and  prominent,  or,  again,  may 
stain  badly.  The  processes  are  wasted.  The  vessels  show  slight  nodular 
sclerosis,  the  perivascular  lymph-spaces  are  dilated,  and  in  their  neighbor- 
hood there  is  some  round-celled  infiltration. 


PROGRESSIVE  METAMORPHOSES. 

The  reparative  powers  of  the  specific  nerve  elements  are  strictly 
limited.  In  children  and  young  people,  in  whom  vegetative  functions 
are  in  excess,  we  sometimes  see  in  the  neighborhood  of  destructive  lesions 
an  attempt  at  regeneration  of  the  nerve-fibers,  but  it  is  of  the  slightest 
description,  and  authorities  are  not  agreed  on  the  interpretation  of  the 
appearances.  Proliferation  of  the  neuroglia  is  much  more  common, 
and  is  met  with  chiefly  about  old,  inflammatory  lesions.  The  so-called 
hypertrophy  of  the  brain,  in  which  the  organ  is  considerably  enlarged  in 
all  directions,  is  due  to  what  may  be  called  a  diffuse  gliomatosis,  prin- 
cipally of  the  white  matter,  and  is  more  properly  included  under  the 
developmental  anomalies  (see  p.  530). 

Tumors. — The  most  important  of  the  primary  new-growths  of  the 
brain  are  the  glioma  and  the  sarcoma.  Angioma,  fibroma,  lipoma,  osteoma, 
cholesteatoma,  and  dermoid  cysts  have  been  described,  but  are  much  more 
rare. 

Glioma. — The  most  frequent  and  characteristic  tumor  is  the  glioma, 
which  is  found  in  all  parts  of  the  central  nervous  system  and  its  deriva- 
tives, as  the  retina.  It  is  most  frequently  met  with  in  childhood  and 
adolescence,  but  is  not  uncommon  after  adult  life.  The  usual  situation 
is  in  the  hemispheres  just  beneath  the  pia,  but  also,  occasionally,  in 
the  corpus  callosum,  pons,  medulla,  and  cerebellum.  On  removal 
of  the  calvarium,  its  presence  may  be  at  first  unsuspected,  for  it  is  rare 
for  it  to  be  visible  externally.  At  most,  there  may  be  slight  fulness  with, 
possibly,  discoloration  of  the  affected  part.  On  section,  the  growth  is 
indefinitely  circumscribed,  merging  imperceptibly  into  the  normal 
brain  substance  or  separated  from  it  merely  by  a  zone  of  softening 
and  degeneration.  In  consistence  it  is  slightly  softer  or  firmer  than 
healthy  brain  substance  according  to  circumstances.  In  appearance  it 


564  THE  BRAIN 

is  grayish,  grayish-white,  or  reddish,  somewhat  translucent,  and  often 
streaked  with  reddish-yellow  or  opaque,  white  bands  and  patches. 
The  tumor  is  vascular,  and  it  is  not  uncommon  for  hemorrhage  to  take 
place  into  its  substance.  This  may  be  so  extensive  as  to  mask  the 
true  nature  of  the  growth  and  give  it  the  appearance  of  an  apoplectic 
focus.  Owing  to  hemorrhage  or  colliquative  necrosis  the  tumor  may  be 
converted  into  a  series  of  cysts  containing  turbid-whitish  or  brownish 
fluid.  In  some  instances  it  is  extremely  difficult  to  distinguish  the 
mass  from  the  brain  substance,  save  that  it  is  a  little  redder  or  paler 
than  the  normal  structures.  It  is  probable  that  some,  at  least,  of  the 
cases  of  hypertrophy  of  the  brain  described  by  the  older  writers  were 
more  properly  gliomas.  This  tumor  does  not  involve  the  membranes 
or  the  bone. 

As  its  name  implies,  the  glioma  originates  in  the  proliferation  of  the 
glial  cells.  Microscopically,  we  have  a  more  or  less  cellular-looking 
growth,  consisting  of  glial  cells  of  the  ordinary  type  which  possess  long 
branching  and  interlacing  processes.  The  cell  bodies  vary  considerably 
in  size  and  contain  large,  deeply-staining  nuclei.  The  cells  themselves 
are  evenly  distributed  or  aggregated  into  groups.  The  fibrils  form  a 
sort  of  meshwork,  which  at  times  is  dense  and  at  others  rather  loose, 
containing  spaces  which  give  the  tumor  somewhat  the  appearance  of 
a  myxoma  (myxogliomd).  The  structure  is  in  some  cases  not  unlike 
a  round-celled  sarcoma,  but  its  true  nature  can  often  be  determined 
by  teasing  out  a  bit  of  the  fresh  tumor  and  discovering  the  branching 
cells.  The  use  of  a  special  stain  for  glia,  such  as  Weigert's,  gives  much 
assistance.  The  bloodvessels  and  sinuses  are  often  numerous  and 
may  be  so  large  and  abundant  as  to  warrant  the  designation  of  glioma 
telangiectaticum.  Hemorrhages  are  frequent.  The  walls  of  the 
bloodvessels  often  present  hyaline  degeneration  and  the  adventitia  may 
proliferate,  so  that,  the  vessels  are  surrounded  by  a  zone  of  cellular  or 
fibrocellular  appearance. 

In  certain  cases,  a  glioma  of  the  structure  just  described  contains 
cells,  sometimes  possessing  more  than  one  nucleus,  which  resemble 
closely  the  ganglion  cells  of  the  brain  and  cord.  These  are  evenly 
scattered  throughout  the  section  or  are  massed  into  groups.  Isolated 
medullated  nerve  fibers  can  also  be  made  out.  To  this  variety  Ziegler 
gives  the  name  of  neuroglioma  ganglionare.  Many  authorities,  however, 
do  not  admit  that  in  these  cases  there  is  a  true  new-formation  of  ganglion 
cells,  or,  more  accurately,  regard  them  as  an  overgrowth  of  congenital 
origin  developing  from  a  "  cell-rest." 

At  the  periphery  of  a  glioma  the  brain  structure  presents  various 
grades  of  degeneration  and  necrosis,  although  it  is  remarkable  how  well 
it  may  be  preserved  in  some  cases.  Not  infrequently,  the  glioma  sends 
out  prolongations  which  gradually  infiltrate  and  surround  portions  of 
the  brain  substance. 

When  a  glioma  is  rapidly  growing  and  so  cellular  that  it  resembles  a 
sarcoma,  it  has  been  customary  to  speak  of  it  as  a  gliosarcoma.  It  should 
be  remarked,  however,  in  this  connection,  that,  inasmuch  as  neuroglia 


GLIOMA  565 

is  epiblastic  in  origin,  it  is  a  mistake  to  regard  tumors  developing  from 
it  as  sarcoma  to  us,  that  is  to  say,  mesoblastic.  A  true  gliosarcoma,  if 
such  a  thing  exist,  would  be  a  mixed  tumor,  consisting  in  part  of  glioma 
and  in  part  of  sarcoma  derived  from  the  atypical  proliferation  of  the 
connective  tissue  forming  the  adventitia  of  the  vessels.  It  is,  therefore, 
better  to  speak,  with  Ziegler,  of  the  sarcomatoid  or  cellular  glioma  as 
a  "medullary  glioma." 

The  etiology  of  glioma  formation  is  somewhat  obscure.  The  occur- 
rence of  the  growth  in  early  life,  the  peculiar  localization,  and  the 
occasional  presence  of  ganglion  cells  have  suggested  that  errors  of  develop- 
ment, such  as  embryonic  "cell-rests,"  may  be  at  work.  This  view  is 
supported  by  the  observation  of  Stroebe,1  who  found  in  a  glioma  cystic 
cavities  lined  with  cylindrical  epithelium.  The  more  delicate  methods 

FIG.  154 


/,-*/'**       **   ''V1-    ^    /*        ^''li* 

r*    4  '  *<   .»    •>•*•••  *  * 

/  *  .«*••*  *    »  *  *»* 

*?•* 


:^-;-rv: 

.  -  *  - .,  •  -** 


Y«L  ?"••    * 


Medullary  glioma  of  the  cerebral  vertex.     Winckel  obj.  No.  6,  without  ocular.     (From 
the  collection  of  Dr   A.  G.  Nicholls.) 

of  staining  devised  by  Weigert,  and  modified  by  Mallory  and  Beneke, 
have  shown  that  in  man  the  glia  cells  possess  protoplasmic  processes  only 
during  the  embryonic  stage,  while  the  adult  glia  is  composed  mainly 
of  fibers  in  which  are  cells  devoid  of  processes.  Now  the  glia  cells,  or 
astrocytes,  as  they  have  been  called,  are  derived  ultimately  from  the 
ependyma,  either  directly  or  indirectly  through  less  highly  differentiated 
cells  (astroblasts).  Apparently  gliomas  exist  which  may  be  compared 
with  glia  cells  in  various  stages,  both  embryonic  and  adult.  Flexner2 

1  Enstehung  d.  Hirngliome,  Centralbl.  f.  allg.  Path.,  v.  855. 

2  Glia  and  Gliomatosis.     Jour,  of  Nervous  and  Mental  Disease,  May,  1898. 


566  THE  BRAIN 

has  described  a  glioma  composed  of  ependyma-like  cells.  The  cellular 
or  sarcomatoid  gliomas  (medullary  gliomas  of  Ziegler)  possibly  originate 
in  the  intermediate  cells  (astroblasts),  which  might  readily  persist  in 
a  latent  form  into  adult  life.  The  gliomas  composed  of  branching  and 
"spider"  cells  suggest  a  derivation  from  the  primitive  astrocytes,  while 
the  denser,  more  fibrous  growths  resemble  the  adult  glia. 

Sarcoma. — Sarcoma  of  the  brain  originates  in  the  connective  tissue 
forming  the  intracerebral  prolongations  of  the  pia  or  adventitia  of  the 
vessels.  It  takes  the  form  of  single  or  multiple  growths  of  irregularly 
globular  shape,  which  are  fairly  well  defined  from  the  brain  substance 
or  even  encapsulated.  In  many  cases,  owing  to  the  degeneration  of 
the  structures  at  the  periphery  of  the  tumor,  it  can  be  shelled  out. 
The  favorite  site  is  in  the  cortex,  where  it  forms  a  projecting  nodular 
growth.  On  section  the  mass  is  of  a  grayish  or  yellowish  appearance  and 
presents  numerous  hemorrhages  and  foci  of  degeneration.  Micro- 
scopically, the  most  common  variety  is  the  round  or  mixed-celled  sar- 
coma, but  spindle-celled  forms  are  also  found.  In  some  cases  the 
sarcoma  is  highly  vascular,  and  seems  to  originate  from  the  adventitia  of 
the  vessels.  The  tumor  then  consists  of  a  series  of  whorls  of  spindle 
cells  arranged  radially  and  apparently  continuous  with  the  vessel  walls 
(angiosarcoma;  perithelioma).  We  have  seen  tumors  of  this  type  both  in 
the  cortex  and  in  the  corpus  callosum. 

Occasionally,  there  is  a  deposit  of  calcareous  matter  in  the  growth 
(psammosarcoma).  The  pia  over  the  sarcoma  is  inflamed  or  infiltrated, 
while  the  adjacent  brain  substance  is  softened  and  degenerated.  The 
ventricles  may  be  dilated.  Secondary  nodules  may  be  found  in  various 
parts  of  the  central  nervous  system. 

Angioma. — Of  the  primary  benign  tumors,  the  so-called  angioma  is 
not  infrequent.  This  presents  the  appearance  not  of  a  tumor,  but  of  a 
diffusely  reddened  patch,  not  unlike  a  congested  area  of  inflammation. 
Microscopically,  it  consists  mainly  of  dilated  bloodvessels  and  sinuses, 
about  which  the  brain  substance  is  softened  and  degenerated.  According 
to  Virchow,  angiomas  are  congenital,  and  are  probably  to  be  classed 
with  the  vascular  nevi. 

The  fibroma  is  a  rare  tumor,  taking  the  form  of  rounded  nodules. 

An  osteoma  of  the  corpus  striatum  has  been  recorded  by  Bidder.1 

Lipomas  are  rare. 

The  peculiar  tumor,  consisting  of  flattened  epithelial  cells  arranged  in 
laminae,  called  cholesteatoma,  has  been  met  with  in  the  brain,  although 
more  common  in  the  meninges. 

Dermoid  cysts  are  decidedly  rare.  A  few  examples  have  been  observed 
in  the  cerebellum. 

The  secondary  tumors  are  sarcomas  and  carcinomas.  They  arise  by 
direct  extension  from  the  meninges,  choroid  plexus,  cranial  bones,  tym- 
panum, and  orbit,  and  also  by  metastasis. 

1  Virch.  Archiv,  88:1882. 


CONTUSIONS  AND  LACERATIONS  567 

Parasites. — The  Eckinococcus  and  Cysticercus  celluloses  have  been 
met  with.  The  resulting  cysts  are  single  and  multiple.  They  are 
situated  usually  in  the  membranes,  but  may  be  found  in  the  brain 
substance. 

TRAUMATIC  DISTURBANCES. 

Injuries  to  the  brain  are  produced  in  a  great  variety  of  ways.  Ordi- 
narily, they  are  caused  by  great  external  violence,  for  the  brain  is 
particularly  well  protected,  not  only  by  the  bony  cranium,  but  by  its 
several  membranes,  and,  moreover,  is  surrounded  by  fluid.  The  most 
important  injuries  are  concussion,  compression,  contusion,  and  laceration. 
The  results  are  often  serious,  and  depend  upon  the  nature  and  extent 
of  the  trauma  and  its  localization. 

Concussion. — Concussion  of  the  brain  is  brought  about  by  falls  or 
blows  upon  the  head.  Partial  or  complete  loss  of  consciousness  results, 
with  muscular  relaxation.  The  condition  may  be  a  transient  one  or 
end  in  death.  The  exact  nature  of  the  lesion  is  somewhat  obscure. 
In  some  cases,  although  by  no  means  invariably,  multiple  small  hemor- 
rhages have  been  noted  in  the  brain  substance.  In  some  others,  certain 
of  the  ganglion  cells  have  been  calcified.  It  is  probable,  therefore,  that 
the  injury  leads  to  rupture  of  the  finer  capillaries,  solution  of  the  con- 
tinuity of  certain  nerve  paths,  and  degenerative  changes  in  the  fibers  and 
ganglia. 

Compression. — Compression  of  the  brain  is  usually  due  to  intra- 
cranial  growths,  hemorrhage,  or  excess  of  the  cerebrospinal  fluid.  Up 
to  a  certain  point  the  brain  is  able  to  accommodate  itself  to  the  abnormal 
condition  of  things,  largely  by  the  extrusion  of  a  corresponding  amount 
of  the  cerebrospinal  fluid.  It  is  often  surprising  what  an  amount  of 
pressure  the  brain  will  bear  without  the  production  of  structural  changes 
or  disordered  function.  Should,  however,  the  compression  exceed  the 
limit,  or  be  rapidly  brought  about,  we  get  flattening  of  the  gyri,  inter- 
ference with  the  free  circulation  of  blood  and  lymph,  resulting  in  marked 
cases  in  anemia,  softening  and  degeneration  of  the  brain  substance. 

Contusions  and  Lacerations. — Contusions  and  lacerations  of  various 
kinds  are  produced  by  fractures,  gunshot  wounds,  and  cutting  or 
stabbing  injuries.  Here  the  effects  are  mainly  local.  The  slighter 
injuries  lead  to  local  softening;  the  more  severe  present,  in  addition  to 
concussion,  signs  of  disintegration  of  the  brain  substance.  The  remoter 
results  depend,  of  course,  upon  the  extent  and  localization  of  the  injury. 
If  none  of  the  important  centres  are  involved,  provided  that  the  site  of 
the  injury  remain  aseptic  and  the  patient  survive,  the  lesion,  anatomic- 
ally speaking,  is  not  unlike  those  met  with  in  anemia  and  hemorrhage. 
The  tissue  actually  destroyed  is  gradually  disintegrated  and  absorbed, 
the  adjacent  nerve  fibrillae  and  ganglia  present  various  forms  of  degenera- 
tion, and  about  the  periphery  we  find  reactive  inflammation  with  pro- 
liferation of  connective  tissue,  which  is  most  noticeable  along  the  course 
of  the  vessels.  In  time  the  granulation  tissue  may  be  converted  into 


568  THE  BRAIN 

fibrous  connective  tissue.  In  the  case  of  trivial  injuries,  healing  may 
take  place  with  the  replacement  of  the  dead  material  by  a  scar,  com- 
posed mainly  of  fibrous  tissue  and  bloodvessels,  but  also  to  a  limited 
extent  of  newly-formed  glia.  In  the  more  extensive  injuries,  degenera- 
tion is  more  widespread  and  persistent. 

When  infection  of  the  wound  has  taken  place,  acute  inflammation 
is  set  up  (traumatic  encephalitis),  with,  some  times  the  formation  of  an 
abscess.  The  meninges  are  apt  to  be  involved  in  such  cases. 


CHAPTER    XXVII. 

THE  SPINAL  CORD. 

IN  order  to  obtain  a  correct  idea  of  the  spinal  cord  and  the  diseases 
to  which  it  is  subject,  it  is  well  to  bear  in  mind  certain  peculiarities  of 
structure  and  of  function.  Owing  to  its  position  within  a  bony  canal  on 
the  dorsal  aspect  of  the  body,  it  is  rather  liable  to  suffer  from  accidents 
and  injuries  of  various  kinds,  and  to  be  involved  in  pathological  processes 
originating  in  the  vertebral  column.  Its  vascularity  and  the  delicacy 
of  the  vessels  make  it  particularly  susceptible  to  circulatory  disturbances 
and  to  the  action  of  various  toxins.  It  is  curious  to  note,  in  this  con- 
nection, how  certain  parts  may  be  picked  out.  Thus,  in  poisoning  with 
lead  and  other  metallic  substances,  and  in  acute  poliomyelitis,  the  ganglion 
cells  of  the  anterior  horns  are  specially  involved.  In  chronic  ergotism 
and  in  locomotor  ataxia,  which,  in  a  large  proportion  of  cases  follows 
syphilis,  the  posterior  columns,  and  in  pernicious  anemia  the  posterior 
and  lateral  columns,  are  attacked.  The  explanation  of  this  is  not  clear. 
Finally,  the  cord  contains  bundles  of  nerve  fibers,  which  serve  to  con- 
duct impulses  from  the  cerebrum  and  cerebellum,  on  the  one  hand,  and, 
on  the  other,  from  the  peripheral  nerves  and  posterior  spinal  ganglia. 
In  this  way  lesions  of  certain  tracts  occur  which  are  to  be  regarded  as 
secondary  to  disease  of  some  distant  part.  Thus,  cerebral  apoplexy, 
cysts,  or  injuries,  involving  the  motor  cortex  of  the  brain,  are  in  time 
followed  by  degeneration  in  the  pyramidal  tracts  of  the  cord,  and  in 
multiple  peripheral  neuritis  lesions  may  be  induced  in  the  posterior 
columns. 

THE  SPINAL  MENINGES. 

Inasmuch  as,  anatomically  and  functionally,  the  spinal  membranes 
do  not  differ  materially  from  those  of  the  brain,  the  discussion  of  the 
lesions  affecting  them  will  be  somewhat  sketchy,  emphasis  being  laid 
only  on  such  points  as  are  of  special  interest. 

The  Spinal  Dura  Mater. 

The  dura  mater  spinalis  is  a  tough  connective-tissue  membrane 
continuous  with  that  covering  the  brain,  completely  enveloping  the 
cord,  and  separated  from  the  external  vertebral  column  by  what  is 
known  as  the  epidural  space. 

It  forms  a  loose  sheath  about  the  cord,  is  adherent  to  the  circumference 
of  the  foramen  magnum  and  to  the  posterior  common  ligament  in  the 


570  THE  SPINAL  DURA  MATER 

extreme  upper  cervical  region,  and  in  the  lower  end  of  the  spinal  canal. 
Below  the  level  of  the  third  piece  of  the  sacrum  it  becomes  impervious, 
but  continues  down  as  a  slender  thread  to  the  back  of  the  coccyx,  where 
it  blends  with  the  periosteum.  The  space  between  it  and  the  bone  is 
filled  with  loose,  areolar  tissue  and  a  plexus  of  veins.  On  each  side, 
opposite  the  intervertebral  foramina,  it  has  two  openings,  giving  exit 
to  the  sensory  and  motor  roots  respectively  of  the  corresponding  spinal 
nerve.  Prolongations  of  dura  surround  the  roots  until  it  is  lost  in  their 
sheaths. 

The  pia-arachnoid  of  the  cord  is  similar  to  that  of  the  brain.  The 
two  membranes  enclose  between  them  the  subarachnoid  space,  which 
contains  cerebrospinal  fluid.  The  outer  layer,  the  arachnoid,  is  not 
normally  adherent  to  the  dura,  except  perhaps  in  the  cervical  region. 
The  pia  covers  the  entire  surface  of  the  cord,  to  which  it  is  intimately 
adherent,  and  sends  a  process  down  into  its  anterior  median  fissure. 
It  also  invests  the  spinal  roots  in  their  exit  from  the  cord. 


CIRCULATORY  DISTURBANCES. 

Hemorrhage. — Hemorrhage  into  the  dura  of  the  cord  may  arise  from 
injury.  It  is  met  with  sometimes  in  infants  who  have  been  delivered 
by  instruments.  It  occurs  also  in  asphyxia  and  tetanus.  Minute 
petechial  extravasations  of  blood  are  found  in  cases  of  meningitis. 


INFLAMMATIONS. 

Pachymeningitis. — Acute  inflammation — pachymeningitis — is  most 
frequently  due  to  the  extension  of  disease  from  the  neighboring  parts, 
the  pia-arachnoid  and  vertebrae,  or  to  trauma.  The  dura  being  rather 
dense,  the  resulting  exudate,  which  may  be  cellular  or  fibrinous,  is  apt 
to  collect  on  either  the  outer  (pachymeningitis  externa)  or  inner  surface 
(pachymeningitis  interna)  of  the  membrane.  In  the  severer  forms, 
what  are  practically  abscesses  are  produced,  and  may  lead  to  com- 
pression and  destruction  of  the  cord  at  that  spot.  Where  healing  tends 
to  take  place,  organization  of  the  exudate  is  gradually  brought  about, 
with  the  formation  of  delicate  vascular  fibrous  adhesions  between  the 
dura  and  adjacent  structures. 

A  modified  form  of  internal  pachymeningitis  is  that  known  as 
pachymeningitis  interna  hemorrhagica,  analogous  to  the  disease  of  the 
same  name  occurring  in  the  cerebral  dura.  Its  etiology  is  rather 
obscure,  save  that  it  is  apt  to  be  found  resulting  from  syphilitic  or 
tuberculous  disease  of  the  vertebral  bones  or  pia.  In  the  severer  forms 
adhesions  are  liable  to  take  place  between  the  dura  and  pia-arachnoid 
which  result  in  degeneration  in  the  cord. 

Charcot  and  Joffroy  have  described  a  chronic  hypertrophic  cervical 
pachymeningitis,  which  leads  to  compression  and  degeneration  of  the 


PARASITES 


571 


nerve-roots  and  cord  in  the  cervical  region.  The  condition  eventually 
involves  the  pia-arachnoid  and  the  peripheral  zone  of  the  cord,  with 
resulting  fibrosis.  Complete  transverse  softening  of  the  cord,  from 
occlusion  of  the  vessels,  may  occur.  Most  cases  seem  to  be  due  to 
syphilis. 

Tuberculosis. — Tuberculosis  of  the  dura  is  almost  invariably  sec- 
ondary to  Pott's  disease  of  the  spine,  less  commonly  to  tuberculosis  of 
the  pia-arachnoid  and  cord.  In  the  earlier  cases,  scattered  granular 


FIG.  155 


Pachymeningitis  hypertrophiea  cervicalis  of  syphilitic  origin.     Secondary  degeneration  of 
the  cord  is  well  shown.     (From  the  collection  of  Dr.  Colin  K.  Russel.) 

tubercles  may  form  on  the  outer  surface  of  the  dura.  These  may 
coalesce,  so  that  large  caseating  masses  of  granulation  tissue  are 
produced,  which  lead  to  compression  and  degeneration  of  the  cord. 
Clinically,  then,  we  get  the  features  of  a  transverse  myelitis.  Delicate 
inflammatory  membranes  may  in  some  cases  be  found  on  the  inner 
surface  of  the  dura.  Or  tubercles  may  make  their  appearance  here, 
and  eventually  extensive  tuberculous  granulation. 

Syphilis. — Syphilitic  granulation  may  be  primary,  but  is  usually 
secondary  to  syphilis  of  the  pia-arachnoid,  less  often  of  the  bone.  It 
leads  to  dense  cicatricial  adhesion. 

Parasites. — The  Echinococcus  and  the  Cysticercus  celluloses  have  been 
found  both  in  the  epidural  and  subdural  spaces.  These  affections  are 
usually  secondary. 


572  'THE  SPINAL  DURA  MATER 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — The  primary  tumors  of  the  dura  spinalis  are  the  sarcoma, 
psammoma,  lipoma,  fibroma,  myxoma,  and  chondroma.  They  are  all 
rare. 

Carcinoma  is  met  with  in  metastasis  from  carcinoma  elsewhere, 
usually  in  the  mamma. 

The  Spinal  Pia-arachnoid. 

CIRCULATORY  DISTURBANCES. 

Hemorrhages. — Hemorrhages  into  the  meninges  are  due  usually 
to  trauma.  Petechial  spots  are  sometimes  to  be  noted  in  infectious 
diseases  and  the  hemorrhagic  diatheses. 

INFLAMMATIONS. 

Leptomeningitis. — Inflammation  of  the  pia-arachnoidea  spinalis — 
leptomeningitis — is  hematogenic,  derived  by  extension  from  adjacent 
parts,  or  traumatic.  According  to  the  character  of  the  exudate,  we  may 
recognize  a  purulent,  a  seropurulent,  a  fibrinopurulent,  and  a  caseous 
form.  The  inflammation  may  be  localized  to  a  particular  district, 
may  extend  along  the  dorsal  or  ventral  aspects  of  the  cord,  or  may 
involve  the  whole  length  of  the  cord.  In  not  a  few  cases  it  is  combined 
with  inflammation  of  the  cerebral  pia-arachnoid  (cerebrospinal  menin- 
gitis). It  is  common  for  the  process  to  extend  by  the  fissure  and 
along  the  perivascular  lymphatics  to  the  cord,  where  small  hemor- 
rhages and  infiltrations  with  round  cells  are  not  infrequent  in  the 
cortical  portion  (meningomyelitis).  The  nerve-roots  may  also  be 
infiltrated  (neuritis).  As  examples  of  hematogenic  infection  may  be 
cited  the  so-called  idiopathic  or  sporadic  spinal  meningitis,  and  the 
epidemic  cerebrospinal  meningitis  (see  p.  544). 

Traumatic  meningitis  is  due  to  infective  agents,  usually  the  pus- 
producing  cocci,  which  are  introduced  into  the  spinal  canal  at  the 
time  of  injury,  or  invade  the  wound  subsequently. 

Should  the  meningitis  heal,  it  is  not  uncommon  to  find  circumscribed 
areas  of  fibrous  thickening,  of  a  pearly  white  appearance,  on  the  mem- 
branes, or  the  formation  of  more  or  less  extensive  adhesions  between 
the  neighboring  structures.  When  the  nerve-roots  are  involved  in 
a  cicatricial  process  we  get  secondary  degenerations  of  the  fibers. 
In  meningomyelitis  the  peripheral  portions  of  the  cord  may  show 
atrophy  and  sclerosis. 

Tuberculous  Spinal  Meningitis. — Tuberculous  spinal  meningitis  is 
occasionally  hematogenic,  but  in  the  great  majority  of  cases  arises 


TUMORS  573 

by  extension  of  tuberculous  disease  from  the  cerebral  meninges, 
vertebral  bones,  or  spinal  cord.  The  most  common  is  the  cervical 
leptomeningitis  that  so  often  accompanies  tuberculosis  of  the  cerebral 
membranes;  next,  the  form  resulting  from  Pott's  disease  of  the  spine. 
In  the  latter  case  the  infection  probably  extends  inward  from  the 
dura.  In  the  milder  or  less  advanced  conditions,  one  sees  small, 
isolated,  tuberculous  nodules,  arranged  chiefly  along  the  vessels. 
In  more  severe  cases,  however,  there  is  a  more  widespread  inflamma- 
tion, with  the  production  of  a  seropurulent  or  fibrinopurulent  exudate, 
of  yellowish-white  color,  sometimes  mixed  with  blood. 

The  process  may  extend  to  the  cord  and  nerve-roots,  so  that  a  tuber- 
culous meningomyelitis  or  neuritis  is  produced.  This  leads  to  more 
or  less  widespread  degeneration  of  the  nerve  elements. 

Syphilitic  Spinal  Meningitis. — Syphilitic  spinal  meningitis  is  rather 
rare.  It  takes  the  form  of  a  circumscribed  or  flattened,  diffuse,  and 
superficial  infiltration,  which  may  extend  to  the  cord,  on  the  one 
hand,  or  to  the  dura,  on  the  other.  Occasionally,  the  process  begins  in 
the  bone  or  dura  and  extends  to  the  pia-arachnoid  secondarily.  The 
process  in  time  leads  to  inflammatory  induration  and  thickening  of  the 
pia-arachnoid,  with  the  formation  of  adhesions  between  the  various 
structures.  In  the  central  portions  of  the  infiltrations,  necrosis  or 
gummatous  degeneration  is  frequently  observed.  The  sequelae  are 
in  all  respects  similar  to  those  in  the  tuberculous  form. 

PROGRESSIVE  METAMORPHOSES. 

Tumors. — Small,  flattened  plates  of  bone  are  not  infrequently 
found  in  the  arachnoid,  which  are  supposed  to  be  due  to  degenerative 
changes  in  the  connective  tissue.  They  are  supplied  with  vessels 
from  the  dura.  It  is  questionable  whether  they  belong  to  the  cate- 
gory of  true  osteoma.  Cartilaginous  plaques  are  also  found  in  the 
arachnoid. 

Varicose  dilatations  of  the  veins  of  the  pia,  which  occasionally  assume 
the  form  of  cavernous  angiomas,  are  sometimes  met  with.  They  cause 
more  or  less  extensive  compression  of  the  cord  and  nerve  roots. 

Of  primary  tumors  proper,  the  sarcoma,  fibroma,  myxoma,  lipoma, 
angioma,  psammoma,  and  cholesteatoma  should  be  mentioned.  It 
may  be  remarked  that  all  tumors  of  the  cord  and  its  membranes  tend 
to  assume  a  flattened,  elongated  form,  owing  to  the  contracted  and  un- 
yielding boundaries  within  which  they  lie.  The  sarcomas  form  circum- 
scribed or  flattened  diffuse  growths,  which  tend  to  invade  the  neighboring 
structures.  The  alveolar  endothelioma  is  the  most  important  variety. 
Owing  to  the  abundant  formation  of  bloodvessels  in  certain  tumors, 
we  may  distinguish  angiomas  and  angiosarcomas.  Myxomatous  degen- 
eration is  not  uncommon  in  these  cases,  and  hyaline  changes  may  be 
so  marked  as  to  warrant  us  in  calling  the  growth  a  cylindroma. 

Fibromas  are  rounded  or  flattened,  firmish  growths,  of  pale  color  and 
smooth  surface,  which  originate  commonly  from  the  perineurium  of 


574  THE  SPINAL  PIA-ARACHNOID 

the  nerve-roots.  They  are  often  multiple,  and,  as  we  have  seen  in  one 
case,  may  lead  to  notable  compression  of  the  cord. 

Lipomas  are  found,  commonly  in  association  with  spina  bifida. 

Secondary  tumors  of  the  spinal  meninges  are  the  carcinoma,  sarcoma, 
and  myeloma.  As  a  rule,  the  vertebral  column,  nerve-roots,  and  the 
cord  itself  are  involved  as  well,  and  the  symptoms  of  compression  myelitis 
are  produced,  root  pains,  spastic  paralysis,  increased  knee-jerks,  and 
bladder  symptoms. 


THE  SPINAL  CORD  AND  MEDULLA  OBLONGATA. 


CIRCULATORY  DISTURBANCES. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  is  commonly 
met  with  in  the  acute  inflammations  of  the  cord  or  meninges.  In  such 
cases  the  white  matter  presents  a  delicate,  rosy  flush,  and  the  gray  matter 
is  somewhat  brownish. 

Passive  Hyperemia. — Passive  hyperemia  is  found  in  chronic  cardiac 
and  pulmonary  affections,  and  in  those  who  have  been  bedridden  for 
prolonged  periods. 

Hemorrhages. — Hemorrhages  into  the  substance  of  the  cord  are 
either  punctate  or  massive.  This  condition  is  distinctly  less  frequent 
than  cerebral  hemorrhage.  The  causes  are,  however,  much  the  same  in 
both  cases.  Fatty,  hyaline,  or  calcareous  degeneration  of  the  vessels, 
thrombosis,  embolism,  sudden  alterations  in  blood  pressure,  and  trauma- 
tism,  play  the  leading  role.  With  the  exception  of  the  traumatic  forms, 
the  lesions  are  more  apt  to  be  found  in  the  medulla  and  upper  part  of 
the  cord  than  elsewhere.  Hemorrhage  into  the  medulla  is  the  main  cause 
of  the  disease  known  as  acute  bulbar  paralysis. 

The  minute,  punctate,  or  capillary  hemorrhages  are  commonly  asso- 
ciated with  the  infections  and  intoxications,  and  are  met  with  in  such 
conditions  as  active  and  passive  hyperemia,  inflammations,  tetanus, 
hydrophobia,  strychnine  poisoning,  degenerative  softening,  and  con- 
cussion of  the  spine.  They  are  often  met  with,  also,  in  the  so-called 
"  caisson  disease." 

The  hemorrhages  appear,  on  cross-section  of  the  cord,  as  minute, 
reddish  dots,  scattered  irregularly  through  the  substance.  In  traumatic 
cases  the  lesions  are  generally  found  in  the  gray  matter,  and  usually 
in  the  dorsal  horns. 

In  the  more  extensive  extravasations,  the  effused  blood  generally  follows 
the  line  of  least  resistance.  The  whole  thickness  of  the  cord  may  be 
involved,  local  collections  may  be  formed,  or  the  blood  may  dissect  its 
way  along  the  fibers  up  and  down  the  cord.  Subsequently,  degenera- 
tion of  the  nerve-substance  takes  place,  with  the  formation  of  extensive 
cavities  in  the  cord  (hematomyelopore),  Van  Gieson).  Occasionally, 
the  blood  may  escape  into  the  central  canal,  which  thereupon  becomes 


ACUTE  HEMATOGENIC  MYELITIS  575 

dilated  (hematomyelia),  or  into  the  membranes.  Should  the  patient 
survive,  the  subsequent  changes  in  the  destroyed  area  are  similar  to 
those  described  in  the  case  of  cerebral  hemorrhage. 

Anemia. — Anemia,  or,  perhaps,  more  correctly,  in  the  majority  of 
instances,  ischemia,  is  not  infrequent,  and  is  due  to  a  variety  of  causes. 
The  arteries  of  the  cord  are  practically  all  end-arteries,  so  that  their 
occlusion  by  endarteritis,  thrombosis,  or  embolism  is  followed  by  local 
areas  of  anemia,  softening,  and  necrosis.  The  pressure  of  inflammatory 
collections,  effused  blood,  or  tumors  on  the  cord  is  followed  by  ischemia. 

The  cord  is  also  ischemic  in  cases  of  pernicious  and  other  systemic 
anemias. 

INFLAMMATIONS. 

Myelitis. — Myelitis  in  the  stricter  sense,  or  inflammation  of  the 
spinal  cord,  is  due  to  hematogenic  causes,  to  the  extension  of  inflamma- 
tory processes  from  the  meninges  or  veretebral  column,  less  commonly 
from  the  central  canal,  and  to  traumatism.  According  as  the  process 
affects  the  gray  or  the  white  matter,  writers  have  been  accustomed  to 
differentiate  a  poliomyelitis  and  a  leukomyelitis.  Several  terms,  also, 
are  used  to  designate  certain  localizations  of  the  inflammation,  which 
sufficiently  explain  themselves.  Such  are  disseminated,  diffuse,  trans- 
verse, central,  and  annular  myelitis. 

The  earlier  stages  of  the  process  are  not  precisely  understood.  It  is 
probable,  however,  that  many  cases  begin  with  degeneration  or  obstruc- 
tion of  the  vessels,  followed  by  the  production  of  minute  hemorrhages 
and  the  ordinary  manifestations  of  inflammation.  These  changes  may 
be  brought  about  by  bacterial  or  other  toxins,  such  as  ergot,  lead,  or 
arsenic,  or  by  the  actual  localization  of  bacteria  in  the  cord.  The  agents 
particularly  concerned  are  the  pyogenic  cocci,  the  Pneumococcus,  the 
tubercle  bacillus,  and  the  toxic  products  of  rabies,  tetanus,  leprosy,  and 
syphilis. 

Coincident  with  this,  or  following  hard  upon  it,  are  well-marked 
degenerative  changes  in  the  nerve-fibers  and  cells,  brought  about,  prob- 
ably, by  the  combined  action  of  the  toxin,  the  interference  with  the  circu- 
lation, and  the  pressure  of  the  inflammatory  products. 

Acute  Hematogenic  Myelitis. — Acute  hematogenic  myelitis  arises 
occasionally  in  the  course  of  such  affections  as  rabies,  typhoid  fever, 
dysentery,  influenza,  rheumatism,  tonsillitis,  pneumonia,  and  gonorrhoea. 
In  some  few  cases,  it  appears  to  be  due  to  infective  emboli  that  have 
reached  the  cord  from  the  cardiac  valves,  or  from  suppurative  or  other 
inflammatory  foci  in  some  distant  part  of  the  body.  Occasionally,  cases 
arise  which  are  on  analogy  infective  and  hematogenic,  yet  a  definite 
cause  cannot  be  made  out. 

No  particular  rule  is  followed  with  regard  to  the  distribution  of  the 
lesions.  They  are  apt  to  be  multiple,  and  may  be  confined  to  one  small 
section  of  the  cord,  or  may  involve  several  segments.  In  some  cases  cer- 
tain "systems"  are  picked  out  and  the  disease  may  extend  rapidly  along 
the  cord. 


576  THE  SPINAL  CORD 

According  to  the  nature  of  the  invading  microorganism,  we  may 
distinguish  simple  and  suppurative  forms  of  myelitis. 

When  removed  from  the  body,  the  spinal  cord  in  these  cases  will  pre- 
sent a  variable  picture  according  to  the  severity  and  extent  of  the  process. 
The  cerebrospinal  fluid  may  be  increased.  The  pia  is  commonly  in- 
jected, especially  over  the  affected  areas.  When  palpated,  the  myelitic 
foci  can  readily  be  detected  by  their  soft,  pultaceous  consistence,  quite 
unlike  the  firm,  resilient  feel  of  the  normal  cord.  On  section,  in  ad- 
vanced cases,  these  spots  are  semiliquid  and  the  distinctive  structure 
of  the  cord  can  no  longer  be  made  out.  In  color,  the  destroyed  substance 
is  reddish  or  reddish-brown  (red  softening).  In  the  later  stages  it  may  be 
more  yellowish  (yellow  softening).  In  milder  forms,  however,  the  cord 
may  show  only  scattered  patches  of  congestion,  and  the  inflammatory 
nature  of  the  lesions  is  first  detected  on  making  a  microscopic  examina- 
tion. 

We  may  now  refer  more  in  detail  to  the  leading  forms  of  acute  hemato- 
genic  myelitis 

Acute  Anterior  Poliomyelitis. — Acute  anterior  poliomyelitis,  the  in- 
fantile palsy  of  clinicians,  is  a  disease  of  the  cord  in  which  the 
ganglion  cells  of  the  anterior  horns,  the  axones  proceeding  from  them, 
and  the  muscles  supplied,  are  the  parts  chiefly  affected.  It  is  generally 
believed  now  that  this  affection  is  due  to  some  hematogenic  infection  or 
intoxication.  It  is  found  almost  exclusively  in  young  children,  occa- 
sionally assuming  an  epidemic  character.  It  follows  exposure  to  cold, 
and  is  an  occasional  sequel  of  certain  of  the  infectious  fevers,  such  as 
measles  and  scarlatina.  It  seems  probable  that  the  causative  agent 
acts  first  upon  the  bloodvessels  and  subsequently  on  the  ganglia,  contrary 
to  what  used  to  be  thought.  Clinically,  the  disease  is  characterized  by 
a  flaccid  paralysis  of  one  or  more  extremities,  followed  by  atrophy  of 
certain  groups  of  muscles  and  eventually  contractions  and  deformities. 
The  reflexes  are  absent.  Sensation  is  not  impaired. 

Post  mortem,  the  cord  presents  congestion  of  the  gray  matter,  particu- 
larly of  the  ventral  horns,  which  are  often  the  seat  of  red  softening.  The 
pia  is  injected,  especially  in  its  anterior  portion,  and  the  cerebrospinal 
fluid  may  be  increased.  With  low  magnification  it  can  generally  be 
made  out  that  one  horn  is  affected  more  than  the  other. 

Microscopically,  the  most  marked  changes  are  to  be  found  in  the 
antero-exterior  part  of  the  ventral  horn.  Occasionally  the  anterior 
lateral  tracts  of  the  white  substance  are  involved,  rarely  the  posterior 
columns.  The  vessels  of  the  affected  region  are  congested,  and  there 
may  be  small  hemorrhages,  while  the  perivascular  lymph-spaces  are 
distended  with  inflammatory  leukocytes.  The  glia  is  cedematous  and 
infiltrated  in  places  with  round  cells.  The  ganglion  cells  vary  in  appear- 
ance according  to  the  age  and  intensity  of  the  process.  They  may  be 
swollen,  turbid,  and  stain  badly.  The  chromatin  granules  are  arranged 
in  irregular  clumps,  the  nucleus  stains  diffusely,  the  nucleolus  is  vacuo- 
lated,  and  the  protoplasmic  processes  are  irregular.  Later,  the  nuclei 
have  disappeared,  the  cells  are  shrunken,  deformed,  and  the  proto- 


ACUTE  TRANSVERSE  MYELITIS  577 

plasmic  processes  have  disappeared,  leaving  only  a  thickened  axis 
cylinder  attached  to  the  degenerated  cell-body.  Finally,  many  of  the 
ganglia  disappear.  The  fibers  in  the  anterior  roots  present  the  usual 
features  of  nerve  degeneration,  the  myelin  sheath  is  breaking  down 
into  droplets  of  myelin  and  fat,  the  axis-cylinders  are  swollen  and  frag- 
mented. The  degeneration  may  extend  down  the  peripheral  nerves  and 
lead  to  atrophy  of  the  corresponding  muscle  groups.  The  glia  is  usually 
somewhat  increased. 

FIG.  156 


Old  anterior  poliomyelitis;  hypoplasia  of  the  right  half  of  the  shoulder-girdle,  thorax,  and  right 
upper  extremity.     (From  the  Medical  Clinic  of  the  Montreal  General  Hospital.) 

As  the  disease  subsides,  or  becomes  chronic,  the  ventral  horn  is  found 
to  be  considerably  atrophied.  The  ganglion  cells  are  diminished  in 
number,  and  those  that  remain  show  evidences  of  degeneration.  Certain 
fibers  of  the  anterior  roots  have  also  disappeared.  The  peri  vascular 
infiltration  with  round  cells  is  still  noticeable,  and  compound  granular 
37 


578  THE  SPINAL  CORD 

cells  may  be  found  in  fair  numbers.  Years  after,  when  the  disease  has 
completely  subsided,  that  portion  of  the  cord  corresponding  to  the  site  of 
the  lesion  is  distinctly  atrophied,  and  its  contour  is  thereby  altered.  On 
section,  one  or  both  of  the  ventral  horns  is  markedly  atrophied.  As  a 
rule,  one  horn  only  is  noticeably  involved,  while  the  other  is  normal  or  but 
slightly  affected.  Occasionally,  the  horn  is  about  normal  size,  although  the 
normal  structure  is  lost,  owing  to  what  appears  to  be  a  colloid  degenera- 
tion of  the  neuroglia.  In  certain  parts  the  ganglia  have  entirely  dis- 
appeared. Those  that  remain  are  usually  normal.  The  bloodvessels  are 
large  and  their  walls  thickened.  The  glia  is  considerably  increased,  and 
appears  as  a  delicate  meshwork  containing  abundant  nuclei.  Deiters' 
cells,  or  astrocytes,  may  be  found  in  considerable  numbers.  The  medul- 
lated  fibers  of  the  anterior  roots  have  more  or  less  degenerated,  and  the 
nerve-trunk  looks  thin  and  atrophic.  Occasionally,  a  few  fibers  in  the 
pyramidal  tracts,  in  the  immediate  neighborhood  of  the  primary  lesion, 
may  be  degenerated,  but  this  is  only  trifling. 

The  muscles  innervated  from  the  affected  region  of  the  cord  rapidly 
atrophy,  the  fibers  waste  and  disappear,  the  connective  tissue  is  increased 
and  may  be  infiltrated  with  fat.  In  children,  not  only  the  muscles,  but 
the  bones  and  vessels  are  markedly  involved,  and  the  limb  may  remain 
stunted  or  lag  behind  in  its  development. 

Acute  Transverse  Myelitis. — Acute  transverse  myelitis  is  occasionally 
met  with  in  infectious  diseases,  but  sometimes  without  very  evident  cause. 

In  the  early  stages,  the  whole  thickness  of  the  cord  is  swollen  and  soft- 
ened for  a  short  distance.  On  section,  the  affected  part  is  reddened, 
cedematous,  and  may  present  hemorrhages.  The  cut  surface  assumes 
a  convex  form.  Microscopically,  the  vessels  are  congested  and  are 
surrounded  by  clumps  of  leukocytes,  chiefly  of  the  polymorphonuclear 
variety.  The  interstitial  substance  is  cedematous.  The  glia  cells  are 
swollen  and  increased  in  numbers.  The  axis-cylinders  are  swollen, 
fragmented,  or  atrophied,  and  the  myelin  sheaths  are  degenerating. 
The  ganglion  cells  stain  irregularly,  their  nuclei  are  dislocated,  and  the 
protoplasmic  processes  are  varicosed  or  fragmented.  Later,  numbers 
of  granular  cells  are  found,  which  are  probably  connective-tissue  cor- 
puscles containing  the  debris  of  the  broken-down  tissue.  "Amyloid" 
bodies  may  also  be  seen.  This  stage  may  be  regarded  as  one  of  red 
softening.  It  soon  gives  place  to  yellow  softening.  The  cord  is  some- 
what swollen,  and  of  a  yellowish  color.  The  gray  matter  is  distinctly 
wasted.  The  microscopic  appearances  are  similar  to  those  just  described, 
save  that  the  neuroglia  is  more  swollen  and  looser,  consequently,  in 
texture,  the  glia  cells  begin  to  show  degeneration,  granular  cells  are  still 
more  numerous,  and  the  parenchymatous  changes  are  more  marked. 
The  ganglion  cells  are  swollen,  irregular,  and  vacuolated,  or,  again, 
they  may  be  shrunken,  stain  badly,  and  contain  no  nuclei.  Some 
may  be  represented  by  a  protoplasmic  sac  containing  brownish  pig- 
ment, and  some  have  totally  disappeared,  as  is  proved  by  the  fact 
that  the  number  of  ganglia  is  reduced.  In  very  severe  cases,  the  degen- 
eration is  so  extreme  that  the  affected  portion  of  the  cord  is  reduced  to 


LANDRY'S  PARALYSIS  579 

a  pultaceous  mass,  composed  of  fat  globules,  granular  cells,  and  detritus. 
In  other  cases,  where  the  process  has  somewhat  subsided  in  intensity, 
reparative  changes  make  their  appearance.  The  ganglia  and  nerve 
fibers  have  largely  disappeared,  the  granular  cells  are  less  numerous  and 
are  found  mainly  about  the  bloodvessels,  while  the  glia  shows  prolifera- 
tion and  is  more  abundant.  Secondary  degeneration  may  appear  in 
certain  tracts  above  and  below  the  inflamed  area. 

Mvelitic  processes  heal,  when  they  do  so,  with  the  replacement  of  the 
destroyed  nerve-elements  by  dense  scar  tissue,  giving  the  cord  at  the 
affected  part  a  shrunken,  grayish  appearance  and  a  somewhat  hard 
consistence.  This  is,  in  part,  due  to  hyperplasia  of  the  glial  cells,  but 
in  part,  also,  to  proliferation  of  the  connective  tissue  derived  from 
the  sheaths  of  the  bloodvessels  and  the  prolongations  of  the  pia. 

The  clinical  features  depend  on  the  position  of  the  lesion.  Transverse 
myelitis  of  the  thoracic  cord  gives  rise  to  spastic  paraplegia  of  the  lower 
extremities  without  atrophy,  paralysis  of  the  abdominal  muscles,  par- 
alysis of  the  bladder  and  intestines,  and  anesthesia  below  the  level  of  the 
lesion.  A  lesion  of  the  lower  cervical  region  causes  flaccid  paralysis  of 
the  arms  with  atrophy,  spastic  paralysis  of  the  legs,  loss  of  sensation 
in  the  arms  and  below  the  level  of  the  second  rib,  pupillary  changes, 
and  respiratory  embarrassment.  When  the  lumbar  region  is  involved 
we  get  flaccid  paralysis  of  the  legs  with  atrophy,  paralysis  of  the  bladder 
and  bowels,  and  loss  of  the  reflexes. 

Acute  Suppurative  Myelitis. — Acute  suppurative  myelitis,  when  of 
hematogenic  origin,  is  rare.  It  occasionally  is  found  secondary  to 
bronchiectasis,  suppuration  in  the  genito-urinary  tract,  and  dysenteric 
abscess  of  the  liver.  The  suppuration  may  be  somewhat  diffuse,  fol- 
lowing the  line  of  the  bloodvessels,  or  small  abscesses  may  be  produced. 
The  pathological  changes  do  not  differ  materially  from  those  in  other 
forms  of  acute  myelitis,  except  that  the  process  is  more  intense.  When 
the  abscesses  are  small  and  isolated,  the  degenerative  changes  are  found 
only  in  the  immediate  neighborhood,  while  the  intervening  tissue  is 
comparatively  or  entirely  free. 

Acute  myelitis,  arising  by  extension,  can  usually  be  traced  to  inflam- 
mation of  the  membranes  and  nerve-roots,  or,  rarely,  to  infection  from 
the  central  canal.  In  the  former  case  the  disease  is  really  a  meningo- 
myelitis.  This  may  be  suppurative,  tuberculous,  or  syphilitic.  Here, 
the  infective  agents  probably  travel  by  means  of  the  bloodvessels  and 
perivascular  lymphatics.  As  would  be  expected,  the  lesions  are  most 
marked  at  the  periphery  of  the  cord. 

Myelitis  of  central  origin  is  generally  due  to  abscesses  or  suppurative 
inflammation  somewhere  in  the  ventricles,  the  infective  agents  from  which 
set  up  inflammation  along  the  whole  length  of  the  cord.  The  changes 
produced  are  oedema,  swelling,  and  disintegration  of  the  cells  in  the  gray 
commissure,  extending  into  the  ventral  and  dorsal  horns,  and  occasion- 
ally even  into  the  white  substance. 

Landry's  Paralysis. — Apart  from  tuberculosis  and  syphilis  of  the  cord, 
which  will  be  described  hereafter,  perhaps  the  most  important  affection 


580  THE  SPINAL  CORD 

coming  under  this  category  is  acute  ascending  paralysis  (Landry's  par- 
alysis). This  affection,  as  its  name  implies,  is  an  acute  one,  character- 
ized clinically  by  the  occurrence  of  paralysis,  usually  extending  from 
below  upward,  and  ending  quickly  in  death,  owing  to  involvement  of 
the  bulbar  nuclei.  Sensory  symptoms  are  in  abeyance,  and  the  affected 
muscles  preserve  their  faradic  irritability.  The  spleen  is  enlarged, 
sometimes  also  the  various  lymph-nodes,  and  the  kidneys  show  degener- 
ative changes.  This  would  suggest  a  general  systemic  infection  as  the 
cause,  although  the  nature  of  it  is  quite  obscure.  Probably  a  variety 
of  agents  may  cause  it.  Judging  from  clinical  features,  the  lesions 
may  be  primary  in  the  cord  and  medulla,  or  may  extend  to  it  from  the 
peripheral  nerves. 

The  histological  features  of  the  disease  have  been  much  debated. 
Landry  did  not  find  changes  in  the  central  or  peripheral  nervous  system. 
Others  believe  the  cord  to  be  extensively  involved,  while  the  majority 
regard  the  disease  as  primary  in  the  peripheral  motor  neurones.  The 
lesions  apparently  vary.  In  one  case,  they  are  practically  those  of  an 
acute  poliomyelitis.  In  another,  there  is  an  acute  exudative  inflamma- 
tion of  the  connective  tissue  of  the  peripheral  nerves,  which  may  be 
simple,  hemorrhagic,  or  suppurative,  leading  to  extensive  degeneration 
of  the  nerve-elements. 

Traumatic  Myelitis. — Traumatic  myelitis  includes  all  those  forms  which 
result  from  injuries,  such  as  gunshots,  cutting  or  stabbing,  contusions, 
fractures,  and  dislocations  of  the  vertebrae.  These  injuries  lead  to 
more  or  less  laceration  and  compression  of  the  cord,  or  even  to  solution 
of  continuity  or  complete  destruction  of  the  cord  at  the  affected  region. 
The  amount  of  inflammation,  of  course,  depends  on  the  nature  and  extent 
of  the  injury.  The  injury  leads  to  the  rupture  and  disintegration  of 
certain  of  the  nerve-fibers  and  ganglia,  and  this  leads  to  secondary 
degeneration  in  the  tracts  functionally  connected  with  the  destroyed 
structures.  The  glia  and  connective  tissue  mav  also  be  involved.  In 
severe  cases  the  section  of  the  cord  affected  may  soften  and  become 
liquefied.  Should  there  be  but  little  blood,  we  get  the  well-known 
"white"  softening.  Where  blood  is  effused,  we  have  "red"  and  later 
"yellow"  softening.  Interference  with  the  free  flow  of  blood  or  lymph 
in  the  vessels  may  lead  to  foci  of  softening  in  districts  remote  from  the 
injury.  If  infection  with  pyogenic  microorganisms  take  place,  we  may 
have  abscess  of  the  cord  with  extension  of  purulent  inflammation  to  the 
meninges.  In  the  milder  forms,  healing  may  take  place  by  sclerosis, 
or  replacement  of  the  destroyed  tissue  by  proliferated  glia  cells.  In 
the  severer  cases,  especially  where  the  membranes  are  involved,  a  dense 
connective-tissue  scar  may  be  produced. 

Tuberculosis. — Tuberculosis  of  the  cord  is  rarely  primary.  Collins1 
has  met  with  one  case  of  tuberculous  myelitis  in  which  no  other 
tuberculous  lesion  was  detected  in  the  body.  As  a  rule,  however,  there 

1  American  Text-book  of  Pathology,  W.  B.  Saunders  &  Co.,  Phila.  and  London, 
1901 :  567. 


RETROGRESSIVE  METAMORPHOSES  581 

is  tuberculosis  elsewhere.  The  disease  takes  three  forms — multiple 
mi  I  in  ry  granulomas,  larger  single  or  multiple  nodules,  and  meningo- 
myelitis. 

In  the  first  mentioned  form,  numerous  small  tubercles,  often  of  micro- 
scopic size,  are  found  both  in  the  gray  and  the  white  substance,  usually 
about  the  vessels,  indicating  a  hematogenic  mode  of  infection.  The 
obstruction  to  the  circulation  thus  caused  and  the  consequent  lack  of 
nutrition  leads  to  areas  of  ischemic  softening,  and  there  may  even  be 
secondary  degeneration  of  the  fibers  near  by. 

In  the  second  variety  granulomas  sometimes  as  large  as  a  hazelnut 
may  be  found,  which  present  extensive  central  caseation  or  even  lique- 
faction. The  tubercles  may  extend  to  the  meninges  or  break  into  the 
central  canal,  so  that  more  widespread  infection  may  take  place.  In  some 
cases  the  continuity  of  the  cord  is  practically  entirely  interrupted. 
Secondary  degeneration  of  neighboring  fibers  occurs. 

The  most  common  form  is  the  tuberculous  meningomyelitis.  Here  the 
infection  spreads  from  the  meninges  by  means  of  the  intraspinal  prolonga- 
tions of  the  pia,  through  the  perivascular  lymphatics.  Small  tubercles 
are  found  about  the  vessels,  which  may  in  time  reach  a  fair  size,  caseate, 
and  lead  to  considerable  destruction  of  the  nerve-substance. 

Syphilis. — Some  writers  are  inclined  to  believe  in  the  existence  of 
a  simple  acute  myelitis  occurring  in  the  earlier  years  of  syphilis,  which 
is  not  unlike  the  non-specific  forms  of  diffuse  myelitis  before  referred 
to.  There  is  the  same  degeneration  of  the  specific  nerve-elements  with 
cellular  infiltration  of  the  connective  tissue.  The  bloodvessels  appear 
to  be  specially  implicated.  They  show  endarteritis,  may  be  thrombosed, 
and  in  their  vicinity  small  hemorrhages  may  be  seen.  Less  questionable, 
however,  is  syphilitic  meningomyelitis.  This  is  most  common  in  the 
cervical  and  dorsal  regions  of  the  cord.  The  main  characteristic  is 
the  thickening  or  degeneration  of  the  vessels  with,  sometimes,  the  forma- 
tion of  small  gummas  along  their  course.  This  leads  to  degeneration 
of  the  nerve-fibers  and  ganglia.  Secondary  degeneration  is  common. 
There  is  a  small-celled  infiltration  about  the  vessels,  and  the  glia  is 
increased. 

Leprosy. — In  a  few  cases  we  find  merely  atrophy  and  degeneration 
of  the  nerve-elements,  particularly  the  ganglia.  As  a  rule,  however, 
there  are  areas  of  softening  and  hemorrhagic  extravasation.  Micro- 
scopically, the  nerve  structures  are  degenerated,  and  the  connective  tissue 
shows  inflammatory  exudation  and  hemorrhage.  The  lepra  bacilli  have 
been  found  in  the  connective  tissue,  both  of  the  gray  and  the  white 
substance. 

RETROGRESSIVE  METAMORPHOSES. 

We  pass  on  now  to  discuss  the  various  forms  of  atrophy  and  degenera- 
tion to  which  the  spinal  cord  is  liable.  We  have  to  premise,  however, 
that  the  subject  is  beset  with  great  difficulties.  As  has  been  before 
remarked  (p.  556),  the  nervous  tissue  is  the  most  delicate  and  highly 


582  THE  SPINAL  CORD 

specialized  structure  in  the  body.  It  is,  consequently,  particularly 
susceptible  to  the  action  of  all  kinds  of  deteriorating  agencies,  while 
its  recuperative  powers  are  slight.  This  explains  why  it  is  that  disinte- 
gration and  degeneration  are  the  most  constant  and  striking  pathological 
changes  which  meet  the  investigator.  These  retrogressive  phenomena 
are  produced  by  the  most  diverse  causes,  and  it  is  not  always  possible, 
in  any  given  case,  to  determine  the  etiological  factor  chiefly  or  entirely  to 
blame.  Thus,  the  distinction  between  inflammatory  and  pure  degenera- 
tions cannot  always  be  made.  Nevertheless,  it  is  the  custom  among 
clinicians  to  apply  the  generic  term  myelitis,  or  inflammation  of  the  cord, 
to  all  forms  of  degeneration,  irrespective  of  the  cause,  inasmuch  as  they 
are  characterized  by  fairly  definite  and  constant  symptoms,  depending 
on  the  localization  of  the  lesion.  The  term  "myelitis,"  in  this  wide 
sense,  is  a  convenient  one  and  thoroughly  established  by  custom,  but 
should  be  used  with  a  certain  mental  reservation. 

In  order  to  get  a  clear  understanding  of  degeneration  of  nerve-tissue 
and  its  results,  it  is  important  to  bear  certain  facts  in  mind. 

According  to  the  "  neurone"  concept  of  the  histological  structure  of 
the  nervous  system,  commonly  held  at  the  present  day,  the  brain  and 
spinal  cord,  with  their  prolongations,  the  peripheral  nerves,  are  to  be 
regarded  in  the  main  as  a  peculiar  aggregation  of  highly  specialized  cells, 
consisting  of  a  large  cell-body  with  protoplasmic  processes  (the  ganglion 
cell)  from  which  proceeds  a  single  long  and  attenuated  thread  (axis- 
cylinder  or  neuraxone).  The  whole  constitutes  the  neurone.  Each 
neurone  is,  so  to  speak,  self-contained,  and  has  no  communication  with 
adjacent  neurones,  save  by  contiguity. 

The  various  constituents  of  the  neurone  may  act  differently  when 
subjected  to  abnormal  conditions.  In  general,  it  may  be  said  that  a 
nerve-fiber  or  neuraxone  when  severed  from  any  cause  from  its  nutrient 
centre,  the  ganglion  cell,  will  degenerate.  The  process  begins  at  the 
distal  extremity  and  extends  gradually  backward  to  the  site  of  the  lesion. 
In  some  cases  the  degeneration  is  primary;  that  is  to  say,  it  is  due  to 
some  cause  acting  locally  and  directly  on  a  fiber  or  bundle  of  fibers. 
The  lesion  may  be  chiefly  or  entirely  confined  to  one  physiological  tract 
of  the  cord,  and  we  then  speak  of  a  primary  "system"  disease.  Or  more 
than  one  tract  may  be  involved  and  we  have  a  combined  ''system'' 
disease.  In  other  cases,  and  probably  the  majority,  the  degeneration 
may  be  referred  to  some  lesion  at  a  distance,  such  as  destruction  of 
the  ganglion  cells  nourishing  the  fibers,  or  anything  which  interferes 
with  the  conducting  power  of  the  fibers.  This  is  called  secondary 
degeneration. 

In  primary  degeneration  certain  tracts  appear  to  be  specially  picked 
out.  These  are  the  sensory  neurones  of  the  cord,  which  may  be  traced 
from  the  posterior  nerve-roots  into  the  column  of  Burdach,  thence  into 
the  column  of  Goll,  to  end  finally  in  the  medulla  in  the  nuclei  of  Goll 
and  Burdach;  the  central  motor  neurones,  starting  in  the  pyramidal  layer 
of  the  motor  cortex,  and  passing  through  the  internal  capsule,  the  pyra- 
mids, into  the  pyramidal  tracts ;  the  peripheral  motor  neurones,  beginning 


RETROGRESSIVE  METAMORPHOSES  583 

in  the  ganglia  of  the  ventral  horns,  and  extending  through  the  anterior 
roots  to  the  muscles. 

Secondary  degeneration  is  divided  into  ascending  and  descending, 
according  to  the  direction  it  takes  in  the  cord.  Ascending  degeneration 
is  generally  found  in  the  posterior  columns,  the  direct  cerebellar  tract, 
and  the  anterolateral  tract  of  Gowers.  It  may  be  associated  with  lesions 
of  the  ganglia  in  Clarke's  columns.  The  process  terminates  in  the  resti- 
form  bodies  of  the  medulla.  Descending  degeneration  mainly  affects 
the  pyramided  tracts,  Thus,  in  the  case  of  a  unilateral  lesion  above 
the  decussation,  say,  in  the  motor  cortex,  we  find  degeneration  in  the 
anterior  pyramidal  tract  on  the  same  side  and  in  the  lateral  or  crossed 
pyramidal  tract  on  the  opposite  side.  A  few  motor  fibers  appear  not  to 
decussate  and  pass  down  through  the  lateral  columns.  In  long-standing 
cases,  atrophy  of  the  ganglion  cells  of  the  ventral  horns  has  been  observed. 
Descending  degeneration  has  at  times  been  noted  in  the  posterior  columns. 
In  the  upper  part  of  the  cord  it  affects  two  small  tracts  passing  outward 
and  backward  from  a  point  slightly  behind  the  gray  commissure  (comma-- 
degeneration of  Schultze).  Lower  down  the  fibers  approach  the  posterior 
commissure,  where  they  form  the  oval  field  of  Flechsi^. 

Secondary  degeneration  is  apt  to  be  of  considerable  extent,  since  the 
nerve  paths  are  so  elongated  and  their  correlation  is  close. 

The  causes  of  nerve  degeneration  are  numerous.  Chief  among  them 
may  be  mentioned  mechanical  trauma;  circulatory  disturbances,  such 
as  anemia,  embolism,  thrombosis,  endarteritis,  and  hemorrhage;  and 
toxins  of  bacterial,  mineral,  or  vegetable  origin.  In  some  cases  there 
may  be  a  combination  of  factors  at  work. 

Considerable  difficulty  is  experienced  when  one  attempts  to  decide 
upon  a  logical  classification  of  the  degenerative  affections  of  the  spinal 
cord.  We  have  referred  above  to  "primary"  and  "secondary"  degenera- 
tions. Many  authorities  speak  of  "primary  system  disease,"  meaning 
by  that  a  disease  in  which  the  lesions  are  confined  to  a  definite  "system" 
or  nerve-tract,  involving  a  greater  or  less  extent  of  its  course,  which  can- 
not be  referred  etiologically  to  any  obvious  or  gross  external  anatomical 
change.  This  is  certainly  a  convenient  clinical  generalization,  but  it 
may  well  be  doubted,  from  the  point  of  view  of  the  pathological  histol- 
ogist,  whether  the  lesions  in  question  ever  are  restricted  to  one  physio- 
logical nerve-tract.  Again,  in  many  cases,  opinions  are  divided  as  to 
the  propriety  of  the  term  "primary,"  inasmuch  as  our  knowledge  of  the 
origin  and  course  of  the  pathological  changes  is  still  imperfect,  while 
primary  and  secondary  manifestation  are  often  so  intimately  associated. 
Of  course,  wiiere  it  is  possible,  our  classifications  of  disease  should  be 
based  upon  pathogeny  and  morbid  anatomy,  and  it  should  be  our  aim 
to  show  the  harmony  between  these  and  the  clinical  features.  In  many 
cases,  however,  as  here,  we  must  to  a  large  extent  be  guided  by  expedi- 
ency. It  has  been  deemed  wiser  here,  therefore,  not  to  draw  too  fine 
distinctions,  but  to  adopt  a  mainly  "regional"  classification. 

Among  the  commonest  forms  of  spinal  cord  degeneration  is  that  due 
to  pressure,  the  so-called  "compression  myelitis."  As  a  rule,  the  lesion 


584  THE  SPINAL  CORD 

is  a  transverse  one,  affecting  all  the  elements  of  the  cord  in  a  compara- 
tively restricted  area.  It  may  be  caused  by  traumatism,  such  as  fracture 
of  the  vertebral  column  with  pressure  of  a  lamina  upon  the  cord,  but  is 
often  also  due  to  tuberculous  caries  of  the  spine,  tuberculosis  of  the 
meninges,  and  primary  or  secondary  tumors  in  the  vertebral  canal  or 
in  the  cord  itself.  Central  degeneration  may  be  caused  by  the  accumu- 

Fio.  157 


Compression  myelitis.     Cervical  cord.      (From  the  collection  of  Dr.  Colin  K.  Russel.) 

FIG.  158 


Compression  myelitis.     Dorsal  cord.     Degeneration  in  the  posterior  and  lateral  columns. 
(From  the  collection  of  Dr.  Colin  K.  Russel.) 

lation  of  blood  or  fluid  in  the  central  canal.  Any  of  these  conditions  will 
cause  marked  destruction  of  the  nerve-elements  at  the  site  of  the  lesion, 
with  widespread  ascending  and  descending  degeneration  in  the  associated 
tracts.  The  local  effects  produced  may  be  referred  in  part  to  the  direct 
influence  of  the  pressure,  but  much  more  to  the  disturbance  of  the  blood 
and  lymph-circulation.  The  degeneration  is  first  manifested  in  the 


MULTIPLE  OR  DISSEMINATED  SCLEROSIS  585 

white  substance,  the  fibers  of  which  swell  up  and  disintegrate,  much 
as  has  been  described  in  the  case  of  transverse  section  of  the  nerves. 
The  axis-cylinders  swell  and  become  varicosed,  and  the  myelin  sheaths 
break  down  into  fat.  The  ganglion  cells  are  somewhat  more  resistant, 
but  ultimately  undergo  vacuolation  and  chromatolysis.  Granular  cells 
appear  early  and  in  considerable  numbers.  In  the  course  of  a  few  dajs 
the  degenerative  changes  may  be  traced  to  the  extremities  of  the  neurones. 
Later,  both  the  fibers  and  their  sheaths  will  have  almost  entirely  dis- 
appeared, although  degenerated  and  varicosed  fibers  may  here  and 

FIG.  159 


Compression  myelitis.     Lumbar  cord.     (From  the  collection  of  Dr.  Colin  K.  Russel  ) 

there  be  seen,  the  number  of  fibers  remaining  being,  of  course,  de- 
pendent on  the  extent  of  the  original  lesion.  The  place  of  the  degener- 
ated fibers  is  taken  up  by  newly-formed  glial  tissue,  which  eventually 
leads  to  contraction  and  sclerosis  of  the  cord.  The  cord  as  a  whole 
shrinks,  becomes  firmer,  and  assumes  a  grayish  color. 

It  should,  perhaps,  be  remarked  that  in  cases  such  as  tuberculosis  of 
the  meninges  the  resulting  lesions  in  the  cord  may,  in  some  instances, 
not  be  entirely  due  to  pressure  and  circulatory  disturbances,  but  to 
inflammation  as  well. 

Multiple  or  Disseminated  Sclerosis. — Multiple  or  disseminated 
sclerosis  (see  p.  562)  is  a  disease  which  affects  the  nervous  system  as 
a  whole.  Not  only  may  the  spinal  cord  be  involved,  but  the  brain  and 
peripheral  nerves.  The  lesions  are  irregularly  distributed,  apparently 
without  much  rhyme  or  reason,  and  may  be  chiefly  localized  in  the 
brain,  or,  again,  in  the  cord. 

In  the  case  of  the  cord,  one  finds  multiple,  grayish  foci,  generally  in 
the  white  substance,  but  also  to  some  extent  in  the  gray  matter,  which 


586  THE  SPINAL  CORD 

may  be  the  size  of  a  pin-head  or  smaller,  or  may  involve  nearly  the  whole 
transverse  thickness  of  the  cord.  These  foci  vary  somewhat  in  appear- 
ance, being  at  one  time  rather  soft  and  gelatinous-looking,  of  grayish- 
white  color,  rather  badly  defined  from  the  normal  tissues;  at  another, 
fairly  firm,  of  a  uniform  gray  color,  and  sharply  differentiated.  Occa- 
sionally, the  lesions  are  to  be  found  in  relation  with  various  bloodvessels. 
Microscopically,  in  these  areas  one  finds  droplets  of  myelin  and  fat, 
fattily  degenerated  cells,  granule  cells,  and  detritus.  The  vessels  com- 
monly present  hyaline  thickening  of  their  walls,  and  there  may  be 
accumulations  of  round  cells  in  the  perivascular  lymph-spaces.  The 
glia  invariably  shows  proliferation,  which  may  be  extensive,  amounting 
to  definite  sclerosis. 

FIG.  160 


Disseminated  sclerosis.     The  cord  shows  irregularly  distributed  patches  of  degeneration. 
(From  the  collection  of  Dr.  Colin  K.  Russel.) 

The  etiology  of  the  disease  is  obscure.  The  affection  of  the  vessels 
might  suggest  a  vascular  origin,  such  as  a  circulating  toxin  or  infection, 
or,  again,  an  ischemic  necrosis.  From  the  histological  appearance  of 
the  lesions,  Ziegler  would  recognize  two  varieties,  secondary  multiple 
sclerosis,  which  results  from  a  previous  focal  degeneration  or  inflamma- 
tion, and  primary  multiple  sclerosis,  due  to  a  pathological  hyperplasia 
of  the  glia,  akin  to  what  occurs  in  syringomyelia,  and  probably  referable 
to  some  error  in  development.  The  latter  type  affects  chiefly  the  pos- 
terior columns  and  the  neighborhood  of  the  ventricles,  and  is  character- 
ized by  a  peculiarly  dense  overgrowth  of  the  glial  substance,  in  which 
may  be  found  scattered  nerve  fibers  that  present  little  or  no  degeneration. 

Chronic  Anterior  Poliomyelitis. — In  some  instances  the  ganglion  cells 
of  the  ventral  cornua,  together  with  the  peripheral  motor  neurones, 
undergo  degeneration.  Chronic  anterior  poliomyelitis  may  be  taken  as 
the  type. 

This  disease  is  similar  in  its  clinical  features  to  the  acute  anterior 
poliomyelitis  described  before  (p.  576),  save  that  it  begins  insidiously  and 


LATERAL  SCLEROSIS  587 

runs  a  chronic  or  subchronic  course.  Some  cases  are  believed  to  have 
an  inflammatory  basis,  and  the  histological  appearances  are  strictly 
comparable  to  those  found  in  the  acute  form.  In  other  cases  the  affec- 
tion seems  to  be  a  pure  degeneration,  the  lesions  being  atrophy  of  the 
cells  of  the  ventral  horns,  with  slight  interstitial  changes  in  the  white 
substance,  degeneration  of  the  peripheral  motor  neurones,  and  wasting 
of  the  muscles  supplied  by  them. 

Progressive  Bulbar  Paralysis. — Analogous  to  this  in  all  respects 
is  the  progressive  bulbar  paralysis,  or  glossolabiolaryngeal  paralysis,  in 
which  the  motor  nuclei  of  the  medulla,  usually  the  nuclei  of  the  hypo- 
glossal,  vagus,  accessorius,  facial,  and  glossopharyngeal  nerves,  are 
involved.  There  may  or  may  not  be  degeneration  of  the  pyramidal 
tracts.  When  degeneration  in  this  situation  is  present  we  have  really  an 
amyotrophic  lateral  sclerosis.  The  nature  of  the  disease  is  obscure. 
Sometimes  it  shows  a  familial  distribution.  It  is  most  probably  an 
abiotrophic  condition. 

Progressive  Spinal  Muscular  Atrophy. — Closely  resembling  chronic 
anterior  poliomyelitis,  clinically  as  well  as  anatomically,  is  the  disease 
known  as  progressive  spinal  muscular  atrophy  of  the  Aran-Duchenne 
type.  Here,  also,  there  is  atrophy  of  the  ganglion  cells  of  the  ventral 
horns,  with  degeneration  of  the  peripheral  motor  nerve-fibers  and 
the  corresponding  muscles.  There  are,  however,  in  addition,  more 
or  less  marked  changes  in  the  white  substance  of  the  cord,  notably  the 
pyramidal  tracts  and  the  anterolateral  ground-bundle.  The  ganglia 
are  found  in  various  stages  of  atrophy,  or  may  have  disappeared.  The 
anterior  horns,  as  a  whole,  do  not  seem  to  shrink,  as  is  the  case  in  chronic 
poliomyelitis,  but  are  transformed  into  a  fine  reticulum,  containing 
large  numbers  of  spindle-shaped  cells.  The  anterior  roots  are  wasted, 
and  many  of  the  fibers  forming  the  peripheral  nerve-trunks  are  partially 
or  completely  degenerated.  According  to  Gowers,  the  pyramidal  tracts 
are  invariably  involved  to  some  extent.  In  the  most  severe  cases  the 
degeneration  can  be  traced  upward  as  far  as  the  motor  cortex. 

The  affected  muscles  show  simple  atrophy,  or  fatty  or  vitreous  degener- 
ation. The  muscle  nuclei  are  often  increased,  and  there  may  be  increase 
of  the  interstitial  connective  tissue. 

The  process  begins  in  the  cervical  region.  The  muscles  affected 
are  first  those  of  the  thenar  and  hypothenar  eminences,  the  lumbricales 
and  interossei,  later  those  of  the  forearm  and  shoulder.  Besides  this, 
the  more  usual  Aran-Duchenne  type,  there  are  other  forms,  notably 
one  in  which  the  wasting  begins  in  the  lower  extremities. 

The  cause  of  the  disease  is  obscure.  From  the  now  well-recognized 
fact  that  certain  mineral  substances,  such  as  lead,  and  bacterial  toxins, 
like  that  of  diphtheria,  are  occasionally  productive  of  degenerative  changes 
in  the  motor  nuclei  and  peripheral  nerves,  it  may  be  inferred  that  circu- 
lating poisons,  either  exogenous  or,  perhaps,  of  a  metabolic  nature,  are 
at  work. 

Lateral  Sclerosis. — Degeneration  in  the  lateral  or  pyramidal  tracts  is 
commonly  known  as  lateral  sclerosis.  It  may  be  a  descending  degenera- 


588  THE  SPINAL  CORD 

tion,  secondary  to  disease  of  the  ganglion  cells  of  the  motor  cortex  of  the 
brain  or  in  any  part  of  the  upper  motor  neurone  above  the  site  of 
the  lesion,  or  may  be  primary  in  the  lateral  tracts  of  the  cord.  The 
symptoms  are  entirely  motor,  consisting  in  paresis  or  paralysis  of  the 
muscles,  hypertonus,  and  increased  knee-jerks  (spastic  spinal  paralysis). 
There  is  no  muscular  wasting.  Somewhat  similar  phenomena  are  noted 
in  the  so-called  "  compression"  myelitis,  transverse  myelitis,  and  certain 
cases  of  disseminated  sclerosis,  but  here  sensory  symptoms  are  com- 
monly observed,  although  less  marked  than  the  motor  ones.  Primary 
sclerosis  of  the  lateral  tracts  is  quite  rare. 

Posterior  Sclerosis. — Posterior  sclerosis  (locomotor  ataxia;  tabes 
dorsalis)  is,  anatomically  speaking,  a  degeneration  of  the  sensory 
neurones  of  the  posterior  roots  and  posterior  columns  of  the  cord,  with 
less  constant  changes  in  the  spinal  ganglia  and  peripheral  nerves.  The 
disease  process  is  not  confined  to  the  spinal  nervous  mechanism,  but 
involves  the  brain  as  well.  The  sensory  nuclei  and  fibers  in  the 
medulla  are  not  infrequently  attacked,  and  degeneration  of  the  nuclei 
of  the  oculomotor  nerves  and  of  the  optic  nerves  may  often  be 
observed. 

The  symptoms  in  the  first,  or  what  is  known  as  the  preataxic  stage, 
are  chiefly  lancinating  pains  in  various  parts,  usually  the  lower  extremi- 
ties; loss  of  the  knee-jerk;  Argyll-Robertson  pupil;  occasionally,  diplopia, 
myosis,  and  a  "girdle"  sensation.  Later,  we  get,  in  addition,  muscular 
incoordination  (ataxic  stage),  diminution  of  sensibility  to  touch,  pain, 
heat,  and  cold,  sometimes  atrophy  of  the  optic  nerve,  and,  finally,  com- 
plete disability  (paralytic  stage). 

To  obtain  a  proper  conception  of  the  pathological  changes  that  occur 
in  tabes,  it  is  necessary  to  bear  in  mind  certain  peculiarities  in  the 
embryological  development  of  the  cord. 

The  posterior  columns  are  formed  at  a  different  period  from  the  rest  of 
the  cord,  and  are  developed  from  the  posterior  nerve-roots,  and  these 
from  the  spinal  ganglia.  The  posterior  columns  must,  therefore,  be 
regarded  as  ingrowths  into  the  cord  of  fibers  of  exogenous  origin.  There 
are,  howeve^  some  few  fibers  of  endogenous  nature  derived  from  cells 
situated  in  the  gray  matter  of  the  cord. 

It  is  now  well  known  that  the  fibers  composing  certain  tracts  or  nerve 
bundles,  that  are  apparently  homologous,  become  medullated  at  different 
stages  of  embryonic  development.  On  this  basis  Flechsig  and  others 
would  divide  the  posterior  columns  into  the  following  components: 
(1)  An  anterior  or  ventral  root-zone,  next  the  posterior  commissure  and 
gray  matter;  (2)  a  middle  root-zone,  consisting  of  two  sorts  of  fibers, 
known  as  the  fibers  of  the  first  system  and  the  fibers  of  the  second  system 
of  the  middle  root-zone;  (3)  a  middle  zone,  next  the  posterior  fissure  and 
distinct  from  the  column  of  Goll;  and  (4)  a  posterior  zone,  being  the  dorsal 
portion  of  the  posterior  column,  divided  into  a  median  part  and  a  lateral 
part,  the  zone  of  Lissauer.  The  order  in  which  these  various  zones  are 
medullated  is  as  follows:  (1)  The  anterior  root-zone,  and  soon  after 
this  the  middle  zone  and  the  first  system  of  the  middle  root-zone;  (2) 


POSTERIOR  SCLEROSIS  589 

the  column  of  Goll,  the  postero-internal  root-zone,  and  the  second  system 
of  the  middle  root-zone,  all  approximately  about  the  same  time. 

Each  fiber  derived  from  the  posterior  root  divides  after  entering  the 
cord  into  two  parts,  a  long,  ascending  branch  and  a  short,  descending 
branch.  Each  branch  gives  off  collaterals  which  help  to  make  up  the 
posterior  columns.  The  fibers  entering  the  lower  segments  of  the  cord 
pass  into  the  internal  parts  of  the  posterior  columns  to  form  eventually 
the  columns  of  Goll.  They  terminate  in  the  medulla  in  arborizations 
about  the  nucleus  of  Goll's  column.  The  root  fibers  that  enter  the  dorsal 
and  cervical  regions  of  the  cord  run  in  the  other  parts  of  the  posterior 
columns  and  form  their  terminal  arborizations  about  the  nuclei  in  the 
posterior  horns.  The  anterior  root-zone  contains  fibers  derived  directly 
from  the  posterior  roots,  and  commissural  fibers  which  unite  the  gray 
substance  at  different  levels.  The  first  system  of  the  middle  root-zone 
is  composed  of  fibers  from  the  posterior  roots,  which  run  for  a  short 
distance  in  the  posterior  column  and  then  enter  Clarke's  column.  The 
second  system  of  the  middle  root-zone  consists  of  fibers  from  the  poste- 
rior root-zone  which  form  the  column  of  Goll  higher  up.  The  formation 
of  Flechsig's  middle  zone  is  not  yet  settled.  The  postero-external  root 
zone  of  Flechsig  (column  of  Lissauer)  is  composed  of  delicate,  closely 
packed  fibers,  probably  collaterals  from  the  posterior  roots,  which,  after 
running  for  some  distance  in  the  cord,  enter  the  substantia  gelatinosa. 

The  macroscopic  changes  in  the  cord  in  locomotor  ataxia  vary 
according  to  the  extent  of  the  disease.  The  earliest  stage  is,  of  course, 
rarely  seen,  except  in  those  cases  associated  with  general  paresis.  Here 
the  cord  presents  little  or  no  deviation  from  the  normal  save  on  micro- 
scopic examination.  In  a  moderately  advanced  case  the  posterior 
portion  of  the  cord  is  distinctly  shrunken,  firm,  and  of  a  grayish-white 
color.  The  posterior  roots,  as  a  rule,  seem  to  be  somewhat  wasted, 
although  not  invariably  so.  The  other  parts  of  the  cord  are  normal. 
The  pia-arachnoid  is  somewhat  thickened  and  opaque  over  the  dorsal 
aspect  of  the  cord.  The  dura  is  unaltered.  The  cerebrospinal  fluid 
may  be  increased. 

When  stained  by  the  Pal-Weigert  or  other  method  for  myelin  staining, 
marked  abnormalities  can  readily  be  observed  in  the  posterior  columns. 
These  vary  somewhat  in  the  different  regions  of  the  cord.  In  cervical 
tabes,  practically  the  whole  of  the  posterior  columns  may  be  involved. 
As  a  rule,  however,  the  columns  of  Goll  are  affected,  particularly  in  the 
posterior  portion,  or  there  may  be  two  narrow  bands  just  external  to  the 
columns  of  Goll  and  Lissauer.  In  the  dorsal  region  there  are  usually  two 
streaks  of  degeneration  in  Burdach's  columns,  and  the  process  apparently 
tends  to  involve  those  fibers  nearer  the  median  line.  In  the  lumbar 
cord,  the  degeneration  commonly  affects  more  or  less  completely  the 
tracts  of  Lissauer.  The  fact  that  a  portion  of  the  Lissauer's  tract  lies 
ventrally  to  the  posterior  roots  has  given  rise  to  the  erroneous  view  that 
in  tabes  the  lateral  tracts  are  involved  as  well  as  the  posterior  ones,  and 
that,  therefore,  the  disease  is  a  combined  sclerosis.  Embryologically  and 
functionally,  however,  the  external  portion  of  Lissauer's  tract  belongs 


590 


THE  SPINAL  CORD 


Cervical   tabes  dorsalis. 

cord.     (From  the  collection  of  Dr.  Colin  K.  Russel.) 

FIG.  162 


to  the  posterior  column.  Not  infrequently  the  ventral  portion  of  the 
posterior  columns,  or  the  anterior  root-zones  of  Flechsig,  escape.  Owing 
to  proliferation  of  the  ependyma,  the  central  canal  is  often  obliterated. 
Besides  the  changes  just  described,  there  are  others,  less  constant  and 
important,  in  other  parts,  such  as  the  gray  substance  of  the  dorsal  and 

ventral  horns,  and  the  cell 

FIG.  i6i  column    of    Clarke.      The 

cells  here  show  eccentricity 
of  the  nuclei  and  more  or 
less  chromatolysis. 

The  degenerative  changes 
are  much  the  same  as  in 
secondary  degeneration  else- 
where. The  myelin  sheaths 
break  down,  the  axis-cylin- 

section  through  the  cervical  ders  swe11  UP>  become  frag- 
mented, and  disappear. 
To  replace  these  the  glia 
proliferates,  and  there  is  an 
increase  of  connective  tissue, 
derived  from  the  trabeculse 
of  the  pia  extending  into 
the  cord.  The  fibers  which 
enter  the  gray  matter  from 
the  posterior  columns  are 
occasionally  degenerated. 
The  vascular  lesions  are 
never  extreme.  The  vessels 

FIG.  163  in  certain  regions  may  pre- 

sent some  fibrous  thicken- 
ing, especially  of  the  adven- 
titia,  or  may  show  hyaline 
degeneration.  There  may 
be  an  accumulation  of  gran- 
ular cells  in  the  adventitia. 
The  pia  may  show  some 
thickening  and  fibrosis. 

The  changes  occurring  in 
the  spinal  ganglia  have  not 
yet  been  established  beyond 
cavil.  It  may  be  said,  at  all 
events,  that  they  are  rarely 

extreme  and  do  not  account  for  the  extensive  lesions  found  in  the  pos- 
terior columns.  Stroebe  found  the  cells  of  the  ganglia  to  be  shrunken, 
irregular  in  outline,  many  of  them  vacuolated  and  markedly  pigmented. 
In  advanced  tabes,  the  ganglion  cells  had  largely  disappeared,  the  inter- 
capsular  space  was  distended,  and  the  cells  of  the  capsule  were  prolifer- 
ating. The  interstitial  substance  showed  hyperplasia.  The  posterior 
roots  external  to  the  cord  were  markedly  degenerated. 


Tabes  dorsalis.      Dorsal  cord.      (From  the  collection  of 
Dr.  Colin  K.  Russel.) 


Tabes  dorsalis.     Lumbar  cord.     (From  the  collection  of 
Dr.  Colin  K.  Russel.) 


POSTERIOR  SCLEROSIS  591 

The  changes  in  the  peripheral  nerves  are  inconstant  and  have  been 
variously  interpreted.  The  difficulty  is  to  decide  whether  the  degenera- 
tion that  is  sometimes  present  is  primary  or  secondary.  The  small, 
cutaneous  nerves  are  the  ones  usually  picked  out.  The  myelin  sheaths 
are  disintegrated,  the  axis-cylinders  are  swollen,  and  there  is  some  increase 
of  the  interstitial  connective  tissue.  The  cranial  nerves,  particularly 
the  optic,  and  the  sympathetic  fibers  are  similarly  involved. 

Atrophy  of  the  cells  in  the  motor  cortex  of  the  brain  is  sometimes  met 
with. 

The  interpretation  of  the  histological  findings  is,  as  one  can  readily 
understand,  fraught  with  great  difficulty,  and  much  debate  has  taken 
place  as  to  what  constitutes  the  primary  lesion.  It  may,  first  of  all, 
be  taken  as  certain  that  the  degenerative  process  is  essential  to  the  nerve 
fibers  and  is  not  to  be  regarded  as  secondary  to  the  increased  formation 
of  glia.  Nor  is  there  any  evidence  of  a  primary  inflammatory  disturb- 
ance. The  main  views  are  (1)  that  locomotor  ataxia  is  a  primary 
sclerosis  of  the  posterior  colums  of  the  cord,  and  (2)  that  the  changes  in 
the  cord  are  secondary  to  degeneration  of  the  posterior  roots.  The 
latter  is  the  one  supported  by  the  greatest  amount  of  evidence,  and  is 
accepted  by  the  majority  of  neuropathologists  at  the  present  time. 

Dejerine  would  find  the  essential  cause  in  endarteritis  of  the  vessels 
supplying  the  intramedullary  portions  of  the  posterior  roots  with  con- 
secutive fibrosis.  In  refutation  of  this  opinion,  it  may  be  remarked 
that  the  vascular  disturbances  found  in  tabes  are  similar  to  those  found  in 
other  degenerative  diseases  of  the  cord,  and  are  never  a  striking  feature 
in  the  histological  picture.  The  vascular  changes,  moreover,  are  not 
uniformly  distributed,  and  could  hardly  account  for  such  a  marked 
"system"  sclerosis.  The  preponderance  of  evidence  goes  to  show  that 
the  disease  process  begins  in  the  extramedullary  portion  of  the  posterior 
roots.  Some  have  described  certain  anatomical  conditions  that  might 
account  for  this.  Redlich  and  Obersteiner  found  a  thickening  of  the 
pia  and  dense  sclerosis  of  the  peripheral  zones  of  the  neuroglia,  which, 
in  their  opinion,  lead  to  the  degeneration  in  question  by  compression 
of  the  posterior  roots  at  the  point  where  they  enter  the  cord.  The 
medullary  sheaths  are  much  thinner  here  than  at  any  other  part  of  the 
fiber,  which  makes  it  probably  the  least  resistant  point.  This  meningitis, 
it  need  hardly  be  said,  is  not  always  present  in  tabes,  and,  conversely, 
we  do  not  find  the  anatomical  lesions  of  tabes  in  cases  of  meningitis  and 
meningomyelitis. 

It  is  not  impossible,  either,  that  toxins  circulating  in  the  cerebrospinal 
fluid  might  affect  deleteriously  the  nerve  roots  during  their  intrameningeal 
course.  Lesions  in  the  cord,  somewhat  similar  to  those  in  tabes,  have 
been  described  in  connection  with  chronic  ergotism  and  in  pellagra, 
a  disease  lately  shown  to  be  due  to  a  variety  of  aspergillus.  In  ergot 
poisoning,  the  posterior  roots  are  degenerated,  and  also  the  columns  of 
Burdach  in  the  cord.  The  columns  of  Goll  are  not  primarily  affected, 
but  may  be  secondarily  involved  in  advanced  cases.  The  anterior 
root-zone,  the  median^portion  of  the  middle  zone,  and  Lissauer's  tract 


592  THE  SPINAL  CORD 

escape.  In  pellagra,  the  posterior  roots  are  not  involved,  and  Marie, 
on  this  account,  regards  it  as  an  endogenous  disease  of  the  cord.  It  is, 
in  fact,  a  primary  degeneration  of  toxic  origin. 

Oppenheim  believes  that  the  toxic  agent  at  work  in  tabes  dorsalis  has 
a  selective  action  on  the  posterior  spinal  ganglia  and  their  homologues, 
the  Gasserian  and  jugular  ganglia,  etc.  This  toxin  is  just  powerful 
enough  to  cause  atrophy  of  the  distal  portion  of  the  neurone  in  the  pos- 
terior columns  of  the  cord  and  in  the  peripheral  nerves. 

Marie  considers  that  the  disease  is  due  to  a  syphilitic  lesion  involving 
the  lymphatic  channels  of  the  posterior  columns  and  of  the  correspond- 
ing pia-arachnoid,  inasmuch  as  he  has,  in  many  cases,  found  a  cloudiaess 
and  thickening  of  the  pia-arachnoid  on  the  dorsal  aspect  of  the  cord. 

When  such  divergent  views  are  expressed  as  to  the  exact  meaning  of 
the  anatomical  changes  in  tabes,  it  is  not  surprising  that  the  etiology 
of  the  disease  is  not  altogether  clear.  It  seems  fairly  certain  that  tabes 
is  not  a  disease  of  the  cord  alone,  but  of  the  whole  nervous  system,  involv- 
ing the  sensory  neurones,  and,  probably,  of  exogenous  origin.  We  must 
bear  this  in  mind,  therefore,  when  searching  for  the  cause.  Clinicians 
usually  attribute  the  disease  to  syphilis,  overwork,  traumatism,  exposure 
to  cold,  or  sexual  excess.  It  is  a  fact,  as  Erb  and  many  others  have 
pointed  out,  that  the  majority  of  tabetic  patients  (from  50  to  90  per  cent.) 
give  a  history  or  present  signs  of  previous  syphilis.  Recently,  Wasser- 
mann  has  shown  that  his  precipitation  test,  which  is  positive  in  active 
syphilis,  is  also  positive  in  tabes  dorsalis.  This  is  highly  significant. 
It  cannot  be  denied,  however,  that  the  affection  occurs  in  those  who 
have  never  had  syphilis.  Tabes,  moreover,  rarely  comes  on  during 
the  active  stages  of  syphilis,  being  usually  found  from  five  to  ten  or  fifteen 
years,  or  even  longer,  after  infection.  It  is  a  matter  of  common  observa- 
tion, too,  that  antisyphilitic  remedies  have  little  or  no  effect  on  the  course 
of  the  disease.  If,  then,  we  admit  the  importance  of  syphilis,  as  we 
needs  must,  we  have  to  regard  tabes  as  a  parasyphilitic  affection,  rather 
than  as  due  directly  to  the  infective  agent  of  syphilis.  We  must  hold, 
therefore,  with  Obersteiner,  who  assumes  a  multiple  causation  for  tabes, 
in  which  syphilis  is  the  most  frequent  and  important  single  cause. 

Perhaps,  the  view  that  fits  in  most  perfectly  with  the  observed  facts  is 
that  of  Edinger,1  the  so-called  "  exhaustion  theory."  This  is  based  on 
the  well-known  idea  of  Weigert  and  Roux,  that  the  constituent  tissues 
of  an  organ  are  normally  in  a  state  of  equilibrium,  so  correlated  one  to 
another  that  no  cell  can  disappear  without  its  place  being  taken  by 
hyperplasia  of  the  surrounding  tissue,  and  when  one  constituent  becomes 
weaker  or  less  resistant,  the  energy  of  growth  of  its  neighbors  tends  to 
repress  it  still  farther.  According  to  this  conception,  cirrhosis  of  the 
liver  is  primarily  a  degeneration  of  the  parenchymatous  cells  with  a 
secondary  overgrowth  of  the  interstitial  tissue.  The  gliosis  that  occurs 
in  the  spinal  cord  of  an  old  hemiplegic  case  can  be  explained  in  the 
same  way,  and  when  a  cell  or  fiber,  or  even  a  whole  neurone,  becomes 

1  Deut,  med.  Woch.,  1904: 1633,  1800,  and  1921;  1905:  4  and  135. 


POSTERIOR  SCLEROSIS  593 

so  weak  that  it  is  unable  to  hold  in  check  the  proliferative  capacity  of  the 
neighboring  tissue,  we  must  expect  to  find  the  same  process  going  on. 

Further,  function  involves  breaking  down  of  the  active  tissue.  Nor- 
mally, the  destructive  process  is  compensated  by  a  sufficient  supply 
of  nutrition,  so  that  the  production  of  living  substance  is  constant. 
If  this  does  not  take  place,  the  normal  equilibrium  of  the  parts  is  dis- 
turbed and  a  progressive  degeneration  is  the  result.  Edinger  applies 
this  to  the  nervous  system.  He  assumes  that  if  the  supply  of  nutrition 
be  deficient,  or  if,  though  it  be  normal,  excessive  function  be  demanded 
of  the  cell,  that  is  to  say,  if  the  normal  relation  between  combustion  and 
repair  be  disturbed,  either  by  relative  or  by  absolute  suprafunction,  the 
energy  of  growth  of  the  resting  tissue  will  lead  to  a  degeneration  of  the 
less  resistant  active  parts,  a  result  that  will  occur  the  more  easily  if  both 
factors  be  at  work.  As  examples  of  this,  Edinger  cites  the  hammer 
palsy  of  smiths  and  the  atrophic  paralysis  of  the  forearm  muscles  in 
drummer  boys,  which,  according  to  him,  occur  most  frequently  or  only 
in  badly-nourished  subjects. 

To  apply  this  to  tabes.  The  reparative  processes  in  the  specific 
cells  are  impaired,  as  a  result  of  some  toxin  circulating  in  the  system, 
this  toxin  in  most  cases  being  syphilitic  in  origin.  The  neurones  which 
are  normally  most  active,  or  are  most  constantly  at  work,  are  those  which 
suffer.  These  are  the  sensory  nerves  from  the  muscles  which  play  an 
important  role  in  the  regulation  of  muscular  contraction,  and  are  con- 
stantly submitting  those  stimuli  by  which  we  become  aware  of  the 
condition  of  our  muscular  system  and  the  position  of  our  limbs.  Clin- 
ically, this  defect  is  manifested  in  the  loss  of  muscular  tone  and  the  sense 
of  position,  and  the  consequent  ataxia.  Secondly,  the  purely  sensory 
nerves  which  are  constantly  submitting  sensations  from  the  skin  and 
mucous  membranes  would  be  likely  to  suffer  and  give  rise  to  sensory 
disturbances.  Thirdly,  the  eyes  would  suffer,  and  above  all  the  con- 
strictor of  the  iris,  so  constantly  active  in  the  reflex  contraction  of  the 
iris  to  light,  which  must  be  almost  constantly  at  work  in  comparison 
with  the  reflex  for  accommodation.  In  this  way  the  Argyll-Robertson 
pupil  can  be  explained.  The  paralysis  of  the  external  ocular  muscles, 
the  bladder  disturbance,  the  occasional  atrophic  muscular  palsies,  may 
all  be  explained  in  terms  of  this  theory. 

The  peripheral  motor  neurones,  which  are  normally  capable  of  re- 
sponding to  two  sets  of  stimuli,  those  from  the  upper  motor  neurone, 
which  are  relatively  seldom  at  work,  and  those  from  the  peripheral  sensory 
neurone,  keeping  up  the  tone  of  the  muscles,  have  the  opportunity  to 
repair  when  they  are  not  at  the  service  of  voluntary  impulses.  They, 
therefore,  would  not  be  so  likely  to  become  exhausted  as  the  sensory 
neurones.  When,  too,  the  sensory  neurones  have  degenerated  and  they 
do  not  get  stimuli  from  them,  they  have  still  more  time  to  repair,  though 
this  is  probably  in  part  counterbalanced  by  the  excessive  energy  that 
an  ataxic  patient  puts  into  any  voluntary  movement. 

In  this  way  Edinger  escapes  the  dilemma  of  believing  that  every 
person  having  tabes  must  be  syphilitic.  On  this  conception  we  can  see 
38 


594  THE  SPINAL  CORD 

how  trauma,  exposure  to  cold  and  wet,  and  excesses  of  various  kinds 
may  act  as  predisposing  causes  in  bringing  about  depreciation  of  the 
nerve  unit.  The  fact  that  tabes  is  more  common  in  men  than  in  women 
becomes  explainable,  also,  men  being  more  exposed  to  those  deleterious 
influences  which  Edinger  brings  into  the  etiology  of  the  disease. 

Besides  the  degenerative  diseases  of  the  cord  hitherto  described,  which 
affect  the  nerve  elements  of  single  tracts,  there  are  others  in  which  several 
neurone  groups  of  differing  function  are  involved.  These  are  the  so- 
called  "  combined  system  diseases,"  of  which  posterolateral  sclerosis, 
Friedreich's  ataxia,  and  amyotrophic  lateral  sclerosis  are  the  most  prom- 
inent members. 

Posterolateral  Sclerosis. — Posterolateral  sclerosis,  or  combined  scle- 
rosis, the  ataxic  paraplegia  of  the  clinicians,  is  characterized  by  sclerosis 
of  the  posterior  and  lateral  tracts.  In  the  posterior  tracts,  the  postero- 
internal  and  the  dorsal  portion  of  the  postero-external  columns  are  the 
regions  specially  picked  out.  In  the  lateral  tracts  it  is  usually  the 
crossed  pyramidal  tracts,  but  not  infrequently  the  direct  cerebellar  tracts, 
the  columns  of  Gowers,  and  the  lateral  limiting  layers.  Collins  has 
recorded  also  degeneration  of  the  cells  of  Clarke's  column  and  of  the 
fine,  white  fibers  of  the  anterior  horns. 

The  disease  appears  to  be  a  primary  one,  but  the  exact  pathogeny 
is  not  known.  Many  consider  it  a  true  combined  "system"  disease. 
Others  think  that  the  parts  affected,  owing  to  their  comparatively  poor 
blood  supply,  are  less  able  than  other  parts  to  withstand  the  deleterious 
action  of  the  morbific  agent. 

The  disease  occasionally  follows  exposure  to  cold.  Syphilis  plays  an 
unimportant  role  in  the  etiology.  The  main  clinical  features  are  ataxia, 
muscular  weakness,  hypertonus,  and  gradually  increasing  rigidity. 
Sensory  symptoms  are  rare  and  trifling.  In  advanced  cases,  owing  to 
cerebral  involvement,  the  disease  may  resemble  general  paresis. 

Friedreich's  Ataxia. — Friedreich's  ataxia,  or  hereditary  ataxia,  is  a 
curious  disease  first  described  by  Friedreich1  in  1861 .  It  is  distinctly 
a  familial  disease,  but  may  or  may  not  be  hereditary.  The  great  majority 
of  the  cases  are  met  with  in  children  before  the  age  of  puberty. 

Clinically,  the  affection  is  characterized  by  ataxia  o'f  a  swaying  or 
staggering  character  involving  all  four  extremities,  nystagmus,  scanning 
speech,  and  muscular  contractions,  giving  rise  to  scoliosis  and  talipes 
equinus.  The  knee-jerks  are  usually  absent. 

The  cord  is  found  to  be  of  less  than  normal  thickness,  a  condition  of 
things  which  is  most  marked  in  the  cervical  and  upper  dorsal  region. 
Microscopically,  the  lesions  are  found  chiefly  in  the  posterior  and  lateral 
columns.  The  columns  of  Goll,  the  pyramidal  tracts,  and  Clarke's 
columns  are  extensively  involved,  less  so  Burdach's  and  Gowers'  tracts 
and  the  direct  pyramidal  tracts.  There  may,  in  some  cases,  be  atrophy 
of  the  posterior  roots  and  peripheral  nerves.  The  lesions  consist  in 
degeneration  and  atrophy  of  the  nerve  fibers  and  their  myelin  sheaths, 

1  Virch.  Archiv,  70,  1877:  140. 


HEREDITARY   CEREBELLAR  ATAXIA  595 

together  with  increase  of  the  neuroglia.  The  pia  is  somewhat  thickened, 
especially  over  the  posterior  aspect  of  the  cord,  which  probably  accounts 
for  the  peripheral  or  annular  degeneration  present  in  some  cases.  The 
bloodvessels  are  slightly  thickened,  but  the  vascular  phenomena  are  not 
obtrusive.  Dana  has  recently  observed  a  peculiar  porosis  of  the  cord, 
both  of  the  white  and  gray  substance,  which  is  due  to  dilatation  of  the 
peri  vascular  spaces. 

Many  different  opinions  have  been  expressed  as  to  the  nature  of  the 
disease.  The  fact  that  the  affection  is  most  frequently  met  with  in 
childhood,  and  is,  moreover,  apt  to  run  in  families,  suggests  that  some 
anomaly  of  development  is  at  fault.  Some  have  held  that  this  consists 
in  hypoplasia  of  the  third  primary  vesicle  and  neural  canal,  whereby 
the  cerebellum,  medulla,  and  cord  lag  behind  in  their  development. 
Dejerine  and  Letulle  have  suggested  as  the  cause  a  primary  gliosis  of 
developmental  nature  in  the  posterior  columns.  Others  consider  the 
degeneration  of  the  nerve  elements  to  be  primary  and  the  sclerosis  sec- 
ondary. Senator's  idea  that  the  essential  lesion  is  atrophy  or  hypoplasia 
of  the  cerebellum  has  not  been  widely  accepted. 

FIG.  164 


Friedreich's  ataxia.     Lumbar  cord.     (From  the  collection  of  Dr.  Colin  K.  Russel.) 

Hereditary  Cerebellar  Ataxia. — Somewhat  similar  to  the  hereditary  ataxia 
of  spinal  origin,  just  described,  is  what  is  known  as  hereditary  cerebellar 
ataxia  (cerebellar  heredo-ataxia  of  Marie). 

In  this  affection,  which  comes  on  somewhat  later  in  life,  the  ataxia 
is  less  marked,  and  there  is  no  scoliosis  or  club-foot.  Atrophy  of  the 
optic  nerves  is  frequent  and  the  knee-jerks  are  increased.  Spasm  of 
the  muscles  comes  on  quite  late.  The  most  striking  lesion  found  is 
hypoplasia  of  the  cerebellum.  The  posterior  and  lateral  tracts  are 
not  involved.  At  most  there  is  atrophy  of  the  anterior  and  posterior 
nerve-roots.  The  spinal  cord,  however,  is  somewhat  smaller  than 
normal.  Transitional  forms  between  hereditary  cerebellar  ataxia  and 
hereditary  spinal  ataxia  have  been  described. 

The  influence  of  toxic  substances  in  bringing  about  degeneration  is 
well  illustrated  by  the  spinal  lesions  which  occasionally  accompany 


598  THE  SPINAL  CORD 

such  diseases  as  pernicious  anemia,  tuberculosis,  diabetes,  and  carci- 
noma. The  first-named  condition  may  be  taken  as  the  type,  the  others 
being  similar  save  that  they  are  not  so  extreme. 

Pernicious  Anemia. — The  lesions  in  pernicious  anemia  are  most 
commonly  found  in  the  posterior  and  lateral  columns  of  the  cord,  less 
frequently  in  the  anterior.  The  posterior  columns  are  more  extensively 
and  uniformly  affected  than  are  the  others.  In  the  early  stages,  the  de- 
generation is  systemic,  but  as  the  disease  progresses  the  lesions  become 
more  irregular  and  extensive.  Annular  sclerosis  may  be  found,  or 
multiple  scattered  foci  not  unlike  those  in  disseminated  sclerosis. 
The  lesions  in  the  posterior  columns  are  those  of  ascending  degeneration 
in  the  cervical  and  upper  dorsal  regions.  The  postero-internal  columns 
are  much  more  markedly  involved  than  are  the  postero-external.  The 
ventral  portion  of  the  postero-external  columns,  the  Lissauer's  tracts, 
and  the  posterior  nerve-roots  escape,  constituting  a  marked  difference 
between  this  form  of  degeneration  and  tabes  dorsalis.  In  the  dissemi- 
nated form,  the  lesions  differ  from  those  of  disseminated  sclerosis  in  that 
in  the  latter  certain  nerve-fibers  within  the  sclerotic  areas  are  to  some 
extent  at  least  preserved. 

In  the  early  stages  the  medullary  sheaths  are  swollen  and  stain  badly, 
but  the  axis-cylinders  may  be  fairly  well  preserved.  Later,  the  axis- 
cylinders  are  degenerated  or  have  entirely  disappeared,  leaving  small 
cavities  in  the  myelin  sheaths  in  which  they  formerly  lay.  The  vessels 
are  usually  not  much  altered.  In  the  most  advanced  cases  the  connective 
tissue  is  considerably  increased,  the  walls  of  the  smaller  vessels  are 
thickened,  and  there  is  proliferation  of  the  cells  of  the  adventitia. 

The  exact  pathogenesis  of  the  degeneration  is  unknown.  It  may 
be  that  the  important  element  is  the  lack  of  nutrition  due  to  the  destruc- 
tion of  the  blood  corpuscles.  Most  observers  seem  to  think  that  this 
is  not  the  case,  but  that  the  degeneration  of  the  nerve  fibers  and  of  the 
blood  cells  is  referable  to  the  same  primary  cause. 

Amyotrophic  Lateral  Sclerosis.— The  last,  and  one  of  the  most 
important,  system  diseases  with  which  we  shall  deal  is  amyotrophic 
lateral  sclerosis  (Charcot).  This  disease  is  one  of  the  entire  motor 
system,  and,  anatomically,  presents  the  combined  lesions  of  lateral 
sclerosis  and  progressive  spinal  muscular  atrophy.  The  pathological 
changes  are  found  especially  in  the  cord  and  peripheral  motor  nerves, 
but  extend  frequently  to  the  medulla,  and  in  some  instances  to  the  internal 
capsule  and  motor  cortex  of  the  brain. 

Clinically,  the  disease  is  characterized  by  muscular  wasting,  a  variable 
amount  of  spasm,  increased  knee-jerks,  with,  in  some  cases,  evidences 
of  involvement  of  the  motor  nuclei  of  the  medulla  (glossolabiolaryngeal 
paralysis),  and  occasionally  tremors. 

The  cord  is  firm  and  somewhat  wasted  looking,  particularly  in  the 
cervical  region.  On  section,  the  lateral  pyramidal  tracts  are  grayer  than 
the  rest  of  the  white  substance,  and  the  gray  matter  is  somewhat  softened 
and  reddened.  Analogous  changes,  though  less  extensive,  are  sometimes 
to  be  observed  in  the  medulla.  The  anterior  nerve-roots,  and  often 


AMYOTROPHIC  LATERAL  SCLEROSIS 


597 


the  hypoglossal  and  glossopharyngeal  nerves,  are  atrophied.  The  cor- 
responding muscles  are  atrophic,  and  there  may  be  deformities  and 
fixation  of  the  joints. 


Fio.  165 


Amyotrophic  lateral  sclerosis.     Cervical  cord.     (From  the  collection  of  Dr.  Colin  K.  Russel.) 

FIG.  166 


Amyotrophic  lateral  sclerosis.     Degeneration  is  evident  in  all  parts  of  the  cord  save  the 
posterior  columns.     (From  the  collection  of  Dr.  Colin  K.  Russel.) 

Microscopically,  as  well  as  to  gross  appearance,  it  is  in  many  cases 
evident  that  the  most  marked  changes  are  in  the  lateral  tracts.  There  is 
more  or  less  marked  degeneration  of  the  direct  and  crossed  pyramidal 


598  THE  SPINAL  CORD 

tracts.  In  some  instances,  the  anterolateral  column,  the  lateral  limiting 
layer,  and  those  tracts  extending  from  the  gray  matter  of  the  ventral 
horns  to  the  surface  of  the  cord  are  involved.  Degeneration  in  the 
posterior  columns,  especially  the  columns  of  Goll,  has  been  noted  by 
Hektoen,  Marie,  and  others.  It  must  be  remarked  that,  while  the 
degeneration  of  the  lateral  columns  is  very  striking,  when  present,  it 
is  the  most  inconstant  of  the  lesions.  On  the  other  hand,  wasting  of  the 
motor  ganglia  of  the  ventral  horns,  or  of  their  homologues,  is  invariably 
found.  The  substance  of  the  ventral  horns  does  not  present  much 
shrinkage,  but  the  ganglia  are  greatly  affected.  They  are  not  only 
diminished  in  number,  but  those  that  remain  are  wasted,  stain  irreg- 
ularly or  more  diffusely  than  normal,  and  the  nuclei  may  have 
disappeared.  The  protoplasmic  processes  are  atrophied  or  absent.  In 

FIG.  167 


Amyotrophic  lateral  sclerosis.     Lumbar  cord.     All  parts  are  degenerated  to  some  extent 
except  the  posterior  columns.     (From  the  collection  of  Dr.  Colin  K.  Russel.) 

some  preparations  the  ganglia  may  have  dropped  out,  giving  the  horns 
a  somewhat  porous  appearance.  The  bloodvessels  are  more  or  less 
thickened,  the  perivascular  lymphatics  are  dilated,  and  there  may  be 
minute  hemorrhages  here  and  there.  The  changes  in  the  fibers  of  the 
pyramidal  tracts  are  those  that  have  so  often  been  described  before. 
The  glia  is  but  slightly  increased. 

The  involvement  of  the  medulla  is  not  so  extreme  as  that  of  the  cord. 
The  nucleus  of  the  hypoglossal  nerve  and  the  nucleus  of  Roller  are 
degenerated,  somewhat  rarely  the  motor  nucleus  of  the  fifth  nerve  and 
the  posterior  nucleus  of  the  vagus.  The  pyramidal  tracts  in  this  region 
are  only  slightly  involved. 

The  degeneration  of  the  pyramidal  tracts  has  been  in  some  cases  fol- 
lowed into  the  brain.  The  large  pyramidal  cells  of  the  motor  cortex  and 
the  tangential  fibers  have  been  found  to  be  degenerated.  Some  of  the 


TUMORS  599 

cells  have  disappeared.  In  the  deeper  layers  of  the  cortex  the  cells  may 
he  atrophied,  while  the  tangential  fibers  are  unaffected. 

The  degeneration  of  the  peripheral  motor  fibers  is  slight,  not  nearly  so 
marked  as  that  of  the  anterior  roots. 

The  muscles  corresponding  to  the  affected  ganglia  are  atrophied,  while 
there  is  an  interstitial  lipomatosis. 

Two  main  theories  have  been  advanced  to  explain  the  process.  The 
original  view  of  Charcot,  in  which  he  has  been  followed  by  Erb,  is  that 
aniyotrophic  lateral  sclerosis  is  a  system  disease,  the  main  lesion  being 
situated  in  the  pyramidal  tracts,  the  changes  in  the  gray  substance  and 
peripheral  nerves  being  secondary.  Gowers,  v.  Leyden,  Dana,  and 
others  regard  the  disease  as  a  form  of  progressive  muscular  atrophy. 
The  affection  of  the  peripheral  motor  neurone  is  primary,  that  of  the 
central  motor  neurone  is  secondary  or  associated.  The  question  cannot 
be  considered  as  settled.  There  is,  however,  reason  to  suppose,  from 
the  clinical  course  of  some  cases  and  the  study  of  the  cord  in  certain 
anomalous  forms,  that  the  degeneration  of  the  pyramidal  tracts  may 
antedate  the  changes  in  the  ganglion  cells  of  the  ventral  horns  and 
the  peripheral  motor  neurones.  Possibly,  aniyotrophic  lateral  sclerosis 
is  one  of  the  diseases  to  which  Edinger's  "exhaustion"  theory  would 
apply. 

PROGRESSIVE  METAMORPHOSES. 

Tumors. — The  most  frequent  and  important  new-growth  found  in 
the  spinal  cord  is  the  glioma.  This  rarely  forms  a  circumscribed  tumor, 
but  is  usually  met  with  as  a  diffuse  proliferation  of  glial  tissue  (glioma- 
tosis)  in  the  neighborhood  of  the  central  canal,  often  extending  for  a 
considerable  distance  along  the  cord.  At  times  it  is  composed  of  firm 
glia,  and  at  others,  of  a  more  delicate,  gelatinous,  or  mucoid  glia  (myxo- 
glioma).  Occasionally  it  is  markedly  vascular  (glioma  telangiectaticum). 
The  newly-formed  glia  seems  particularly  prone  to  softening  and  degener- 
ation, and  cavities  are  not  infrequently  produced  which,  if  at  all  exten- 
sive, lead  to  the  conditions  known  as  hydromyelia  and  syringomyelia. 
Doubtless  many  cases  of  diffuse  gliomatosis  of  this  type  are  to  be  attrib- 
uted to  errors  of  development;  others  are  more  akin  to  new-growths. 

Fibromas,  sarcomas,  and  angiosarcomas  are  rare.  Multiple  fibromas 
of  the  cord  are  sometimes  associated  with  fibromas  of  the  peripheral 
nerves. 

Secondary  tumors  are  carcinomas,  sarcomas,  and  myelomas. 


CHAPTER    XXVIII. 

THE  PERIPHERAL  NERVES. 

THE  peripheral  nerve-mechanism  consists  of  three  parts,  the  ganglia, 
the  nerve  trunks,  and  the  end  plates.  The  nerve  trunks,  which  will 
concern  us  most,  are  composed  of  medullated  fibers,  continuous  with 
the  central  nervous  system,  and  of  non-medullated  fibers,  derived  from 
the  sympathetic  ganglia. 

CIRCULATORY  DISTURBANCES. 

Anemia. — Anemia  can  be  recognized  with  difficulty,  but  may 
be  supposed  to  be  present  in  cases  of  general  systemic  anemia,  and, 
locally,  in  obstruction  of  the  nutrient  vessels,  and  from  the  pressure  of 
tumors  or  dislocated  bones. 

Hyperemia. — Hyperemia  is  met  with  in  cases  of  inflammation. 

(Edema. — (Edema  is  rare.  It  may  sometimes  be  observed  where 
nerves  pass  through  inflammatory  foci. 

Hemorrhages. — Hemorrhages,  usually  petechial  in  character,  are 
found  in  inflammation  and  traumatism. 


INFLAMMATIONS. 

Neuritis. — Inflammation  of  the  peripheral  nerves,  or  neuritis,  is 
due  to  circulating  toxins  or  bacteria,  to  trauma,  or  to  the  extension 
of  inflammation  from  adjacent  parts.  It  has  been  customary  with 
some  writers  to  distinguish  a  parenchymatous  neuritis,  in  which  the 
primary  lesion  is  degeneration  of  the  nerve-fibers,  and  an  interstitial 
neuritis,  in  which  the  changes  begin  in  the  connective-tissue  portion  of 
the  nerve  trunk.  It  may  be  again  remarked  here  that  degeneration 
and  true  inflammation  of  the  nerve-substance  cannot  always  be  differ- 
entiated. For,  atrophy  and  degenerative  changes  in  the  nerve-fibers 
may  be  followed  by  reactive  inflammation  in  the  interstitial  substance 
of  a  secondary  nature,  while  primary  interstitial  inflammation  quickly 
leads  to  secondary  wasting  of  the  specific  nerve-elements.  In  whatever 
way  the  condition  may  be  initiated,  degeneration  of  the  nerve-trunks 
leads  to  well-defined  results,  and  all  such  conditions  are  grouped  indis- 
criminately by  the  clinicians  under  the  term  neuritis.  Primary  paren- 
chymatous neuritis  is  practically  the  same  thing  as  atrophy  of  the  nerve 
fibers,  and  is  dealt  with  more  precisely  under  the  "Degenerations"  later 


INTERSTITIAL  NEURITIS 


601 


FIG.  168 


(p.  603).  In  its  causation,  alcohol,  lead,  arsenic,  and  the  toxins  of  the 
various  infective  diseases  play  a  leading  part.  The  disease,  endemic  in 
Japan  and  the  Orient,  known  as  Beri-beri  or  "Kakke"  probably  comes 
into  this  category. 

Acute  Interstitial  Neuritis. — Acute  interstitial  neuritis,  or  neuritis,  in 
its  restricted  sense,  is  hematogenic  or  lymphogenic  in  origin,  and  may 
be  produced  by  various  infective  agents 
or  toxins.  The  affected  nerve-trunk  is 
swollen,  oedematous,  and  hyperemic, 
with,  sometimes,  minute  hemorrhages 
into  the  substance.  Microscopically, 
we  find  in  the  endoneurium  and  epi- 
neurium  all  the  ordinary  signs  of 
inflammation.  The  vessels  are  con- 
gested, the  interstitial  substance  is  in- 
filtrated with  serum  and  inflammatory 
leukocytes.  If  the  process  have  gone 
on  for  some  time,  it  is  common  to  find 
the  ordinary  degenerative  manifesta- 
tions in  the  axis-cylinders  and  myelin 
sheaths.  In  suppurative  cases  small 
abscesses  may  be  found  here  and  there 
in  the  interstitial  substance.  Neuritis 
may  also  arise  by  direct  involvement 
or  lymphogenous  extension,  as  in  those 
cases  where  an  abscess  or  infective 
granuloma  has  formed  in  the  neighbor- 
hood of  a  nerve-trunk,  or  where  men- 
ingitis involves  secondarily  the  cranial 
nerves  or  the  roots  of  the  spinal 
nerves. 

Traumatic  neuritis  arises  from  sec- 
tion of  a  nerve  by  accident  or  design, 
especially  where  the  wound  has  be- 
come infected,  or  from  contusions  or 
lacerations. 

Slight  grades  of  neuritis  heal  with- 
out causing  any  permanent  damage. 
More  severe  forms  may  lead  to  degen- 
eration of  the  nerve  elements,  with 
consecutive  atrophy  of  the  associated 
muscles,  or  to  actual  necrosis  or  gan- 
grene of  the  nerve  trunk. 

Chronic  Interstitial  Neuritis. — Chronic  interstitial  neuritis  arises  by 
hematogenic  or  lymphogenic  infection  or  intoxication,  and  by  the 
extension  of  chronic  inflammation  from  neighboring  parts.  It  may 
also  occur  without  obvious  cause.  Microscopically,  the  connective-tissue 
cells  have  markedly  proliferated,  and  the  stroma  may  show  collections 


Multiple  peripheral  neuritis  (wrist  and 
foot-drop)  in  chronic  lead  poisoning. 
(From  the  Medical  Clinic  of  the  Mont- 
real General  Hospital.) 


602  THE  PERIPHERAL  NERVES 

of  small  round  cells  (neuritis  prolifera).  This  overgrowth  of  the  inter- 
stitial substance  may  be  so  great  that  the  nerve  trunk  is  considerably 
enlarged.  Sooner  or  later,  the  nerve-fibers  atrophy  and  disappear. 
Dejerine  has  drawn  special  attention  to  this  form  under  the  name 
chronic  hypertrophic  neuritis 

Neuritis,  especially  those  varieties  due  to  hematogenic  infection  and 
intoxications,  is  apt  to  be  symmetrical.  It  affects  a  number  of  nerve 
trunks  (multiple  peripheral  neuritis  or  polyneuritis),  and,  moreover, 
the  toxins  are  liable  to  single  out  particular  regions.  Where  extensive 
degeneration  has  taken  place  we  may,  in  some  cases,  find  an  ascending 
degeneration  involving  the  posterior  nerve  roots,  the  posterior  columns 
of  the  cord,  or  even  the  nutrient  centres.  The  condition  leads  to 
paralysis  and  wasting  of  the  muscles  innervated. 

Tuberculosis. — This  is  probably  always  secondary,  and  is  found  most 
commonly  in  the  roots  of  the  cranial  or  spinal  nerves,  as  a  result  of  the 
extension  of  a  tuberculous  meningitis.  Occasionally,  nerve-fibers  are 
implicated  in  cases  of  "cold"  abscess,  tuberculous  periostitis,  and  teno- 
synovitis.  The  process  is  an  interstitial  one,  in  which,  in  the  perineurium 
and  epineurium,  there  forms  the  characteristic  granulation  tissue,  which 
eventually  undergoes  caseation.  The  infiltration  may  extend  to  the 
endoneurium  and  the  fibers  undergo  secondary  degeneration.  In 
other  cases  there  form  areas  of  connective-tissue  induration. 

Syphilis. — Like  tuberculosis,  syphilis  generally  attacks  the  roots 
of  the  cranial  and  spinal  nerves,  inasmuch  as  the  process  commonly 
originates  in  syphilitic  meningitis.  The  interstitial  substance  is  infil- 
trated with  granulation  tissue,  which  gradually  is  converted  into  dense, 
fibrous  material.  The  nutrient  vessels  often  show  endarteritic  changes, 
whereby  the  circulation  is  interfered  with.  This,  together  with  the  press- 
ure of  the  newly-formed  fibrous  tissue,  leads  to  marked  degeneration  of 
the  fibers  and  serious  interference  with  function,  such  as  paralysis. 
Gumma  of  the  nerves  seems  to  be  rare. 

Leprosy. — Leprosy  of  the  nerves  constitutes  one  of  the  well-known 
clinical  types  of  this  disease.  The  disease  appears  to  pick  out  more 
especially  the  cutaneous  branches.  Microscopically,  we  find  cellular 
infiltration  of  the  interstitial  substance,  with  a  marked  tendency  to  pro- 
liferation, so  that  scattered  spindle-shaped  nodes  are  formed  on  the 
trunks.  In  the  areas  of  granulation  we  find  large,  epithelioid  cells, 
often  vacuolated,  in  which  the  lepra  bacilli  may  be  readily  detected,  or 
the  organisms  may  lie  free.  The  process  leads  to  degeneration  of  the 
nerve-fibers  and  thus  produces  the  peculiar  anesthetic  and  trophic 
changes  in  the  skin  characteristic  of  this  form  of  leprosy.  Where  the 
ganglia  are  involved,  the  specific  bacilli  can  be  found  also  within  the 
ganglia. 

With  regard  to  the  ganglia  of  the  sympathetic  nervous  system,  it  may 
be  noted  that  they  are  apt  to  be  involved  in  tuberculous  processes. 
Thus,  the  solar  plexus  and  semilunar  ganglia  may  be  involved  in  tuber- 
culosis of  the  suprarenals,  kidney,  or  vertebrae. 


TUMORS  603 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  nerves  is  of  rather  common  occurrence.  It 
may  be  due  to  any  lesion  which  cuts  off  the  nerve-fibers  from  their 
nutrient  centres.  Thus,  destruction  of  the  ganglion  cells,  either  in  the 
brain  or  in  the  cord,  may  lead  to  atrophy  of  the  fibers  proceeding  from 
them.  Pressure,  also,  exerted  upon  the  nerve  from  any  cause,  if  con- 
tinued, will  produce  degeneration  and  atrophy.  Severance  of  a  nerve 
trunk  and  inflammation  are  frequent  causes.  Atrophy  is  also  met  with 
in  old  age. 

Atrophy  and  degeneration  of  nerve-fibers  usually  begins  at  a  point 
the  most  remote  from  the  nutritive  centre  and  progresses  centripetally. 
As  the  type  of  degeneration  may  be  taken  that  form  which  results 
from  the  severance  of  a  nerve-trunk  by  trauma  (Wallerian  degeneration), 
or  follows  continued  pressure  upon  the  trunk,  as,  for  instance,  from  a 
tumor,  enlarged  lymph-glands,  or  constricting  bands.  (See  Introduction, 
p.  520.) 

Besides  the  degeneration  caused  by  solution  of  continuity,  just  referred 
to,  wasting  of  a  similar  kind  may  be  brought  about  by  the  action  of 
circulating  toxins  and  bacteria,  by  certain  circulatory  disturbances,  and 
by  impoverished  nutrition.  Endarteritis  and  other  obstructive  lesions 
of  the  vessels  and  hemorrhages  play  a  part  here,  largely  by  cutting  off  the 
nutrition,  as  do  also  systemic  anemias  and  marasmus.  Degeneration, 
affecting  one  or  more  nerve  trunks,  occurs  sometimes  in  such  conditions 
as  diphtheria,  influenza,  typhoid,  typhus,  variola,  tuberculosis,  the 
puerperium,  and  intoxications  with  mineral  substances,  such  as  lead. 
Here  we  have,  probably,  in  some  cases  at  least,  a  combination  of  im- 
perfect nutrition  and  the  deleterious  action  of  the  poison.  Occasionally, 
from  some  unknown  infection  or  intoxication,  the  ganglion  cells  of  the 
anterior  horns  are  destroyed,  followed  by  secondary  degenerative  changes 
in  the  fibers  connected  with  them. 


PROGRESSIVE  METAMORPHOSES. 

Regeneration  of  the  nerve-elements  after  injury  is  possible  under 
certain  conditions.  Provided  that  the  nerve-centres  or  ganglia  corre- 
sponding to  the  destroyed  fibers  are  intact,  restoration  of  structure  and 
function  is  to  be  looked  for.  For  a  full  discussion  of  this  subject  the  reader 
is  referred  to  vol.  i,  p.  573. 

Tumors. — A  tumor  derived  from  the  proliferation  of  the  nerve 
fibers  in  a  nerve  trunk  would  be  called  a  neuroma.  Such  are  distinctly 
rare.  The  vast  majority  of  tumors  of  the  peripheral  nerves  are  derived 
from  the  interstitial  connective  tissue. 

Fibroma. — The  commonest  growth  is  the  fibroma  in  its  various  forms. 
It  arises  in  the  endoneurium,  as  a  rule,  and  may  extend  externally, 
but  sometimes  internally  into  the  perineurium  and  epineurium,  separating 


604 


THE  PERIPHERAL  NERVES 

FIG.  169 


V 


\ 


Cells  from  a  benign  and  a  malignant  ganglioneuroma  (a  true  neuroma)  respectively;  the 
former  from  the  sacral  region,  the  latter  from  the  retroperitoneal  region  at  the  level  of  the 
pancreas.  (R.  Beneke.) 

FIG.  170 


Multiple  fibromatoid  overgrowths  along  the  course  of  the  cutaneous  nerves.     (Herczel.) 


FIBROMA 


605 


the  different  bundles  one  from  the 
other.  The  nerve-fibers  in  this  way 
become  surrounded  and  compressed 
by  the  proliferating  tissue  and  event- 
ually atrophy.  It  is  believed  by 
some  that  the  nerve-fibers  may  pro- 
liferate and  grow  into  the  tumor,  thus 
form  ing  what  might  be  called  a  neu- 
roma or,  more  correctly,  a  neurofibroma, 
but  this  is  doubtful.  True  neuromas 
are  especially  apt  to  be  formed  in 
connection  with  the  sympathetic  sys- 
tem and  ganglia.  Fibromas  are  gen- 
erally multiple,  and  occasionally  may 
be  found  in  great  numbers  on  most  of 
the  peripheral  nerves.  More  com- 
monly, they  are  restricted  to  partic- 
ular nerve  trunks.  They  are  found 
on  the  main  trunks,  but  exhibit  a 
preference  for  the  smaller  branches, 
especially  the  minute  nerves  of  the 
skin.  Thus,  there  may  be  found  a 
multitude  of  small,  soft  tumors  in  the 
skin,  varying  considerably  in  size 
(multiple  fibromas  of  the  skin;  fibroma 
molluscum).  In  other  cases,  the 
nerve-trunks  present  a  diffuse  and 
widespread  enlargement  of  their  con- 
nective-tissue elements  as  well.  Micro- 
scopically, the  fibromas  are  composed 
of  cellular  connective-tissue,  but  oc- 
casionally dense  or  hard  fibromas  are 
met  with. 

Occasionally,  also,  a  highly  con- 
voluted, tendril-like  growth  develops 
over  a  somewhat  extended  area.  The 
nerve  trunks  are  diffusely  thickened, 
and  studded  with  nodules  and  spin- 
dle-like enlargements  (plexiform  neu- 
roma; Rankenneurom).  They  are 
found  particularly  in  the  distribution 
of  the  spinal  nerves  and  those  of  the 
head,  and  develop  by  preference  in 
the  skin  and  subcutaneous  tissues. 
They  sometimes  form  large,  projecting, 
lobulated  or  folded  growths,  or  may 
lead  to  a  diffuse,  rather  indefinite 
thickening  of  the  skin,  wrhich  resem- 
bles closely  elephantiasis  or  pachy- 
dermia  (elephantiasis  neuromatosa). 


Fio.  171 


Tumors  of  sciatic  nerves  and  their  branches. 
At  a,  large  tumor  connected  with  small  in- 
termuscular  nerve.  (Preble^and  Hektoen.) 


606 


THE  PERIPHERAL  NERVES 


All  forms  of  multiple  fibromas  of  the  peripheral  nerves  are  probably 
to  be  referred  to  errors  of  development,  as  they  are  commonly  found  in 
children,  and  there  may  also  be  a  family  predisposition.  In  rare  cases, 
fibromas  have  been  known  to  undergo  sarcomatous  transformation  and 
produce  metastases  (Westphalen,1  Recklinghausen,  Larkin2). 


FIG.  172 


Multiple  fibroma  of  the  peripheral  nerves.     Reichert  obj.  No.  7,  without  ocular. 
(J.  Alex.  Hutchinson's  case.) 

FIG.  173 


Section  through  .a  fibromatoid  cutaneous  nodule  showing  the  nerve-fibres  (n.  f.)  separated 
by  fibroid  overgrowth.     (After  Ribbert.) 

Myxomas,  lipomas,  and  sarcomas  are  described.  They  are  much  rarer 
than  the  fibromas,  and  form  isolated,  nodular,  or  spindle-shaped  out- 
growths. 

Striated  muscle  cells  have  been  found  in  the  interior  of  nerve-trunks, 
they  have  been  attributed  to  misplaced  embryonal  cells,  but  little  is 
known  about  them. 


1  Virch.  Archiv,  110:1887:29;  ibid.,  114:1888:29. 

2  Jour.  Med.  Research,  (N.  S.)  4: 1903:  217. 


CHAPTER    XXIX. 

THE  SPECIAL  SENSES. 

THE  EYE. 
ANOMALIES    OF    DEVELOPMENT. 

DEFECTS  and  irregularities  in  development  of  the  visual  apparatus 
may  involve  the  eyelids,  the  globe  of  the  eye,  or  any  of  its  component 
parts. 

Complete  absence  of  the  bulb  (anophthalmia)  is  rare.  As  a  rule,  micro- 
scopic examination  will  reveal  traces  of  its  substance.  Anophthalmia 
is  commonly  bilateral,  and  frequently  associated  with  other  anomalies 
of  development,  coloboma,  harelip,  cleft  palate,  and  imperfect  closure  of 
the  cardiac  septum.  Ordinarily,  while  the  bulb  is  extremely  defective, 
the  eyelids,  conjunctival  sac,  muscles,  and  nerves  are  present,  suggesting 
that  at  some  period  the  growth  of  the  bulb  had  been  arrested,  either  as 
a  result  of  a  defect  in  development  or  of  intra-uterine  disease.  A  lesser 
grade  of  the  affection  is  microphthalnria,  in  which  the  globe  of  the  eye 
is  fairly  recognizable  as  such.  All  stages  between  anophthalmia  and 
microphthalmia  exist. 

A  curious  and  rare  anomaly  is  cyclopia  or  synophthalmia,  in  which  the 
entire  visual  apparatus  is  more  or  less  perfectly  fused  into  a  single  organ. 
This  is  one  manifestation  of  the  condition  known  as  cyclencephaly, 
where  the  normal  division  of  the  prosencephalon  fails  to  occur  and  the 
cerebrum  remains  as  a  single  cyst-wall  enclosing  a  single,  more  or  less 
dilated  ventricle.  In  such  a  case,  not  only  are  the  optic  vesicles  involved, 
but  also  the  olfactory  bulbs,  the  bones  of  the  skull  and  face,  and  the  soft 
parts.  In  the  milder  grades  of  the  affection,  two  eyes  may  be  formed, 
but  they  lie  in  a  single  orbit,  or  are  partially  fused.  In  the  more  marked 
form  there  is  but  one  eye,  situated  in  a  single  orbit,  occupying  the  centre 
of  the  forehead,  and  provided  with  but  one  optic  nerve.  In  complete 
cyclopia  the  nose  is  often  rudimentary  or  absent,  the  bones  of  the  face 
are  defective,  and  the  ears  are  anomalously  situated. 

Hydropththalmus. — Hydrophthalmus  is  a  condition  of  early  life, 
believed  to  be  due  to  glaucoma  occurring  during  intra-uterine  existence. 
The  globe  of  the  eye  is  enlarged,  and  more  or  less  fixed.  The  cornea  is 
cloudy,  the  intra-ocular  termination  of  the  optic  nerve  is  excavated,  and 
the  depth  of  the  anterior  chamber  is  increased. 

Among  partial  defects  should  be  mentioned  congenital  ptosis  of  the 
upper  lid;  epicanthus,  a  condition  in  which,  together  with  flatness  of  the 
bridge  of  the  nose,  there  is  a  fold  of  skin  passing  across  the  inner  canthus 


THE  EYE 

from  the  upper  to  the  lower  lid  (a  moderate  grade  of  this  is  normal 
in  the  Chinese  race);  lack  of  the  iris  (irideremia) ;  persistent  pupillary 
membrane;  persistent  hyaloid  artery. 

Among  the  more  striking  anomalies  is  coloboma.  This  is  a  lack  of 
substance,  more  or  less  complete,  in  one  or  more  of  the  primary  mem- 
branes of  the  eye,  due  to  the  failure  of  the  primitive  fetal  cleft  to  close. 
The  retina  and  the  pigment  epithelium  are  derived  from  the  secondary 
optic  vesicle,  about  which  are  formed  the  choroid  and  scleral  mem- 
branes. The  primitive  cleft  in  the  secondary  optic  vesicle  extends 
backward  inferiorly  into  the  optic  stalk.  Should  it  fail  to  close  at  its 
posterior  part,  coloboma  of  the  optic  nerve  sheath  results.  If  the 
middle  portion  does  not  close,  the  ordinary  coloboma  of  the  choroid 
results.  Failure  to  close  anteriorly  results  in  defective  formation  of  the 
lower  part  of  the  iris.  According  to  this,  several  different  forms  and 
grades  may  be  recognized.  In  complete  failure  to  close,  the  choroid 
and  sclera  will  be  imperfectly  formed,  while  the  retina  and  pigment 
epithelium  will  be  lacking.  Should,  however,  the  closure  be  merely 
delayed,  both  retina  and  pigment  epithelium  may  be  completed,  but  the 
choroid  will  be  defective  and  the  superimposed  pigment  epithelium  will 
not  become  pigmented.  In  coloboma  of  the  choroid,  owing  to  thinness 
of  the  sclera,  ectasia  of  this  membrane  is  a  not  uncommon  result.  This 
ectasia,  or  staphyloma,  of  the  sclera  may  be  very  marked,  especially  in 
cases  of  microphthalmus,  and  may  indeed  form  a  cyst  as  large  as  the 
eyeball  itself. 

In  coloboma  of  the  choroid,  one  finds,  microscopically,  in  place  of  the 
proper  retina  and  choroid,  a  thin  connective-tissue  membrane,  in  which 
are  a  few  vessels  and  a  scanty  deposit  of  pigment.  In  other  cases  there 
may  be  present  more  or  less  perfect  retinal  elements. 

Coloboma,  when  affecting  the  lower  part  of  the  vertical  meridian  of  the 
eye  is  a  purely  developmental  error.  Coloboma  in  other  directions,  and 
the  so-called*  coloboma  of  the  macula,  are  due  to  inflammation  occurring 
during  intra-uterine  life,  the  defect  in  formation  being  due  to  the  mechan- 
ical effect  of  adhesions.  Congenitally  defective  eyes  are  liable  to  other 
diseases,  such  as  choroiditis  and  cataract. 

The  Conjunctiva. 

The  upper  and  lower  eyelids  are  covered  externally  with  skin,  similar 
to  that  of  the  forehead  and  cheeks,  but  somewhat  thinner  and  looser. 
Beneath  this  is  a  layer  of  loose,  areolar  tissue;  next,  the  striated  fibers  of 
the  orbicularis  palpebrarum;  then,  the  so-called  tarsal  cartilage,  composed 
of  dense,  white  connective  tissue  and  containing  the  Meibomian  glands, 
practically  identical  in  structure  with  the  sebaceous  glands;  a  subcon- 
junctival  layer,  containing  more  or  less  diffuse  adenoid  tissue;  and  finally 
the  conjunctiva  itself,  composed  of  stratified  epithelium.  On  the  inner 
surface  of  the  lids  the  epithelium  is  of  the  squamous  variety  and  rather 
thin,  but  toward  the  fornix  the  membrane  is  looser,  more  vascular,  and 
thrown  into  folds,  while  the  epithelium  is  columnar.  When  the  con- 


CONJUNCTIVITIS  609 

junctivu  reaches  the  bulb,  the  superficial  cells  tend  again  to  become 
flattened,  and  the  membrane  assumes  more  the  appearance  of  that  cover- 
ing the  cornea.  The  vascularity  of  the  subconjunctival  tissues  and  the 
looseness  of  their  texture  renders  the  membrane,  and,  indeed,  the  lid 
as  a  whole,  particularly  susceptible  to  circulatory  and  inflammatory 
disturbances,  while  the  results  of  these  conditions  are  usually  striking. 


CIRCULATORY  DISTURBANCES. 

(Edema. — (Edema  of  the  lids  and  conjunctiva  is  a  condition  of 
clinical  importance,  as  it  is  frequently  an  indication  of  serious  disease. 
It  is  found,  for  instance,  in  chronic  renal  and  cardiac  affections,  and  in 
anemia.  In  such  cases  the  conjunctiva  is  swollen,  and  has  a  glassy  or 
watery  look  (chemosis).  (Edema  of  the  lids  is,  also,  a  common  accom- 
paniment of  inflammation,  not  only  of  the  eye  itself,  but  of  its  associated, 
structures.  Thus,  it  is  met  with  in  such  conditions  as  conjunctivitis, 
panophthalmitis,  dacryocystitis,  retrobulbar  abscess,  and  suppuration 
of  the  frontal  sinuses. 

Acute  Hyperemia. — Simple  acute  hyperemia  of  the  conjunctiva  is 
a  common  symptom  of  irritation.  A  foreign  body  in  the  conjunctival 
sac,  excessive  crying,  the  exposure  of  the  eyes  to  the  wind  or  sun,  "eye- 
strain,"  irritant  gases,  and  the  use  of  certain  drugs,  such  as  arsenic  and 
potassium  iodide,  may  all  cause  it.  It  is  an  early  feature  of  many  in- 
flammations of  the  eyes,  and  is  met  with  in  acute  rhinitis  and  facial 
neuralgia.  Chronic  congestion  is  most  frequently  due  to  errors  of 
refraction  and  disorders  of  the  ocular  muscles,  but  may  also  be  an  indica- 
tion of  alcoholism,  gout,  nasal  catarrh,  and  inflammation  of  the  lacrymal 
ducts. 

Hemorrhage. — Hemorrhage  into  the  loose  tissue  beneath  the  con- 
junctival membrane  may  be  the  result  of  injury  to  the  eye,  *f racture  of 
the  skull,  or  may  come  on  without  any  particular  cause.  Severe  attacks  of 
sneezing,  coughing,  or  vomiting  have  been  known  to  cause  it.  Possibly, 
sclerosis  or  other  degenerative  changes  in  the  vessel  walls  predisposes  to 
the  condition. 

INFLAMMATIONS. 

Conjunctivitis. — Inflammation  of  the  conjunctiva — conjunctivitis  or 
ophthalmia — may  affect  the  membrane  as  a  whole,  or  any  part  of  it. 
Acute  and  chronic  forms  are  recognized.  Primary  acute  conjunctivitis 
may  result  from  exposure  to  wind  and  weather,  heat,  irritating  gases, 
injury,  or  from  the  action  of  microorganisms.  Secondary  acute  conjuttc- 
liritis  may  be  due  to  the  extension  of  inflammation  from  neighboring 
parts,  face,  eyelids,  nose,  or  lacrymal  ducts,  or  may  accompany  or 
complicate  certain  of  the  infectious  fevers,  such  as  measles,  influenza, 
typhus  and  whooping-cough. 

Acute  conjunctivitis  may  sometimes  also  be  produced  by  the  action 
39 


610  THE  CONJUNCTIVA 

of  certain  drugs.  Simple  catarrhal  inflammation  is  a  not  infrequent 
occurrence  in  persons  who  are  taking  potassium  iodide  or  arsenic.  The 
external  application  of  chrysophanic  acid  in  psoriasis  has  been  known 
to  cause  it.  The  local  use  of  atropine,  eserine,  and  hyoscyamine  may 
also,  if  long  continued,  or  in  susceptible  people,  on  occasion  produce  it. 

A  considerable  number  of  microorganisms  are  now  known  to  give 
rise  to  conjunctivitis.  Chief  among  these  are  the  Morax-Axenfeld 
Diplobacillus,  the  Koch- Weeks  bacillus,  the  pyogenic  cocci,  the  Gono- 
coccus,  and  the  Pneumococcus.  Exceptionally,  Friedlander's  bacillus, 
the  Diphtheria  bacillus,  the  Bacillus  of  Pfeiffer,  and  the  B.  coli  have 
been  found.  McKee,1  in  a  study  of  500  cases,  gives  the  following 
proportions : 

Morax-Axenfeld  Diplobacillus  in  200;  Staphylococcus,  50;  Strepto- 
coccus, 24;  Pneumococcus,  13;  Micrococcus  catarrhalis,  12;  Gonococcus, 
10;  McKee  bacillus,  9;  Koch- Weeks  organism,  7;  B.  coli,  5;  influenza 
bacillus, 3;  Meningococcus,  1;  Bacillus xerosis,  B.Hoffmann;  and  sapro- 
phytes, 102;  negative  results  in  64.  Certain  of  these,  the  Koch-Weeks 
bacillus  and  the  Pneumococcus,  are  not  infrequently  to  be  found  on  the 
normal  conjunctiva,  and  are  usually  innocuous.  On  occasions,  owing  to 
some  increase  of  virulence  or  some  injury  to  the  membrane,  they  may 
assume  pathogenic  properties. 

In  all  forms  of  conjunctivitis  the  process  is  essentially  the  same, 
though  the  intensity  may  vary.  The  conjunctival  vessels  are  congested, 
the  subconjunctival  tissues  are  oedematous  and  infiltrated,  the  lymph- 
adenoid  follicles  are  enlarged,  and  the  superficial  epithelium  is  sodden, 
swollen,  desquamating,  or  eroded.  It  is  usual  to  classify  the  clinical 
varieties  according  to  the  character  of  the  exudate,  which  may  be  serous 
or  seromucoid,  seropurulent,  purulent,  or  membranous. 

Acute  Catarrhal  Conjunctivitis. — In  the  milder  grades  of  acute  catarrhal 
conjunctivitis,  the  inflammation  may  be  confined  to  the  palpebral 
conjunctiva,  which  is  swollen,  reddened,  and  succulent.  There  is  a 
slight  mucoid  discharge  which  tends  to  glue  the  eyelids  together  and 
accumulates  about  the  inner  canthus  and  the  retrotarsal  fold.  In  the 
more  severe  forms  the  bulbar  conjunctiva  is  involved  as  well,  and,  owing 
to  the  congestion,  assumes  a  reddish  color.  The  conjunctiva  is  con- 
siderably thickened,  particularly  at  the  retrotarsal  fold,  where  it  may 
show  papillary  excrescences.  The  discharge  is  more  abundant  and  is 
seropurulent  or  mucopurulent  in  character. 

Acute  Purulent  Conjunctivitis. — Acute  purulent  conjunctivitis  is  a 
severe  affection  of  the  conjunctiva,  due  in  the  vast  majority  of  cases  to 
the  Gonococcus  of  Neisser.  The  disease  is  most  commonly  met  with  in 
newborn  infants  who  have  been  inoculated  from  the  maternal  passages 
during  birth  (ophthalmia  neonatorum),  and  next  in  adults  the  subjects 
of  gonorrhoea  of  the  urethra,  vulva,  or  vagina.  Occasionally,  it  is  found 
in  young,  debilitated  girls,  who  are  suffering  from  non-specific  purulent 

1  A  Clinical  Study  of  Five  Hundred  Cases  of  Conjunctivitis7  Amer.  Jour.  Med. 
Sci.,  134:1907:716. 


PLATE   VI 

FIG.   1 


Koch-Weeks   Bacillus.     (Weeks.) 


FIG.  2 


Morax-Axenfeld    Diplobacillus.     (Weeks. ) 


PARINAUD'S  CONJUNCTIVITIS  611 

vulvitis  and  vaginitis.  Sometimes,  also,  the  acute  catarrhal  form, 
when  passed  rapidly  from  one  individual  to  another,  and  occurring  in 
weakly  persons,  may  become  intensified  into  the  suppurative  variety, 
In  purulent  conjunctivitis  the  inflammatory  process  is  extremely  severe. 
There  is  intense  congestion  and  chemosis  of  the  conjunctiva,  the  lids  are 
tense,  swollen,  and  reddened.  Small  hemorrhages  may  occur  into  the 
conjunctiva,  which  bleeds  at  the  slightest  touch.  The  discharge,  at  first 
serous,  soon  becomes  creamy  and  abundant,  and  is  of  a  yellowish  or 
yellowish-green  color.  In  children,  as  a  rule,  both  eyes  are  involved, 
as  would  be  expected.  In  adults,  one  eye  often  escapes.  There  is 
great  danger  to  sight  in  gonorrhoeal  ophthalmia,  inasmuch  as  the  cornea 
is  apt  to  be  involved  in  neglected,  severe,  or  improperly  treated  cases. 
A  diffuse  opacity  may  spread  over  the  cornea,  or,  in  other  cases,  small 
ulcers  may  form,  leading  sometimes  to  perforation,  with  all  this  implies. 
More  or  less  permanent  thickening  of  the  conjunctiva  may  result  with 
scarring. 

Membranous  or  Croupous  Conjunctivitis. — Membranous  or  croupous 
conjunctivitis  is  characterized  by  the  formation  of  a  solid  exudate  in  the 
form  of  a  patchy  or  confluent  membrane  more  or  less  firmly  attached 
to  the  conjunctival  tissues.  Occasionally,  owing  to  some  peculiarity 
in  the  state  of  health  of  the  affected  person,  a  mucopurulent  or  purulent 
ophthalmia  assumes  this  type.  The  too  vigorous  use  of  caustics  may 
also  cause  it,  as  does  also  the  solution  of  jequirity,  commonly  employed 
in  the  treatment  of  trachoma. 

True  diphtheria  of  the  conjunctiva  is  rare.  It  occasionally  arises  by 
extension  from  the  nasal  passages,  or  by  direct  inoculation  with  infective 
material.  Rarely,  it  may  be  primary.  It  has  also  been  known  to  follow 
operative  procedures.  Both  palpebral  and  bulbar  conjunctiva  are 
involved.  The  lids  are  greatly  swollen  and  congested,  while  they  are 
tense  and  brawny  from  infiltration.  Owing  to  the  interference  with  the 
circulation  there  is  grave  danger  to  the  cornea  in  this  form  of  conjunc- 
tivitis. The  membrane  is  thick,  firm,  and  adherent.  The  subconjunc- 
tival  tissues  are  also  considerably  involved,  and  more  or  less  deeply 
penetrating  ulcerations  are  not  uncommon.  When  healing  takes  place, 
this  leads  to  the  formation  of  scars,  and  not  infrequently  to  incurvation 
of  the  lids.  The  discharge  is  at  first  scanty,  thin,  and  ichorous,  later 
purulent. 

Variola. — Variola  pustules  are  occasionally  found  upon  the  conjunc- 
tiva. They  lead  to  purulent  infiltration  or  ulcerations  of  the  cornea. 
Staphyloma  may  thus  result,  or  the  condition  may  go  on  to  suppurative 
choroiditis  and  panophthalmitis. 

Parinaud's  Conjunctivitis.  —  A  curious,  though  rare,  form  of  acute 
inflammation  is  Parinaud's  conjunctivitis  (lymphoma  conjunctive?).  It 
is  peculiar  in  that  it  is  accompanied  by  local  and  systemic  manifesta- 
tions of  infection. 

The  affection  begins  with  slight  symptoms  of  conjunctivitis,  which, 
in  a  few  days  becomes  worse,  the  lids  become  markedly  swollen,  and 
there  is  slight  ptosis,  with  lacrymation  and  photophobia.  The  con- 


612  THE  CONJUNCTIVA 

junctiva  of  the  lid  is  much  swollen  and  reddened,  and  is  studded  with 
numerous  papillary  granulations,  which  are  especially  in  evidence  in  the 
retrotarsal  folds.  The  conjunctiva  bulbi  is  not  involved,  save  that  it  is 
somewhat  injected.  Soon  the  granulations  enlarge,  forming  relatively 
enormous,  papillary,  and  cock's-comb-like  masses.  The  pre-auricular, 
inframaxillary,  and  cervical  glands  sooner  or  later  become  enlarged, 
and  there  is  a  slight  evening  rise  of  temperature.  The  secretion  is 
scanty  and  mucopurulent  in  character.  Ulceration  does  not  take  place, 
nor  is  a  membrane  produced.  The  enlarged  glands  may  suppurate  or 
may  resolve.  Stirling  and  McCrae,1  in  a  case  met  with  in  Montreal, 
found  a  bacillus  intermediate  in  properties  between  the  Bacillus  diph- 
theria? and  the  Bacillus  xerosis,  which  they  regarded  as  probably  specific. 

Trachoma  or  Granular  Conjunctivitis. — The  most  important  of  the  chronic 
inflammations  of  the  conjunctiva  is  the  so-called  trachoma  or  granular 
conjunctivitis.  This  affection  is  essentially  chronic  in  its  course,  but  mani- 
fests occasional  acute  exacerbations.  Anatomically,  it  is  characterized 
in  the  main  by  overgrowth  of  tissue,  which  in  time,  when  absorption  has 
taken  place,  is  followed  by  cicatricial  contraction.  Although  the  disease 
is  a  common  one  in  certain  countries,  and  has  been  known  for  a  long 
time,  its  etiology  is  still  obscure.  The  disease  is  undoubtedly  contagious, 
being  transmitted  from  person  to  person  by  the  secretion,  usually  by 
means  of  towels.  Sattler  has  described  a  coccus  which  he  regarded 
as  the  specific  cause  of  the  disease,  but  the  evidence  is  as  yet  far  from 
convincing.  Some  hold  that  the  disease  is  the  chronic  form  of  acute 
epidemic  conjunctivitis,  which  is  usually  due  to  the  Gonococcus  or  the 
Koch- Weeks  bacillus. 

The  process  begins  and  is  always  most  marked  in  the  conjunctiva  of 
the  lids  and  retrotarsal  folds,  but  may  eventually  extend  to  the  tarsus  and 
conjunctiva  bulbi.  At  the  onset  and  during  the  acute  relapses,  the 
lids  are  considerably  swollen,  the  conjunctiva  is  much  injected,  and  there 
is  a  moderate  amount  of  mucoid  or  mucopurulent  secretion.  Photo- 
phobia and  blepharospasm  may  be  marked.  Later,  the  conjunctiva 
is  congested  and  considerably  thickened,  being  studded  with  papillary 
outgrowths  (papillary  conjunctivitis)  and  "granulations"  (follicular  con- 
junctivitis). The  trachoma  bodies  or  follicles  are  most  numerous  in  the 
retrotarsal  folds,  and  on  eversion  of  the  lids  can  be  seen  small,  grayish, 
translucent  nodules,  resembling  grains  of  boiled  sago  or  frog's  spawn. 

Histologically,  there  is  found  a  diffuse  infiltration  of  the  conjunctiva 
of  the  lids  with  lymphoid  cells.  The  papillae  are  much  enlarged. 
The  trachoma  bodies  are  not  typical  granulation  tissue,  but  due  to  a 
local  hyperplasia  of  the  lymphoid  and  connective-tissue  elements,  sur- 
rounded by  a  more  or  less  perfect  fibrous  capsule.  The  subconjunctival 
tissue  is  also  hyperplastic.  In  long-standing  cases  cicatricial  contraction 
of  the  connective  tissue  takes  place,  with  atrophy  of  the  conjunctiva, 
so  that  the  retrotarsal  folds  are  gradually  obliterated,  the  movements  of 
the  eyeball  interfered  with,  and  a  condition  of  xerophthalmia  is  induced. 

1  A  Case  of  Parinaud's  Conjunctivitis,  Montreal  Med.  Jour.,  33: 1904:  575. 


ECZEMA  OF  THE  CONJUNCTIVA  613 

The  eyelids  are  often  curved  inward  (entropion)  so  that  the  lashes  are 
directed  against  the  conjunctiva  (trichiasis;  dystrichiasis).  From  the 
combined  results  of  friction  and  inflammation,  the  cornea  sometimes 
becomes  cloudy,  the  superficial  epithelium  somewhat  roughened,  and 
sluggish  ulcers  may  become  manifest.  The  corneal  tissue  may  also  soften 
and  bulge  outward.  Not  infrequently,  the  inflammatory  process  spreads 
under  the  subepithelial  layer  toward  the  centre  of  the  cornea,  the  newly 
formed  tissue  gradually  becoming  vascularized  (pannus). 

Follicular  Conjunctivitis. — Some  authorities,  notably  Saemisch,  describe 
a  follicular  conjunctivitis,  which  bears  a  strong  resemblance  to  trachoma, 
at  least  in  its  early  stages.  The  disease  affects  the  conjunctiva  of  the 
lids  only,  and  leads  to  the  formation  of  nodes  of  hypertrophied  lymphoid 
tissue.  There  are,  however,  no  hypertrophied  papillae.  The  changes 
in  by  far  the  majority  of  cases  are  quite  superficial  and  never  lead  to 
cicatricial  contraction,  ulceration,  or  pannus.  The  condition  may  heal 
without  leaving  any  trace.  The  disease  is  said  to  be  feebly,  if  at  all, 
contagious.  It  must  be  said,  however,  that  it  is  by  no  means  proved 
that  this  affection  differs  essentially  from  trachoma,  either  etiologically, 
or  in  other  particulars.  In  fact,  transitional  forms  are  by  no  means 
unknown.  Some  of  the  cases  are  attributable  to  atropine  irritation. 

Vernal  Conjunctivitis  (Spring  Catarrh;  Gelatinous  Infiltration  of  the 
Limbus;  Phlyctaena  Pallida). — Vernal  conjunctivitis  is  an  apparently 
specific  and  extremely  obstinate  affection  of  the  conjunctiva.  The 
disease  affects  both  eyes  and  is  most  common  in  children.  As  its  name 
implies,  it  is  most  troublesome  with  the  onset  of  warm  weather,  tending  to 
disappear  during  the  winter.  It  may  last  for  years,  and  is  believed  by 
many  to  be  feebly  contagious.  Both  the  ocular  and  palpebral  conjunctiva? 
may  be  involved.  The  conjunctiva  of  the  upper  lid,  which  is  the  one 
usually  involved,  presents  a  peculiar  bluish-white,  milky  appearance, 
characteristic  of  the  disease,  and  is  covered  with  flat,  rounded  elevations 
of  almost  cartilaginous  hardness,  giving  it  a  curious  tessellated  appear- 
ance. When  the  bulb  is  affected,  one  sees  flattened,  elevated,  gelatinous 
masses  of  a  brownish-pink  color,  close  to  the  limbus  of  the  cornea.  The 
growth  may  spread  laterally,  or  occasionally  may  encircle  the  cornea, 
which  may  also  be  more  or  less  encroached  upon. 

Microscopically,  the  patches  are  composed  of  connective  tissue  and 
greatly  thickened  epithelium,  which  tends  to  send  prolongations  into  the 
underlying  structures.  According  to  Fuchs,  the  patches  are  composed  of 
areolar  connective  tissue,  which  has  undergone  hyaline  degeneration, 
covered  with  thickened  epithelium.  There  is  but  little  secretion  in  this 
form  of  conjunctivitis.  There  is  no  tendency  to  ulceration  or  the  forma- 
tion of  pannus.  Occasionally,  the  peripheral  zone  of  the  cornea  undergoes 
fatty  degeneration,  producing  an  appearance  similar  to  that  in  arcus 
senilis. 

Eczema  of  the  Conjunctiva. — Eczema  of  the  conjunctiva  (conjunc- 
tivitis phlyctoenulosa;  conjunctivitis  lymphatica  sive  scrofulosa)  is  usually 
found  in  young  children,  especially  those  who  are  weakly  or  of  the 
so-called  "  scrofulous"  temperament.  Unlike  most  other  forms  of  conjunc- 


614  THE  CONJUNCTIVA 

tivitis,  it  is  invariably  associated  with  some  constitutional  disturbance, 
furred  tongue,  loss  of  appetite,  constipation.  It  is  not  infrequently 
seen  in  combination  with  eczema  of  the  face,  nose,  ears,  and  hands. 
Ordinarily,  one  eye  only  is  affected. 

The  disease  is  characterized  by  the  formation  of  one  or  more  papules 
or  pustules,  varying  in  size  from  that  of  a  mustard  seed  to,  in  rare  cases, 
that  of  a  split  pea.  These  are,  histologically,  composed  of  aggregations 
of  inflammatory  leukocytes,  covered  at  first  with  the  epithelial  layer. 
Eventually,  this  may  give  way,  so  that  an  open  ulcer  is  the  result.  The 
papules,  pustules,  or  ulcers  are  delimited  by  a  zone  of  catarrhal  inflam- 
mation, or  the  bulbar  conjunctiva  as  a  whole  may  be  diffusely  reddened 
and  swollen.  There  is  a  mucopurulent  exudation.  Not  infrequently, 
the  cornea  is  involved  in  a  similar  manner.  The  conjunctiva  of  the 
lids  almost  always  escapes.  The  affection  may  run  an  acute  course,  but 
often  is.  chronic  with  occasional  acute  exacerbations. 

Xerosis. — Xerosis  of  the  conjunctiva  (pemphigus  of  the  conjunctiva) 
is  a  curious  and  somewhat  rare  disease,  the  exact  nature  of  which  is  not 
entirely  understood.  It  occurs  under  the  guise  of  a  chronic  inflamma- 
tion, in  which  the  conjunctiva,  chiefly  that  of  the  bulbus,  slowly  atrophies 
and  contracts,  owing  to  the  formation  in  it  of  cicatricial  tissue.  The 
membrane  itself  is  dry  and  lustreless,  and  is  covered  with  fine,  whitish, 
fatty  scales.  The  process  may  resolve,  but  not  infrequently  progresses 
until  the  free  edges  of  the  lids  are  bound  down  to  the  globe  and  become 
continuous  with  the  cornea,  thus  limiting  greatly,  if  not  entirely,  the  move- 
ments of  the  eyeball.  The  cornea,  in  turn,  may  become  involved.  The 
scales  consist  of  stratified,  keratinized,  epithelial  cells,  often  fattily 
degenerated,  together  with  free  fat-droplets.  The  desquamating  material 
also  contains  a  small  bacillus,  the  so-called  Bacillus  xerosis,  which  bears 
some  resemblance  to  the  Bacillus  of  diphtheria.  Its  specificity  is,  how- 
ever, by  no  means  beyond  question. 

Tuberculosis. — Tuberculosis  is  quite  rare.  So  far  as  is  known  at 
present,  it  is  always  secondary  to  lupus  of  the  face.  In  the  connective 
tissue  of  the  lids  and  bulb  are  formed  more  or  less  circumscribed,  flattened 
outgrowths,  recalling  the  fungoid  masses  seen  in  tuberculous  synovitis. 
These  are  reddish,  warty,  and  bear  a  close  resemblance  to  granulations. 
In  time  they  break  down,  giving  rise  to  larger  or  smaller,  irregular 
ulcers,  in  the  floor  of  which  grayish  or  yellowish,  caseous  tubercles  may 
sometimes  be  seen.  From  the  confluence  of  the  tubercles,  larger  nodules 
may  be  formed.  The  irritation  of  the  tuberculous  process  often,  also, 
leads  to  enlargement  of  the  follicles  in  the  retrotarsal  folds  (follicular 
conjunctivitis). 

Syphilis. — Primary  and  secondary  lesions  may  occur,  and  also 
gummas.  The  condition  is  very  uncommon. 

Leprosy. — Leprosy  is  also  rare. 


PTERYGIUM  615 


PROGRESSIVE  METAMORPHOSES. 

Pinguecula. — Pinguecula,  called  so  from  the  Latin  "pinguis,"  fat, 
owing  to  an  erroneous  notion  as  to  its  structure,  is  a  yellowish,  ele- 
vated mass,  situated  in  the  conjunctiva  bulbi  and  the  subconjunctival 
tissue,  usually  to  the  nasal  side,  but  occasionally,  though  rarely,  to  the 
temporal  side.  It  is  found  in  middle-aged  people.  Its  position  on  the 
most  exposed  portion  of  the  conjunctiva  suggests  that  the  condition  may 
be  the  result  of  irritation,  such  as  exposure  to  wind  and  rain.  Micro- 
scopically, there  is  down-growth  of  the  epithelium  in  places,  with  the 
formation  of  gland-like  processes  and  even  cysts.  The  connective  tissue 
is  increased  and  there  is  a  marked  development  of  new  elastic  fibers. 
Both  fibrous  and  elastic  tissue  present  hyaline  degeneration. 

Pterygium  (nripuS,  a  wing). — Pterygium  is  an  overgrowth  of  the 
conjunctiva  and  subconjunctival  tissues,  usually  occurring  on  the  nasal 
aspect  of  the  eyeball.  It  takes  the  form  of  a  triangular  elevation, 
more  or  less  vascular,  the  apex  being  toward  the  centre  and  the  base 
toward  the  periphery  of  the  eye.  The  growth  tends  to  encroach  upon  the 
cornea.  The  superficial  epithelium  is  thrown  into  folds,  and  is  composed 
of  stratified  cells,  the  deeper  of  which  are  cuboidal,  while  the  others  are 
more  cylindrical  or  pointed. 

The  overgrowth  of  epithelium  in  places  leads  to  the  formation  of  gland- 
like  structures  and  even  cysts.  Numerous  goblet-cells  are  to  be  found 
in  the  shallower  depressions.  Beneath  the  epithelium  there  is  an  aggre- 
gation of  lymphoid  cells  with  numerous  small  vessels.  The  Bowman's 
membrane  is  substituted  by  a  fibrillar  layer  of  connective  tissue,  con- 
taining large  blood-  and  lymph-vessels. 

The  cause  or  causes  are  not  altogether  clear.  Most  authorities  attrib- 
ute the  tissue-overgrowth  to  irritation  or  hyperemia.  Cases  often  follow 
exposure  to  heat,  dust,  or  noxious  vapors,  and,  also  occasionally,  trau- 
matism.  Eyestrain,  by  producing  congestion  of  the  vessels,  may  possibly 
be  a  cause.  Pinguecula  not  infrequently  precedes  pterygium. 

Cysts. — Cysts  are  due  to  dilatation  of  the  lymphatics  or  to  obstruction 
of  the  glands,  with  retention  of  the  secretion.  In  lymphangiectasis, 
small,  rounded  vesicles,  filled  with  clear  fluid,  are  formed  on  the  con- 
junctiva of  the  bulb,  where  they  are  arranged  in  little  clusters  or  after 
the  fashion  of  a  string  of  beads. 

Histologically,  these  formations  consist  of  a  number  of  intercom- 
municating cavities,  containing  a  few  lymphatic  cells,  and  bounded  by 
connective  tissue,  on  which  may  sometimes  be  recognized  the  scanty 
remains  of  an  endothelial  lining. 

Serous  cysts  may  result  from  a  lymphangiectasis  in  which  the  separat- 
ing partitions  between  the  spaces  have  given  way,  thus  producing  a 
single  cavity.  They  are,  however,  occasionally  found  at  birth,  or  result 
from  injury,  or,  again,  may  be  due  to  cystic  degeneration  of  small, 
glandular  lobules  in  the  fornix.  They  form  round,  oval,  or  oblong 
swellings  in  the  conjunctiva,  of  a  somewhat  yellowish  color.  Micro- 


616  THE  CONJUNCTIVA 

scopically,  they  have  a  well-defined  wall  of  connective  tissue,  usually 
lined  with  endothelial  cells.  Teleangiectasis  of  the  tarsal  and  bulbar 
conjunctiva  is  occasionally  me  with,  usually  near  the  lachrymal  caruncle. 

Tumors. — Fibromas. — Fibromas  spring  from  the  palpebral  conjunc- 
tiva or  from  the  cul-de-sac,  and  are  frequently  pedicled.  They  may 
be  flattened  from  pressure  or  may  even  assume  a  cup  shape.  Micro- 
scopically, they  are  composed  of  dense,  fibrous  tissue  with  relatively 
few  vessels,  although  the  smaller  ones  may  be  more  cellular. 

Papilloma.  —  Papilloma  of  the  conjunctiva  is  extremely  rare,  and 
usually  springs  from  the  sclerocorneal  junction.  The  tumor  possesses 
a  pedicle  and  is  composed  of  a  richly-branching  core  of  connective 
tissue  covered  with  a  thick,  stratified  investment  of  epithelial  cells, 
cuboidal,  cylindrical,  or  spindle-shaped.  Toward  the  periphery  of  the 
processes  the  core  is  more  cellular  and  infiltrated  with  leukocytes. 

Lipoma. — Lipoma  is  a  rather  rare  tumor  of  the  conjunctiva.  It  is 
congenital  and  appears  in  the  form  of  an  elevated,  wedge-shaped  mass, 
lobulated,  and  of  a  yellowish  color,  situated  usually  between  the  external 
and  inferior  rectus.  Microscopically,  it  is  composed  of  fatty  and 
fibrous  tissue,  covered  with  thickened  conjunctiva.  It  is  supposed  to 
originate  in  a  hernia  of  the  orbital  fat. 

Dermoid. — Another  congenital  tumor,  occasionally  met  with,  is  the 
dermoid.  As  its  name  implies,  it  is  composed  of  the  elements  of  the 
skin.  It  is  usually  of  small  size,  of  a  smooth,  shiny  appearance,  and 
is  situated  at  the  corneoscleral  junction,  partly  on  the  sclera  and  partly 
on  the  cornea.  The  corneal  sector  is  commonly  bounded  by  an  opaque 
line  similar  in  appearance  to  an  arcus  senilis.  The  superficial  epithelium 
is  somewhat  similar  to  that  of  the  skin,  save  that  it  does  not  become 
keratinized,  but,  on  the  contrary,  is  soft  and  swollen,  apparently  from 
maceration.  Underneath  there  is  a  dense  connective-tissue  membrane, 
containing  elastic  fibers,  bloodvessels,  glands,  and  fat.  Projecting  from 
the  surface  are  more  or  less  numerous  hairs.  Alt1  has  described  what  he 
calls  a  chondro -adenoma,  a  congenital  tumor,  composed  of  gland-tubules 
suggesting  those  of  the  lachrymal  gland,  together  with  a  mass  of  embryonic 
cartilage.  The  two  elements  were  separated  one  from  the  other  by,  and 
enclosed  in,  connective  tissue.  The  growth  was  the  size  of  a  split  pea, 
situated  on  the  bulbar  conjunctiva.  It  was  sessile,  smooth,  and  of  a 
whitish  color.  Probably  this  should  be  classed  as  a  teratoma. 

Osteoma. — Osteoma  has  been  met  with  in  a  few  instances.  It  is  found 
on  the  outer  aspect  of  the  eye,  usually  between  the  points  of  insertion 
of  the  superior  and  external  rectus.  It  appears  not  to  be  congenital. 

The  malignant  tumors  of  the  conjunctiva  are  the  epithelioma  and  the 
sarcoma.  They  may  be  primary,  but  are  most  frequently  secondary  to 
growths  of  the  eyelids  or  orbit. 

Epithelioma. — Epithelioma  is  the  commonest  tumor  of  the  conjunctiva. 
It  begins,  usually,  near  the  corneoscleral  junction,  with  the  formation 

1  Reference  Handbook  of  the  Medical  Sciences,  New  York,  Wm.  Wood  &  Co., 
4:  1902:  109. 


TRAUMA  617 

of  a  small  nodule  covered  with  distended  bloodvessels.  The  growth, 
if  not  removed,  gradually  spreads  to  the  cornea,  and  finally  invades  the 
interior  of  the  eye.  Some  of  these  new-growths  may  be  pigmented 
(melanocancroids). 

Microscopically,  the  tumor  consists  of  a  thickened  epithelium,  which 
sends  out  downgrowths  of  a  finger-like,  branching  form  into  the  deeper 
structures.  The  horny  layer  is  often  considerably  thickened.  About 
the  periphery  of  the  tumor  the  vessels  are  dilated  and  the  tissues 
infiltrated  with  leukocytes. 

Sarcoma. — Sarcomas  are  distinctly  rare,  and  are  usually  pigmented 
Like  the  epithelioma,  they  commonly  begin  at  the  corneoscleral  junction, 
or,  rarely,  in  the  fornix.  They  form  rounded,  sometimes  lobulated, 
growths  of  a  rusty  brown  or  blackish  color.  They  are  very  vascular 
and  bleed  readily.  They  do  not  tend  to  ulcerate,  and  appear  smooth 
and  shiny,  owing  to  the  fact  that  they  are  covered  by  epithelium. 

Microscopically,  the  sarcomas  are  of  the  small,  round-celled  variety, 
more  rarely  spindle-celled.  The  vessels  are  numerous,  and  there  are 
evidences  of  both  old  and  recent  hemorrhages. 


TRAUMATISM. 

The  conjunctiva  may  be  cut  or  torn,  and  such  accidents  usually  give 
rise  to  considerable  hemorrhage  into  the  loose  subconjunctival  tissue, 
together  with  more  or  less  oedema.  The  conjunctiva  may  also  be  in- 
jured by  foreign  bodies  gaining  a  lodgement  on  it;  or  by  the  action  of 
caustic  substances,  such  as  lye,  lime,  acid,  or  molten  metal,  which  have 
sparked  into  the  eye.  More  or  less  congestion  results  in  all  instances, 
and  in  the  more  severe  injuries,  inflammation.  Occasionally,  where 
the  corresponding  portions  of  the  ocular  and  palpebral  conjunctiva  are 
involved,  and  there  is  loss  of  the  epithelium,  union  of  the  eyelid  with 
the  eye  may  take  place  (symblepharoii). 

The  Cornea. 

The  cornea  of  the  eye  is  a  stratified  membrane,  and  is  peculiar  in  that, 
under  ordinary  circumstances,  it  is  perfectly  transparent.  It  may  be 
compared  to  a  window  placed  in  the  frame  of  the  sclera.  It  is  elliptical 
in  outline,  being  slightly  broader  than  it  is  high.  The  outer  and  inner 
curvatures  are,  moreover,  not  parallel.  The  various  layers  of  the  cornea, 
beginning  from  without  inward,  are  as  follows.  There  is  first  a  thin, 
stratified  layer  of  epithelium,  continuous  with  that  of  the  conjunctiva, 
and  similar  to  that  of  the  skin,  save  that  there  is  no  stratum  corneum. 
Next  comes  the  anterior  limiting,  or  Bowman's,  membrane,  a  clear, 
anhistous  sheet,  which  sends  fibers  of  its  substance  into  the  deeper  parts. 
The  main  portion  of  the  cornea  is  composed  of  from  sixty  to  sixty-five 
lamellae  of  white  fibrous  tissue,  the  bundles  of  which  are  somewhat 
flattened  and  arranged  practically  at  right  angles  to  each  other.  Be- 


618  THE  CORNEA 

tween  the  bundles  are  numerous  lymph-spaces  or  lacunae,  communicating 
with  each  other  by  means  of  delicate  canaliculi,  and  continuous  with 
similar  spaces  in  the  sclera.  In  the  lacunse  are  to  be  found  the  so-called 
"corneal  corpuscles,"  fixed  cells  which  send  delicate  processes  into  the 
canaliculi,  recalling  in  appearance  the  osteoblasts  of  bone.  Besides 
these,  wandering  cells  may  be  seen.  Next  comes  the  posterior  limiting, 
or  Descemet's,  membrane,  a  clear,  structureless  sheet.  At  the  edge  of 
the  cornea  it  is  thicker,  forming  a  ring-like  zone  (the  annular  ligament), 
and  may  be  traced  as  far  as  the  insertion  of  the  iris  as  separate  bundles 
of  fibers  (the  pectinate  ligament),  bounded  by  minute  clefts  (the  spaces  of 
Fontana).  The  posterior  component  of  the  cornea  is  a  single  layer  of 
flattened,  endothelial  cells,  similar  to  those  lining  serous  cavities,  and 
continuous  with  those  covering  the  anterior  aspect  of  the  iris. 

The  normal  cornea  is  avascular,  being  nourished  by  the  lymph  system 
above  referred  to.  Bloodvessels  in  the  conjunctiva  and  subconjunctival 
tissues  and  in  the  sclera  send  out  fine  branches  which  pass  in  radially 
in  the  direction  of  the  cornea.  These  branch  dichotomously,  and  form 
an  elaborate  series  of  anastomosing  loops  in  the  limbus  conjunctive, 
but  do  not  encroach  upon  the  cornea.  The  structure  is  so  constituted  as 
to  be  perfectly  transparent,  all  its  components  possessing  the  same 
refractive  index. 

From  its  exposed  position  the  cornea  is  particularly  liable  to  injury 
and  irritation,  and,  being  avascular,  is  apt  to  suffer  in  all  conditions 
of  lowered  vitality  of  the  system.  Under  ordinary  circumstances  it  is 
able  to  deal  with  moderate  injuries  and  grades  of  inflammation  without 
suffering  much  in  the  process,  but  all  severe  processes  of  this  kind  lead 
to  extensive  and  permanent  changes,  often  producing  marked  inter- 
ference with  the  function  of  vision.  Thus,  opacity  in  the  pupillary  area 
interposes  a  physical  impediment  in  the  visual  axis,  and  cicatrices  of 
the  cornea  result  in  alterations  in  the  angle  of  refraction. 

CONGENITAL  ANOMALIES. 

Congenital  abnormalities  of  the  cornea  are  often  associated  with  other 
defects  of  the  eye.  The  cornea  may  be  smaller  than  normal,  as  in 
microphthalmus.  In  this  case  it  may  also  be  somewhat  flattened,  or  its 
curvature  may  be  the  same  as  that  of  the  sclera,  and  its  outline  may 
depart  more  or  less  widely  from  the  normal.  The  cornea  is  larger  than 
normal  in  megalophthalmus,  and,  moreover,  may  be  thinner  than  usual. 
The  anterior  portion  of  the  sclerotic  is  also  thinner  than  normal,  giving 
it  a  bluish  appearance.  In  such  cases  the  anterior  chamber  is  usually 
increased  in  depth  (hydrophthalmus  anterior).  Partial  or  complete 
opacity  of  the  cornea  is  also  sometimes  observed,  and  is  a  frequent 
accompaniment  of  microphthalmus,  megalophthalmus,  and  hydroph- 
thalmus. A  condition  resembling  the  arcus  senilis  is  occasionally  met 
with  at  birth — embryotoxon. 

Congenital  abnormalities  in  the  curvature  of  the  cornea  are  the 
cause  of  many  cases  of  astigmatism. 


KERATITIS  619 


INFLAMMATIONS. 

Keratitis. — The  various  forms  of  inflammation  of  the  cornea,  known 
as  keratitis,  are  of  great  practical  importance,  and,  owing  to  the  anatom- 
ical peculiarities  of  the  part,  present  certain  features  which  differ  from 
those  found  in  inflammations  occurring  in  other  parts  of  the  body. 

Being  an  avascular  structure,  the  cornea  is  at  some  distance  from  its 
base  of  supplies,  and  is,  therefore,  comparatively  poorly  nourished. 
It  is,  consequently,  but  imperfectly  able  to  resist  acute  disease,  and  is 
particularly  liable  to  suffer  from  the  deleterious  influence  of  impoverished 
blood  or  circulating  toxins.  Ulceration  of  the  cornea  may  always  be 
taken  as  an  evidence  of  deficient  vitality  of  the  general  system.  Acute 
infectious  keratitis,  therefore,  if  not  checked  by  treatment,  is  apt  to 
proceed  apace  and  bring  about  serious  damage  to  the  cornea,  while 
the  more  chronic  affections  are  liable  to  be  sluggish. 

Again,  owing  to  its  exposed  position,  the  cornea  is  particularly  ex- 
posed to  traumatism  and  irritation  of  various  kinds,  and  especially  to 
infection. 

Finally,  any  condition  which  interferes  with  the  transparency  of  the 
structure,  or  its  normal  curvature,  will  seriously  impair  its  function 
as  a  refractive  medium. 

Keratitis  may  be  described  as  partial  or  circumscribed,  generalized 
or  diffuse.  As  a  rule,  the  corneal  substance  proper  is  affected,  but  the 
epithelium  on  the  outer  and  inner  aspects  may  alone  be  involved. 
Keratitis,  moreover,  may  be  primary  or  secondary  to  disease  of  the  con- 
junctiva or  other  parts  of  the  eye,  as,  for  instance,  the  iris — iridokeratitis , 
or  sclera — sclerokeratitis ,  or,  again,  may  be  an  expression  of  some  con- 
stitutional deficiency  or  taint,  such  as  syphilis. 

The  changes  in  the  cornea  produced  by  inflammation  differ  somewhat 
according  to  the  nature,  the  localization,  and  the  intensity  of  the  process. 
Three  main  types  are  usually  described,  infiltration,  abscess,  and  ulcera- 
tion. 

Inflammation  of  the  cornea  (keratitis)  always  results  in  diminution  or 
loss  of  its  transparency,  the  degree  depending  on  the  extent  and  the 
severity  of  the  condition.  Should  the  anterior  layer  of  epithelium  be  in- 
volved, the  cornea  has  a  steamy,  pitted  appearance,  somewhat  resembling 
a  mirror  that  has  been  breathed  upon.  Thickening  of  the  epithelium, 
and,  to  a  greater  degree,  infiltration,  causes  the  cornea  to  assume  a 
milky,  somewhat  opalescent  appearance,  passing  on  into  a  whitish, 
grayish,  or  yellowish  opacity.  This  loss  of  transparency  is  due  to  an 
excessive  accumulation  of  leukocytes  within  the  lacunae  and  canaliculi, 
the  result  of  chemotaxis.  The  infiltration  may  be  superficial  or  deeply 
seated,  localized  or  diffuse.  Slight  grades  may  resolve,  leaving  the 
cornea  little  or  none  the  worse,  but  it  is  by  no  means  uncommon  to 
find  some  degree  of  opacity  persisting.  The  condition  may,  however,  be 
so  intense  as  to  give  rise  to  ulceration  or  abscess  with  distinct  loss  of 
substance,  which,  on  healing,  results  in  the  formation  of  a  fibrous  scar. 


620 


THE  CORNEA 


Prolonged  keratitis  of  moderate  severity  frequently  results  in  opacity, 
more  or  less  fibrosis,  and  vascularization  of  the  cornea. 

An  abscess  is  a  circumscribed  infiltration  in  which  the  nutrition  of 
the  part  has  been  so  interfered  with  that  local  death  has  resulted.  It 
may  extend  to  the  surface  of  the  cornea  and  discharge  externally, 
forming  an  ulcer;  more  rarely,  it  may  evacuate  itself  into  the  anterior 
chamber. 

Ulcers  may  be  the  result  of  infiltration  or  abscess,  or  may  exist  as 
such  from  the  first.  Perforation  of  the  cornea  may  occur  or  the  structure 
may  be  almost  wholly  destroyed.  Adhesion  and  prolapse  of  the  iris  are 
not  uncommon  results.  In  not  a  few  instances,  the  inflammation  leads 


FIG.  174 


Mild  grade  of  inflammation  of  the  cornea  in  man  (keratitis  e  lagophthalmo),  characterized  by 
enlargement  and  direct  division  of  the  nuclei  of  the  corneal  corpuscles  c,  with  but  slight  invasion 
of  polynuclear  leukocytes  p,  and  lymphocytes  I.  (Tooke.) 

to  the  deposition  of  pus  in  the  anterior  chamber  (hypopyon).  Should 
the  process  heal,  the  loss  of  tissue  is  made  good  by  the  process  of  cica- 
/trization.  Milder  grades  are  followed  by  slight  opacity  (nebula),  more 
severe  forms  by  the  formation  of  a  dense,  fibrous,  pearly  scar  (leukoma). 
The  reparative  material  being  softer  than  the  normal  corneal  substance 
frequently  gives  way  under  the  intraocular  pressure  and  forms  an 
anterior  protrusion  (corneal  staphyloma).  Sometimes,  again,  the  loss 
of  substance  is  not  entirely  repaired  and  the  surface  of  the  healed  ulcer 
does  not  quite  reach  the  general  level  of  the  cornea,  while  the  regularity 
of  the  curve  is  disturbed  (corneal  facet). 

Diffuse  Keratitis. — Diffuse  (interstitial  or  parenchymatous)  keratitis 
is  characterized  by  a  more  or  less  uniform  inflammatory  infiltration  of 


KERATITIS  BULLOSA  621 

the  cornea  throughout  its  whole  thickness,  which  exhibits  no  tendency 
toward  ulceration  or  abscess-formation.  The  epithelium  presents  a 
stippled  appearance  and  the  underlying  cornea  is  opaque,  somewhat 
resembling  ground  glass.  The  process  generally  begins  with  some 
ciliary  congestion,  followed  by  the  formation  of  nebulous  patches  in 
the  cornea,  which  gradually  extend  until  the  whole  structure  becomes 
involved.  Delicate,  closely-set  vessels,  derived  from  branches  of  the 
ciliary  vessels,  gradually  make  their  way  from  the  periphery  into  the 
cornea,  which  thus  becomes  both  opaque  and  highly  vascularized 
(vascular  keratitis).  These  newly-formed  vessels  are  in  the  substance 
of  the  cornea  and,  consequently,  present  a  dull,  reddish-pink  color 
("salmon  patches"  of  Hutchinson).  Salmon  patches,  when  small,  are 
often  crescentic  in  shape,  but  when  larger  tend  to  assume  the  form  of 
a  sector.  In  other  cases  a  narrow  fringe  of  vessels  is  formed,  continuous 
with  the  plexus  at  the  corneal  margin.  This  is  one  form  of  marginal 
keratitis.  Mixed  forms  are,  however,  not  infrequent.  As  a  rule,  the 
condition  is  bilateral,  but  days  or  weeks  may  elapse  before  the  involve- 
ment of  the  second  eye.  The  affection  is  a  constitutional  one,  and, 
as  a  rule,  due  to  syphilis.  It  is  said  to  be  met  with  also  in  strumous 
and  gouty  individuals.  The  disease  runs  a  chronic  course  and  relapses 
are  common.  Complete  restoration  of  the  transparency  of  the  cornea 
rarely  occurs.  Cases  of  syphilitic  nature  are  usually  also  complicated 
with  iritis  or  iridochoroiditis. 

Vascular  keratitis  may,  however,  at  times  be  much  more  superficial 
and  come  on  without  obvious  cause.  It  may  also  be  secondary  to  tracho- 
matous  conjunctivitis  or  repeated  attacks  of  phlyctenular  keratitis. 

The  superficial  epithelium  becomes  irregular  from  erosion  and  hyper- 
trophic  overgrowth,  while  new  vessels  are  formed  more  or  less  abun- 
dantly between  the  epithelial  layer  and  the  Bowman's  membrane  and  in 
the  corneal  substance  itself  (pannus).  When  the  newly  formed  blood- 
vessels are  few  and  scattered,  the  condition  is  spoken  of  as  pannus 
tennis;  when  so  numerous  as  to  give  the  cornea  a  reddish  appearance, 
pannus  crass  us. 

Neuroparalytic  Keratitis. — Neuroparalytic  keratitis  is  a  form  of  diffuse 
keratitis  of  rather  sluggish  character,  found  in  cases  where  the  function 
of  the  fifth  nerve  is  impaired  or  destroyed.  Symptoms  are  largely  in 
abeyance  owing  to  the  insensibility  of  the  cornea.  There  may  be  merely 
infiltration  of  the  tissue,  but  the  process  is  very  apt  to  go  on  to  suppura- 
tion. The  pericorneal  congestion  is  comparatively  slight. 

Keratitis  Bullosa. — Keratitis  bullosa  is  a  rare  disease,  characterized 
by  the  formation  in  rapid  succession  of  transparent  vesicles  on  the 
surface  of  the  cornea,  accompanied  by  marked  paroxysms  of  pain.  The 
vesicles  are  comparatively  large,  single  or  multiple.  They  may  reach 
4  to  5  mm.  in  diameter.  When  removed,  the  underlying  cornea  is  found 
to  be  cloudy  and  the  seat  of  parenchymatous  keratitis.  As  a  rule, 
the  eye  is  diseased  in  other  ways;  for  instance,  from  glaucoma  or  old 
iridochoroiditis.  The  condition  is  probably  due  to  some  disturbance 
of  the  lymph-channels  of  the  cornea. 


622  THE  CORNEA 

Not  unlike  this  affection  in  some  particulars  is  herpes  of  the  cornea. 
Here,  one  or  more  small  vesicles,  containing  clear  transparent  fluid,  form 
on  the  surface  of  the  cornea.  When  ruptured  an  excoriated  surface  is 
left.  Severe  neuralgic  pains  accompany  the  eruption  of  the  blisters. 
There  is  often  marked  pericorneal  injection.  Herpes  of  the  cornea 
may  come  on  without  obvious  cause,  or  may  be  associated  with  catarrh 
of  the  respiratory  passages.  In  herpes  zoster  ophthalmicus ,  which  is 
probably  due  to  some  inflammatory  disorder  of  the  fifth  nerve,  ulceration 
and  infiltration  of  the  cornea  are  quite  marked  and  the  condition  is 
slow  in  healing.  The  eruption  of  vesicles  occurs  in  the  district  supplied 
by  the  fifth  nerve  and  is  accompanied  by  much  pain  and  local  anesthesia. 
The  cornea  is  apt  to  be  involved  only  in  those  cases  in  which  the  nasal 
branch  of  the  first  division  of  the  fifth  is  affected.  Complication  with 
iritis  and  hypopyon  is  not  uncommon  in  this  form  of  herpes. 

Ulcerative  Keratitis. — Several  varieties  of  corneal  ulceration  are 
recognized.  The  simplest  form  is  the  small  central  ulcer,  met  with  in 
young  badly-nourished  children.  It  begins  as  a  small,  grayish-white 
elevation  at  or  near  the  centre  of  the  cornea.  Sooner  or  later  this 
breaks  down  in  the  centre,  forming  a  minute  excavation.  The  process 
appears  to  be  somewhat  sluggish,  as  the  congestion  is  slight  and  the 
symptoms  usually  unobtrusive.  The  ulcer  is  most  often  single,  but  is  apt 
to  recur,  or  the  other  eye  may  become  involved.  Occasionally,  we  find, 
in  anemic  or  strumous  patients,  somewhat  similar  ulcers,  but  even  more 
sluggish,  which  run  a  chronic  course  with  frequent  relapses.  There  is 
little  or  no  infiltration  and  the  loss  of  substance  is  only  imperfectly  made 
good,  so  that  a  shallow  depression  or  a  flat  facet  is  apt  to  be  left,  but 
without  much  damage  to  the  transparency  of  the  cornea. 

Catarrhal  ulcers  are  not  infrequently  met  with  as  a  result  of  catarrhal 
conjunctivitis  in  elderly  people.  The  ulcer  usually  forms  at  or  near  the 
margin  of  the  cornea  as  a  shallow  sulcus  or  there  may  be  several  minute 
delicate  abrasions  of  the  surface.  There  is  a  moderate  amount  of  peri- 
corneal congestion.  The  ulcer  usually  heals  readily,  unless  it  become 
infected,  when  serious  suppurative  inflammation  may  supervene. 

Phlyctenular  ulceration  is  closely  related  to  phlyctenular  conjunctivitis 
(q.  v.)  and  is  often  associated  with  diffuse  conjunctivitis.  It  begins 
with  one  or  more  superficial  infiltrations  about  the  size  of  a  millet  seed, 
either  on  the  white  of  the  eye  near  the  cornea,  or  just  within  the  corneal 
margin,  or  upon  some  other  part  of  the  cornea.  The  papule  is  circular, 
surrounded  by  a  zone  of  congestion  and  opacity,  and  may  assume  the 
appearance  of  an  acne  pustule.  The  epithelium  is  soon  destroyed,  and 
a  small  abrasion  or  aphthous-looking  ulcer  is  the  result.  Pericorneal 
congestion  is  always  present  and  may  be  marked.  Phlyctenular  ulcers 
tend  to  advance  in  an  almost  radial  direction  toward  the  centre  of  the 
cornea,  carrying  with  them  a  leash  of  vessels  lying  upon  the  track  of 
opacity  left  by  the  ulcer  (phlyctenular  pannus).  When  the  process  ceases 
the  vessels  gradually  disappear  but  more  or  less  opacity  remains.  If  this 
be  present  at  or  near  the  centre  of  the  cornea,  considerable  disturbance 
of  vision  will  result.  Occasionally,  the  condition  develops  into  a  suppu- 


ABSCESSES  623 

rative  keratitis  and  may  perforate.     In  extreme  cases,  the  inflammation 
spreads  to  the  vitreous  and  may  destroy  the  eye. 

Phlyctenular  ulcers  are  met  with  usually  in  children,  sometimes  in 
apparently  good  health  otherwise,  but,  as  a  rule,  the  subjects  are  strumous 
or  suffer  from  grave  errors  in  nutrition.  Not  infrequently,  there  is  a 
historv  of  measles  or  some  other  infectious  disease. 

Crescentic  ulcers,  close  to,  or  actually  upon,  an  arcus  senilis,  are  some- 
times met  with  in  elderly  debilitated  subjects.  They  may  form  deep 
grooves  around  the  cornea,  which,  if  it  be  cut  off  from  its  nutrition,  may 
thereupon  exfoliate. 

Suppurative  Keratitis. — There  are  certain  forms  of  suppurative 
keratitis  which  should  be  referred  to.  Abscess  or  infective  ulcers  may 
originate  spontaneously  or  may  result  from  some  trifling  injury.  Prob- 
ably the  condition  arises  from  the  infection  of  the  cornea  rendered 
possible  by  the  preexisting  injury,  and  assisted  by  debility  and  malnu- 
trition of  the  patient.  Such  a  condition  tends  to  spread  in  one  direction 
while  healing  in  another,  is  unattended  by  the  formation  of  new  vessels, 
so  characteristic  of  healing  inflammation,  and  is  frequently  complicated 
by  hypopyon. 

The  Acute  Serpiginous  Ulcer. — Saemisch  has  described  what  he  calls 
the  acute  serpiginous  ulcer,  a  form  which  tends  to  penetrate  deeply  and 
extend  widely,  especially  in  one  direction.  It  begins  as  a  grayish  spot, 
presenting  slight  ulceration,  and  having  a  sharply-cut  border,  one  part 
of  which  is  more  opaque  than  the  rest.  The  process  is  apt  to  extend 
rapidly,  and  may  lead  to  perforation  of  the  cornea.  Iritis  and  hypopyon 
are  common  complications.  Subacute  and  chronic  serpiginous  ulcera- 
tion is  also  described. 

Keratomalacia. — The  so-called  keratomalacia,  met  with  in  infants 
suffering  from  digestive  disturbances,  is  a  severe  form  of  suppurative 
keratitis,  due  to  infection  with  pyogenic  cocci,  notably  the  Staphy- 
lococcus  aureus.  The  affection  begins  apparently  from  the  infection 
of  some  small  fissure  or  abrasion  of  the  cornea,  which  rapidly  develops 
into  an  ulcer  extending  laterally  and  deeply  into  the  substance  of  the 
cornea.  This  ulcer  has  a  grayish  base  and  yellowish  edge  and  tends  to 
perforate  the  cornea. 

Abscesses. — Abscesses  of  the  cornea  are  probably  also  in  all  cases 
the  result  of  infection.  They  may  occur  as  a  primary  infection,  or  may 
result  from  simple  inflammatory  infiltration,  from  traumatism,  or  ulcera- 
tion. They  usually  form  at  the  centre  of  the  cornea  as  small,  raised  spots 
surrounded  by  a  deeply  congested  area.  The  spots  enlarge  rapidly 
and  break  down,  usually  discharging  forward,  thus  producing  an  ulcer 
covered  with  seropurulent  exudation.  When  perforation  of  the  cornea 
occurs  posteriorly,  hypopyon  results. 

In  some  cases  of  iritis  the  lower  part  of  the  cornea  becomes  secondarily 
involved,  appearing  somewhat  hazy.  Not  infrequently,  a  number  of 
minute  dots,  discrete,  sharply  defined,  and  of  a  whitish  or  grayish-white 
color,  may  be  detected,  especially  by  the  use  of  a  lens,  on  the  posterior 
elastic  lamina.  These  dots  are  arranged  in  the  form  of  a  sector  with 


624  THE  CORNEA 

the  apex  toward  the  centre  of  the  cornea,  the  smallest  dots  being  near 
the  centre  (keratitis  punctata).  Keratitis  punctata  is  nearly  always  the 
result  of  some  affection  of  the  cornea,  iris,  choroid,  or  vitreous. 

The  Results  of  Suppurative  Keratitis. — Ulcers  of  the  cornea,  when  they 
heal,  not  uncommonly  leave  behind  them  traces  in  the  form  of  permanent 
opacities  of  the  tissue  or  even  scars.  These,  if  situated  over  the  pupil 
of  the  eye,  may  seriously  interfere  with  vision,  not  only  by  introducing 
an  opaque  substance  in  the  visual  axis,  but  by  altering  the  curvature  of 
the  refracting  medium.  In  severe  cases  of  ulceration,  the  whole  or  the 
greater  part  of  the  cornea  may  be  destroyed,  and  the  inflammation  may 
spread  to  the  iris,  choroid,  and  the  humors,  leading  to  total  destruction 
of  the  eye. 

Smaller  ulcers  may  perforate  and  lead  to  the  escape  of  the  aqueous 
humor  through  the  opening.  Occasionally  a  permanent  fistula  may 
result.  This,  according  to  De  Wecker,  is  due  to  the  eversion  of  Desce- 
met's  membrane,  which  forms  a  lining  to  the  fistulous  track.  Occasion- 
ally, where  perforation  is  not  quite  complete,  Descemet's  membrane  may 
prolapse  and  present  as  a  small,  clear  vesicle,  resembling  a  glass  bead, 
in  the  base  of  the  ulcer. 

Prolapse  of  the  iris  and  adhesion  of  the  iris  to  the  region  of  the  ulcer 
may  occur. 

Hypopyon. — Hypopyon,  or  pus  in  the  anterior  chamber,  may  occur 
with  any  ulcer,  whether  it  has  perforated  or  not,  and  with  any  suppurative 
condition  of  the  cornea.  The  pus  may  be  derived  from  an  abscess  or 
ulcer  which  has  eroded  through  to  the  posterior  surface  of  the  cornea, 
or,  occasionally,  may  be  due  to  the  extension  of  inflammation  from  the 
iris.  In  some  severe  cases  of  suppurative  keratitis  the  pus  sinks  down 
between  the  lamellae  of  the  cornea  (onyx).  Onyx  and  hypopyon  may 
co-exist. 

Specific  Keratitis. — Tuberculosis,  syphilis,  and  leprosy  only  rarely  give 
rise  to  circumscribed  lesions  in  the  cornea. 

Keratomycosis  aspergillina  has  been  described,  but  is  very  rare.1 


RETROGRESSIVE  METAMORPHOSES. 

ArcilS  Senilis. — Arcus  senilis  (gerontoxon)  is  a  degenerative  change 
in  the  cornea,  found  in  elderly  people.  The  condition  begins  with 
the  formation  of  a  light  gray  arc  at  the  periphery  of  the  cornea.  It 
begins  both  above  and  below,  the  two  arcs  gradually  extending  until  a 
complete  circle  is  produced.  The  ring  is  sharply  defined  from  the  lim- 
bus  and  is  separated  from  it  by  a  narrow  transparent  band,  while  it 
gradually  loses  itself  on  the  concave  side  in  the  clear  cornea.  The  arcus 
is  at  first  silvery  gray  in  appearance,  but  later  becomes  denser  and  creamy. 
Both  eyes  are  usually  affected,  although  the  condition  may  be  unilateral. 
According  to  Fuchs,  the  condition  is  not,  as  has  usually  been  taught, 

1  Leber,  v.  Graefe's  Arch.,  25:  Die  Entstehung  der  Entziindung,  Leipzig,  1891. 


TRAUMATIC  DISTURBANCES  625 

a  fatty  degeneration  or  infiltration  of  the  cornea,  but  a  hyaline  degenera- 
tion of  certain  of  the  connective-tissue  fibers.  This  is  associated  with 
the  deposit  of  lime  salts  in  minute  particles  in  the  superficial  layers  of 
the  cornea  near  the  limbus.  The  condition  is  to  be  attributed,  no  doubt, 
to  the  impoverished  nutrition  of  the  cornea  due  to  senile  changes  in  the 
vascular  loops  encircling  the  cornea. 

Calcareous  Degeneration. — Calcareous  degeneration  of  the  cornea 
is  met  with  occasionally  in  the  form  of  a  transverse  band  of  opacity, 
corresponding  with  the  palpebral  fissure.  This  is  of  a  grayish  or  yellow- 
ish color,  2  to  3  mm.  broad,  and  is  found  in  elderly  or  prematurely  aged 
people.  It  is  also  met  with  in  eyes  affected  with  deeply-seated  disease, 
and  in  those  with  a  tendency  to  glaucoma. 

Pigmentation. — Lead  stains  not  infrequently  occur  after  the  applica- 
tion of  lotions  containing  salts  of  lead  to  an  abraded  or  ulcerated  cornea. 
The  stains  are  dense,  white,  opaque,  and  sharply  defined. 

PROGRESSIVE  METAMORPHOSES. 

Tumors. — Primary  tumors  of  the  cornea  appear  to  be  unknown. 
Tumors  of  the  conjunctiva,  especially  those  which  spring  from  the 
sclerocorneal  junction,  such  as  the  papilloma,  the  dermoid,  the  epithe- 
lioma,  and  the  sarcoma,  may  invade  the  cornea. 

TRAUMATIC  DISTURBANCES. 

The  cornea  being  firm  and  resistant,  and  supported  by  an  elastic 
cushion,  is  never  ruptured  by  a  blow  or  by  any  sudden  increase  of  intra- 
ocular tension.  It  may,  however,  be  injured  by  the  impact  of  foreign 
bodies,  by  abrasion,  incised  or  punctured  wounds,  burns,  scalds,  or 
caustic  substances. 

Blows  upon  the  cornea  from  small  bodies  may  result  in  loss  of  the 
superficial  epithelium,  bruising,  or  even  necrosis  of  the  part.  In  the 
latter  event,  from  the  consecutive  inflammation  and  infection  which  is 
so  liable  to  occur,  a  corneal  abscess  often  results.  The  various  inter- 
stices and  lymph-spaces  become  infiltrated  with  serum  and  pus  cells. 
The  conjunctiva  is  reddened,  and  there  is  ciliary  congestion.  In  a 
few  days  the  injured  part  becomes  opaque  and  of  a  grayish  color.  The 
pus  may  form  in  the  superficial  layers  or  may  extend  more  deeply.  It 
may,  therefore,  discharge  externally,  internally,  or  in  both  directions. 
Pus  may  collect  in  the  anterior  chamber  (hypopyon),  and  the  condition 
may  lead  to  iritis  and  iridocyclitis.  If  the  abscess  discharge  externally, 
the  lens  or  iris  is  apt  to  adhere  to  the  damaged  region  and  give  rise 
to  subsequent  trouble  in  the  form  of  irritation  and  inflammation,  perhaps 
resulting  eventually  in  panophthalmitis  and  phthisis  bulbi.  Anterior 
staphyloma  is  also  a  not  uncommon  result.  Should  the  abscess  heal, 
a  dense,  opaque,  fibrous  scar  is  formed,  which,  according  to  itsjposition, 
will  cause  more  or  less  interference  with  vision.  j^gj 

40 


626  THE  SOLERA 

Slight  wounds,  where  there  is  merely  loss  of  the  superficial  epithelium 
or  of  a  trifling  portion  of  the  deeper  corneal  substance,  heal  up  without 
much  trouble  and  without  any  serious  after-consequences,  except,  pos- 
sibly, in  the  case  of  weakly  or  debilitated  persons  and  from  lack  of  surgical 
cleanliness.  Unless  the  injury  extend  to  Descemet's  membrane  no  scar- 
ring will  result,  but  not  infrequently  the  curvature  of  the  cornea  is  altered 
and  the  refracting  power  of  the  structure  correspondingly  interfered  with, 
a  point  of  great  practical  importance  when  the  injury  is  at  or  near  the 
visual  centre. 

In  the  case  of  larger  wounds,  the  vitreous  may  escape  and  the  iris 
and  lens  become  attached  to  or  incarcerated  in  the  wound,  or  may  even 
be  prolapsed  through  it.  There  is  usually  considerable  loss  of  tissue, 
with  the  formation  of  a  large  cicatrix,  to  which  the  iris  and  lens  may  be 
permanently  attached. 

Burns,  scalds,  or  caustic  erosions  are  more  serious  than  similar  in- 
juries to  the  conjunctiva,  inasmuch  as  they  lead  to  considerable  reaction- 
ary inflammation,  with  opacity  or  scarring  of  the  cornea,  and  even 
symblepharon. 

The  Sclera. 

Scleritis. — The  disorders  of  the  sclerotic  membrane  are  compara- 
tively few.  The  most  important  is  inflammation — scleritis  (episcleritis). 
This  is  much  rarer  than  keratitis  and  usually  involves  the  anterior  half 
of  the  membrane.  It  may  exist  alone  or  in  association  with  inflam- 
mation of  the  cornea  (keratoscleritis),  iris,  or  choroid  (uveoscleritis). 
fc»  Simple  scleritis  occurs  usually  on  the  exposed  portions  of  the  ciliary 
region,  generally  to  the  outer  side,  but  it  may  be  found  at  any  part  of 
the  circle,  and  may,  exceptionally,  extend  widely  and  far  back  out  of 
sight.  The  affection  is  subacute  in  character  and  relapses  are  the 
rule.  The  disease  is  generally  met  with  in  adults,  especially  in  those 
exposed  to  cold,  or  who  have  a  gouty  or  rheumatic  tendency. 

The  process  begins  with  one  or  more  patches  of  congestion  in  the 
ciliary  region,  accompanied  by  swelling,  and  leading  to  elevation  of 
trie  conjunctiva.  The  affected  area  appears  reddish  and  rusty.  The 
conjunctiva  overlying  the  part  is  swollen,  cedematous,  and  congested. 

Microscopically,  one  finds  infiltration  of  the  tissue  with  leukocytes, 
especially  in  the  neighborhood  of  the  vessels,  with  some  dilatation  of  the 
lymphatics.  The  inflammation  may  subside  after  a  longer  or  shorter 
period,  resolving  entirely  or  leaving  a  grayish  discoloration  of  the  sclera. 
Occasionally,  the  inflammatory  infiltration  extends  more  or  less  widely 
into  the  cornea.  Scleritis  may  also  set  up  diffuse,  interstitial  keratitis 
or  chronic  iritis,  or,  again,  choroiditis.  Sclerochoroiditis  leads  to 
thinning  of  the  tunics  of  the  eyeball,  with  ectasia  or  dilatation  of  the 
anterior  part  (staphyloma  scleroe). 

Tuberculosis. — Tuberculosis  of  the  sclera  has  only  rarely  been  ob- 
served. 

Syphilis. — Syphilis,  especially  the  gumma,  is  somewhat  more  common, 


PERSISTENT  PUPILLARY  MEMBRANE  627 


The  Iris. 

The  iris  is  composed  of  five  layers.  These  are,  from  before  backward: 
(1)  The  anterior  epithelial  layer,  consisting  of  transparent,  flattened,  or 
polyhedral  cells,  having  a  spherical  or  slightly  oval  nucleus;  (2)  a  delicate, 
hyaline  basement  membrane,  continuous  with  the  Descemet's  membrane 
of  the  cornea;  (3)  the  substantia  propria,  composed  largely  of  bundles 
of  fibrous  connective  tissue  and  bloodvessels;  (4)  a  hyaline  elastic  mem- 
brane, extending  over  the  ciliary  processes  and  the  choroid  as  the  lamina 
vitrea;  (5)  the  posterior  layer  of  epithelium,  composed  of  polyhedral 
cells. 

The  substantia  propria  contains  in  addition  both  circular  (sphincter) 
bundles  of  unstriped  muscle,  and  radiating  fibers  (dilator  pupillae). 
These  are  under  the  control  of  different  sets  of  sympathetic  nerve  fibers, 
hence  it  is  that  the  condition  of  the  pupil  in  regard  to  contraction  or 
dilatation  is  of  great  diagnostic  value. 

The  color  of  the  iris  depends  upon  the  amount  of  pigment  which  it 
contains.  The  epithelial  layers  are  pigmented  at  birth,  but  the  stroma 
does  not  become  colored  until  later,  so  that  the  eyes  of  infants  are  always 
blue  or  gray.  In  the  blue  eye  of  the  adult  there  is  very  little  pigment 
in  the  stroma,  while  in  the  black  eye  all  the  layers  are  pigmented. 

CONGENITAL  ANOMALIES. 

Albinism. — In  albinism  there  is  a  marked  lack  of  pigment  in  the  iris 
as  \\ell  as  in  the  hair  and  other  structures  of  the  body.  The  iris  is  normal 
otherwise  in  structure,  but  appears  of  a  lilac,  rose,  or  yellowish-white 
color,  according  to  the  illumination.  The  pupil  is  always  narrow. 
Owing  to  the  lack  of  pigment  to  absorb  the  rays  of  light,  photophobia  is 
a  marked  symptom.  The  eyes  are  usually  almost  amblyopic,  and  nys- 
tagmus is  common.  The  condition  is  undoubtedly  due  to  a  defect  of 
development,  and  heredity  seems  to  play  an  important  role. 

Heterochromia. — The  irides  in  the  two  eyes  may  be  of  different 
color — heterochromia ;  for  example,  one  may  be  blue  and  the  other 
brown.  Or,  a  blue  iris  may  be  dotted  or  streaked  with  brown. 

Melanosis  Oculi. — The  iris,  together  with  other  parts  of  the  eye, 
the  conjunctiva,  sclera,  choroid,  optic  nerve,  may  contain  areas  of 
intense  deep  pigmentation,  analogous  to  the  pigmented  moles  of  the 
skin — melanosis  oculi.  These  may  provide  a  starting  point  for  malignant 
new-growths. 

Persistent  Pupillary  Membrane.— One  of  the  commonest  anomalies 
of  the  eyes  is  persistent  pupillary  membrane.  During  foetal  life,  the 
lens  is  surrounded  by  a  vascular  membrane,  the  tunica  vasculosa, 
the  bloodvessels  of  which  are  derived  from  branches  of  the  arteria 
centralis,  which  pass  around  the  edge  of  the  lens  and  anastomose  on  its 
anterior  aspect.  As  the  iris  is  formed,  its  vessels  unite  with  those  of  the 
tunica  vasculosa.  The  portion  of  the  tunica  occupying  what  is  event- 


628  THE  IRIS 

ually  to  be  the  pupil  is  called  the  pupillary  membrane.  As  a  rule,  at 
birth,  the  membrane  and  its  vessels  have  been  absorbed,  but  occasionally 
portions  of  them  persist  as  strands  of  tissue,  often  highly  pigmented, 
which  arise  from  the  anterior  surface  of  the  iris  and  project  into  the  pupil. 

Corectopia. — The  pupillary  opening  is  situated  normally  a  little  to 
the  nasal  side  of  the  central  point.  In  corectopia  the  pupil  is  displaced 
outward  and  upward,  and  is  small  and  irregular  in  outline  as  well.  The 
iris  may  be  otherwise  normal  and  react  perfectly  to  light,  and  in  such 
cases  the  condition  is  usually  unilateral.  Often,  however,  there  are  other 
congenital  defects  in  the  eye,  such  as  buphthalmos,  microphthalmos, 
coloboma  of  the  lid  or  iris,  or  albinism.  Not  infrequently,  there  is 
ectopia  of  the  lens. 

Dyscoria. — Dyscoria,  or  irregularity  of  the  pupil,  is  a  very  common 
condition.  It  is  due  either  to  posterior  synechia  from  foetal  iritis,  or  to 
a  proliferation  of  the  pigmented  epithelium  forming  the  posterior  covering 
of  the  iris. 

Polycoria. — Polycoria,  or  multiplicity  of  the  pupil,  does  not  occur  in 
the  sense  of  a  number  of  pupils,  each  surrounded  by  a  sphincter  muscle. 
The  term  is  commonly  employed,  however,  to  designate  the  condition 
in  which  an  iris  contains  a  number  of  openings  in  addition  to  the  normal 
pupil.  These  openings  usually  appear  as  radial  clefts,  but  may  occur 
at  the  periphery  of  the  iris.  The  appearance  of  polycoria  may  also  be 
produced  by  a  bridge-coloboma  of  the  iris,  or  a  persistent  pupillary 
membrane. 

Aniridia  or  Iridiremia. — Aniridia  or  iridiremia  may,  so  far  as  clin- 
ical examination  goes,  be  total  or  partial.  In  complete  aniridia  both 
eyes  are  involved,  as  a  rule.  The  incomplete  form  is  often  difficult 
to  distinguish  from  coloboma.  Other  congenital  peculiarities,  such 
as  microphthalmos,  ptosis,  persistent  hyaloid  artery,  may  be  present. 
The  most  frequent  complication  is  cataract,  but  corneal  and  vitreous 
opacities,  choroidal  atrophy,  and  detachment  of  the  retina  may  be  met 
with.  Luxation  of  the  lens  may  occur.  Glaucoma  is  another  not  un- 
common complication. 

Goloboma. — Coloboma  of  the  iris  is  one  of  the  most  common  develop- 
mental defects  of  the  eye.  The  cause  has  already  been  mentioned 
(see  p.  608).  In  this  condition  there  is  a  cleft  of  the  iris  which  extends 
into  the  pupil,  forming  with  it  a  pear-shaped  opening.  The  opening 
may  be  complete,  the  defect  extending  to  the  ciliary  border,  or  incom- 
plete, a  bridge  of  iris  remaining  at  the  apex  of  the  gap.  The  opening  is 
situated  downward,  or  downward  and  inward.  The  pupil  is  usually 
also  displaced  downward,  less  often  upward. 


CIRCULATORY  DISTURBANCES. 

Anemia. — Anemia  of  the  iris  occurs  in  all  general  systemic  conditions 
associated  with  anemia  or  loss  of  blood. 

Hyperemia. — Hyperemia  is  met  with  in  the  early  stages  of  iritis  and 
associated  with  tumors  of  the  iris. 


IRITIS  629 

INFLAMMATIONS. 

Iritis. — Inflammation  of  the  iris — iritis — may  occur  as  a  primary 
affection  or  may  be  secondary  to  inflammation  of  other  portions  of  the 
eye.  The  causes  are  local  or  constitutional. 

Among  the  local  causes  may  be  mentioned,  perforating  wounds  of  the 
eyeball,  especially  if  lacerated  and  complicated  with  injury  to  the  lens; 
injury  to  the  lens,  without  wound  of  the  iris,  and  with  only  slight  puncture 
of  the  cornea  (e.  g.,  traumatic  iritis  occurring  after  cataract  operation); 
superficial  wounds  and  abrasions  of  the  cornea;  blows  upon  the  eye; 
ulcers  and  other  forms  of  keratitis,  especially  those  complicated  with 
hypopyon;  and  deep-seated  disease  of  the  eye. 

The  chief  constitutional  causes  are  syphilis>  gout,  and  rheumatism. 
Iritis  may  also  complicate  the  acute  infections  and  other  diseases  which 
have  generalized  systemic  manifestations.  Gonorrhceal  iritis,  analogous 
to  gonorrhceal  arthritis,  is  occasionally  met  with. 

Besides  the  causes  mentioned  there  is  another,  viz.,  trophic  disturbance, 
to  which  certain  writers  are  disposed  to  attach  considerable  importance. 
Such  disturbances  may  be  reflex.  In  this  category  come  those  cases  of 
iritis  occurring  in  sympathetic  ophthalmitis,  in  herpes  zoster  ophthal- 
micus,  and  as  a  result  of  dental  or  uterine  irritation. 

Acute  iritis  may  be  divided  into  three  main  varieties,  according  to  the 
clinical  features,  plastic  iritis,  serous  iritis,  and  suppurative  iritis. 

Plastic  Iritis. — By  far  the  largest  number  of  cases  of  inflammation  of 
the  iris  are  to  be  included  under  the  term  plastic  iritis.  Most  cases  of 
rheumatic,  gouty,  traumatic,  and  sympathetic  iritis  belong  to  this  cate- 
gory. 

The  condition  is  manifested  by  congestion  of  the  whole  eyeball,  but 
particularly  of  the  anterior  ciliary  vessels,  and  the  conjunctival  twigs 
which  surround  the  cornea  (ciliary  and  pericorneal  injection).  This 
results  in  the  formation  of  a  pinkish  zone,  from  3  to  6  mm.  broad,  just 
outside  the  peripheral  margin  of  the  cornea.  The  iris  loses  its  clear, 
bright  appearance  and  becomes  somewhat  muddy,  turbid,  and  altered 
in  color.  An  important  feature  is  the  formation  of  an  inflammatory 
exudate  both  in  the  substance  and  on  the  surface  of  the  iris  which  leads 
to  the  formation  of  adhesions  between  the  iris  and  the  lens-capsule 
(posterior  synechia).  The  mobility  of  the  pupil  is,  therefore,  consider- 
ably interfered  with,  and  under  the  influence  of  atropine  the  opening 
may  be  extremely  irregular.  In  some  cases  the  whole  of  the  posterior 
surface  of  the  iris  becomes  adherent  (total  posterior  synechia).  In  the 
severer  forms,  the  exudate  may  be  very  abundant,  giving  rise  to  occlusion 
of  the  pupil,  turbidity  of  the  vitreous,  and  even  slight  opacity  of  the 
cornea.  The  vigorous  use  of  atropine  may,  in  cases  where  the  adhesions 
have  not  become  fully  organized,  result  in  breaking  them  down,  so  that 
the  pupil  again  becomes  round.  This  procedure,  however,  generally 
results  in  some  pigment  being  left  upon  the  capsule  of  the  lens.  Pigment 
in  this  situation  is  pathognomonic  of  present  or  past  iritis. 


630  THE  IRIS 

Serous  Iritis. — Serous  iritis  is  a  not  uncommon  affection,  the  pathogeny 
of  which  is  by  no  means  clear.  Some  cases  appear  to  be  dependent  on 
a  rheumatic  disposition,  while  others  are  reflex.  In  this  form  there  is 
a  special  tendency  for  the  whole  uveal  tract  to  be  involved.  The  disease 
generally  runs  a  subacute  course  and  is  not  always  very  amenable  to  treat- 
ment. Pericorneal  injection  may  be  trifling  and  the  iris  is  not  greatly 
altered  in  color.  The  tendency  to  the  formation  of  synechise  is  not  so 
great  as  in  the  plastic  form.  The  exudate  is  of  a  grayish  or  grayish- 
brown  color  and  is  found  in  the  form  of  fine  points  on  the  lower  half  of 
Descemet's  membrane.  The  vitreous  may  become  somewhat  cloudy 
and  contain  floating  opacities.  Not  infrequently  the  ciliary  body  and  the 
choroid  are  slightly  inflamed  (cyclitis;  iridochoroiditis).  It  should  be 
noted,  however,  that  cases  of  iritis  are  occasionally  met  with  which  are 
intermediate  in  type  between  the  plastic  and  the  serous  forms.  Thus, 
iritis,  which  to  gross  appearance  resembles  the  plastic  variety,  may 
be  associated  with  considerable  infiltration  of  the  iris  and  the  formation 
of  adhesions,  while  the  plastic  type  may  lead  to  deposits  of  lymph  on 
Descemet's  membrane. 

Suppurative  Iritis. — Suppurative  iritis  is  less  common  than  the  plastic 
variety.  It  may  supervene  upon  plastic  iritis,  but  is  usually  due  to 
trauma,  operations  opening  up  the  globe  of  the  eye,  and  to  ulcerative 
keratitis. 

The  inflammation  is  more  intense,  the  congestion  is  greater,  the  exu- 
date more  abundant.  The  pus  may  collect  in  the  anterior  chamber 
(hypopyon). 

Syphilis. — This  resembles  closely  in  anatomical  features  plastic  or 
serous  iritis,  but  mixed  forms  also  occur  here.  Sometimes  minute 
gummas,  from  2  to  6  mm.  in  diameter,  are  to  be  observed  upon  the 
iris,  and  by  microscopic  examination  even  those  forms  which  appear  to 
resemble  simple  inflammation  can  be  seen  to  be  of  granulomatous  type. 
Gummas  of  the  iris  do  not  differ  appreciably  from  those  elsewhere, 
and  are  made  up  of  young,  proliferating  connective-tissue  cells,  newly 
formed  and  congested  vessels,  and  the  ordinary  vascular  changes  charac- 
teristic of  syphilis. 

Tuberculosis. — Tuberculous  iritis  is  rare,  and,  unlike  syphilis,  tends 
to  affect  only  one  eye.  It  may  begin  in  the  form  of  serous  iritis,  but  small 
grayish  nodules  can  usually  be  seen  on  the  iris  near  the  ciliary  process^ 
and  in  Fontana's  space.  These  nodules  gradually  enlarge  and  finally 
coalesce,  so  that  we  get  a  warty,  grayish-red  mass  containing  fine  vessels, 
which  encroaches  more  or  less  upon  the  anterior  chamber.  The  cornea 
usually  shows  some  fogginess  and  vascularization.  The  process  at  times 
may  retrogress  and  finally  come  to  an  end,  but  very  commonly  the  in- 
filtration extends  to  the  ciliary  process  and  the  adjacent  sclera,  resulting 
in  caseation  and  total  destruction  of  the  eye. 


CYCLITIS  631 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  iris,  characterized  by  thinning  of  its  sub- 
stance, loss  of  pigment,  and  fibrous  transformation,  occurs  as  a  result 
of  inflammation,  especially  if  anterior  synechia  be  present,  and  in  chronic 
glaucoma.  The  bloodvessels  are  thickened  and  hyaline,  so  that  their 
lumina  are  often  obliterated. 

Pigmentation. — The  application  of  nitrate  of  silver  and  protargol 
to  the  conjunctiva  has  resulted  in  permanent  staining  (argyria  conjunc- 
tivcB).  The  membrane  becomes  of  an  olive  or  slaty  color.  A  similar 
result  has  been  reported  in  the  case  of  certain  persons,  who,  from  the 
nature  of  their  occupation,  have  been  exposed  to  the  action  of  silver  dust. 

In  cases  of  icterus,  whether  of  the  obstructive  or  toxic  forms,  the 
conjunctiva  is  invariably  involved.  In  fact,  so  early  and  so  characteristic 
is  the  staining  that  we  always  look  at  the  conjunctiva?  for  the  first  evidence 
of  jaundice.  The  "white"  of  the  eye  in  such  cases  presents  a  more  or 
less  intense  shade  of  yellow.  A  dull,  earthy,  or  subicteroid  coloration 
is  also  met  with  in  obstructive  cardiac  disease,  pernicious  anemia, 
toxemias,  and  in  the  cachexia  of  chronic  wasting  disease. 


PROGRESSIVE  METAMORPHOSES. 

Sarcoma. — Sarcoma,  usually  pigmented,  is  the  only  tumor  originating 
in  the  iris  and  is  the  rarest  form  of  intra-ocular  sarcoma.  Much  more 
often  sarcoma  originates  in  the  choroid. 

The  Ciliary  Body. 

Cyclitis. — Inflammation  of  the  ciliary  body — cyclitis — is  commonly 
associated  with  inflammation  of  the  iris  or  choroid.  The  process  is 
evidenced  by  slight  clouding  of  the  aqueous  humor  and  the  anterior 
portion  of  the  vitreous.  With  this  there  is  a  deposition  of  exudate  upon 
the  posterior  surface  of  the  cornea,  with  slight  exudation  into  the  pupillary 
area.  The  whole  posterior  aspect  of  the  iris  becomes  adherent  to  the 
capsule  of  the  lens  with  retraction  of  the  ciliary  portion  of  the  iris,  so 
that  the  anterior  chamber  becomes  enlarged.  The  exudation  which 
collects  between  the  iris  and  the  lens  and  between  the  periphery  of  the 
iris  and  the  ciliary  process  gradually  undergoes  organization  and  leads 
by  its  contraction  to  dislocation  of  the  iris  backward.  Similarly,  the 
exudation  before  and  behind  the  lens,  as  it  is  transformed  into  connective 
tissue,  leads  to  traction  upon  the  ciliary  body  away  from  the  sclera  and 
in  the  direction  of  the  axis  of  the  bulb.  The  involvement  of  the  vitreous, 
which  is  a  constant  accompaniment  of  cyclitis,  with  the  deposit  in  it  of 
a  cellular  and  fibrinous  exudation,  in  the  same  way  results  in  complete 
separation  of  the  retina  with  cataractous  transformation  of  the  lens. 
Severe  cyclitis  may  become  suppurative  and  lead  to  the  production  of 


632  THE  CHOROID 

hypopyon,  or  even  involvement  of  the  whole  uveal  tract  (panophthal- 
mitis).  Fibrinous  cyclitis,  inasmuch  as  it  is  not  so  severe  an  affection, 
leads  gradually  to  phthisis  bulbi,  with  more  or  less  diminution  of  the 
intra-ocular  tension. 

Apart  from  inflammations  which  spread  from  the  iris  or  choroid,  the 
chief  causes  of  cyclitis  are  injuries,  especially  such  as  are  due  to  wounds 
or  foreign  bodies.  Occasionally,  wounds  of  the  sclera  near  the  margin 
of  the  cornea,  when  cicatrizing,  cause  tension  upon  the  iris  and  ciliary 
body,  and  eventually  inflammation. 

One  of  the  most  important  consequences  of  cyclitis  and  iridocyclitis, 
especially  when  the  result  of  penetrating  wounds  of  the  tunics  of  the  eye, 
as,  for  instance,  stabs,  incisions,  or  of  rupture,  ulcers,  or  foreign  bodies, 
is  the  so-called  sympathetic  ophthalmitis,  which  affects  the  uninjured  eye. 

Sympathetic  ophthalmitis  usually  sets  in  from  six  to  twelve  weeks 
after  the  primary  injury  to  the  fellow  eye.  It  rarely  occurs  before  three 
weeks  after  the  injury,  and  exceptionally  its  appearance  is  delayed  for 
many  years.  '  The  process  tends  to  relapse,  and  may  continue  with 
alternate  exacerbations  and  ameliorations  for  months  or  even  a  year 
or  two.  Anatomically  speaking,  the  affection  takes  the  form  of  a 
plastic  iridocyclitis  or  iridochoroiditis  with  exudation  leading  to  total 
posterior  synechia.  In  the  early  stages  there  is  apt  to  be  a  dotted  deposit 
on  the  posterior  surface  of  the  cornea,  clouding  of  the  vitreous,  and  often 
neuroretinitis.  The  vessels  perforating  the  sclera  near  the  ciliary  region 
are  congested.  The  intra-ocular  tension  is  often  increased.  The  mildest 
%  cases  do  not  go  farther  than  a  chronic  serous  iritis,  with  keratitis  punctata 
and  disease  of  the  vitreous,  usually  also  with  neuroretinitis.  In  more 
severe  cases  the  eye  remains  glaucomatous,  with  total  posterior  synechia, 
corneal  opacity,  and  a  varying  amount  of  ciliary  staphyloma.  In  the 
worst  cases  the  eye  finally  shrinks. 


The  Choroid. 


CONGENITAL  ANOMALIES. 

Coloboma. — Coloboma  affecting  the  lower  part  of  the  choroid  may 
exist  alone  or  in  association  with  coloboma  of  the  iris.  Occasionally 
the  coloboma  is  limited  to  a  small  area  around  the  nerve,  or  it  may  be 
separate  from  it  (see  p.  608).  Albinism  has  also  already  been  suffi- 
ciently dealt  with. 


CIRCULATORY  DISTURBANCES. 

Hyperemia. — Hyperemia  of  the  choroid  is  not  infrequent.  It  occurs 
in  cases  of  systemic  passive  congestion,  congestion  of  the  head  itself,  and 
in  early  inflammation  of  the  choroid,  retina,  or  associated  parts. 

Anemia. — Anemia  is  met  with  in  general  anemia,  if  severe. 


CHOROIDITIS  633 

Hemorrhages. — Hemorrhages  into  the  choroid  in  the  form  of  multiple 
minute  extravasations  are  occasionally  met  with.  They  often  lead  to 
atrophy  and  pigmentation  of  the  membrane.  The  cause  is  obscure. 
Larger  hemorrhages  may  be  due  to  traumatism,  smaller  ones  to  disease 
of  the  vessels. 

INFLAMMATIONS. 

Choroiditis. — The  term  choroiditis  is  often  used  in  a  loose  way 
to  include  not  only  the  frankly  inflammatory  affections,  but  also  some 
forms  of  atrophy  which  are  by  no  means  closely,  if  it  all,  related  to  inflam- 
mation. We  shall,  however,  in  the  course  of  the  following  remarks, 
employ  it  in  the  strict  sense,  namely,  to  designate  inflammation  of  the 
choroid. 

Owing  to  the  close  relationship  that  exists  between  the  choroid  and  the 
retina,  disease  of  one  membrane  is  exceedingly  apt  to  extend  to  the 
other.  Thus,  changes  in  the  pigment  epithelium  which  forms  part  of 
the  retina  may  be  due  to  deep-seated  retinitis,  or,  again,  to  superficial 
choroiditis.  It  is,  therefore,  not  always  easy  to  determine  in  which  mem- 
brane the  inflammatory  process  has  begun.  Moreover,  the  retina, 
even  if  not  directly  implicated,  often  shows  secondary  atrophic  changes 
as  a  result  of  choroiditis.  On  the  other  hand,  in  cases  of  equally  severe 
choroidal  inflammation,  the  retina,  curiously  enough,  may  escape. 

The  causes  of  choroiditis  are  not  very  varied.  Some  few  cases  are 
considered  to  be  due  to  some  systemic  dyscrasia,  such  as  gout;  others 
are  due  to  traumatism;  others,  again,  and  by  far  the  larger  number, 
are  manifestations  of  infection,  usually  metastatic  in  type.  In  the  last 
mentioned  class  syphilis  is  the  most  important  single  factor.  Choroiditis 
may,  however,  be  also  found  in  other  infections,  such  as  tuberculosis, 
rheumatism,  occasionally  in  typhoid  and  relapsing  fevers,  rarely,  in 
leprosy  and  gonorrhoea. 

According  to  the  nature  of  the  exudate  produced,  we  can  recognize 
purulent  and  non-purulent  forms. 

Suppurative  Choroiditis. — Suppurative  choroiditis  is  invariably  due  to 
infection  with  pyogenic  microorganisms.  This  may  be  brought  about 
by  penetrating  wounds  of  the  eye,  ulceration  of  the  cornea  or  sclera,  by 
embolism,  or  by  extension  from  the  meninges.  The  trouble  begins 
acutely  with  chemosis  of  the  conjunctiva  of  the  bulb,  moderate  exudation 
into  the  pupillary  area,  and  hypopyon.  The  exudation  into  the  vitreous 
causes  the  appearance  of  a  yellowish-gray  reflex  on  optical  examination. 
In  the  case  of  some  of  the  milder  forms  of  infection,  such  as  that  occurring 
in  cerebrospinal  meningitis,  the  disease  may  behave  much  as  an  ordinary 
cyclitis,  but  in  many  instances  the  process  extends  to  the  whole  uveal 
tract,  and  finally  results  in  panophthalmitis. 

In  this  condition  the  inflammation  is  intense.  The  conjunctiva  and 
the  eyelids  are  usually  enormously  swollen,  and  the  loose  tissue  of  the 
orbit  is  infiltrated,  so  that  the  eyeball  is  pushed  forward.  The  intraocular 
tension  is  usually  much  increased,  leading  to  diminution  in  depth  of 


634  THE  CHOROID 

the  anterior  chamber,  but  occasionally  it  is  somewhat  diminished. 
The  cornea  eventually  is  infiltrated  and  may  even  slough,  allowing  the 
exudation  to  appear  externally  in  the  form  of  discharge.  After  some 
days  the  severity  of  the  process  diminishes,  and  in  three  or  four  weeks 
the  acute  symptoms  come  to  an  end,  with  gradual  shrinkage  of  the  globe 
(phthisis  bulbi). 

Metastatic  choroiditis  is  a  manifestation  of  a  generalized  septicemia. 
The  choroid  of  one  or  both  eyes  may  be  involved  together  with  other 
parts  of  the  body,  or  may  be  the  sole  area  of  metastatic  deposit. 
Embolic  or  metastatic  choroiditis  is  due,  of  course,  to  the  dissemination 
of  pyogenic  organisms  throughout  the  system  and  their  deposit  in  the 
capillaries  of  the  choroidal  membrane.  The  primary  source  of  the 
infection  varies.  It  may  be,  for  example,  an  infected  wound,  the 
puerperal  uterus,  acute  endocarditis,  smallpox,  pneumonia,  or  erysipelas. 

Serofibrinous  Choroiditis. — Several  forms  of  non-suppurative  choroiditis 
are  described,  most  of  which  are  somewhat  sluggish  in  their  course. 
One  of  the  most  important  is  serofibrinous  choroiditis.  This  affection 
begins  somewhat  suddenly  and  runs  its  course  in  from  six  weeks  to  six 
months.  The  choroid  is  injected  and  slightly  oedematous,  and  the  peri- 
corneal  vessels  are  occasionally  engorged.  There  soon  appears  an 
exudation  of  serofibrinous  material  into  the  vitreous  humor,  obscuring 
the  ophthalmoscopic  picture  of  the  fundus,  which  may  eventually  in- 
volve the  anterior  chamber  and  the  posterior  surface  of  the  cornea. 
The  condition  may  finally  clear  up  with  little  or  no  impairment  of 
vision,  but  not  infrequently  the  choroid  shows  small  patches  of  atrophy. 
Adhesion  of  the  iris  to  the  anterior  surface  of  the  lens-capsule  may  occur. 

Chronic  Choroiditis. — Chronic  choroiditis  may  be  disseminated  or 
diffuse. 

Choroiditis  disseminata  begins  with  the  formation  of  rounded  patches 
of  exudation,  rather  poorly  defined  at  the  margins,  in  certain  parts  of 
the  fundus,  usually  near  the  periphery.  The  patches  multiply  in 
number,  and  some  of  them  may,  in  time,  coalesce,  involving  a  large 
part  of  the  surface  of  the  fundus.  The  retina  overlying  the  spots  in 
question  is  not  elevated,  but  occasionally  appears  to  be  somewhat  hazy, 
indicating  some  infiltration  of  its  substance  with  inflammatory  products 
(chorioretinitis  disseminata).  Usually  the  cornea  and  the  humors  are 
unaffected  and  remain  clear,  but  occasionally,  the  condition  may  be 
complicated  with  parenchymatous  keratitis.  Gradually  the  exudation 
disappears,  the  patches  become  paler,  and  at  the  margins  become 
irregularly  pigmented,  apparently  owing  to  an  increased  deposit  of  pig- 
ment at  certain  points.  Spots  of  pigment  may  often  also  be  observed 
in  the  patches  themselves.  Occasionally  pigmentation  does  not  occur. 
Eventually  the  exudate  disappears  entirely  or  is  partially  organized, 
while  the  affected  areas  go  on  to  complete  atrophy  with  obliteration  of 
the  vessels  and  the  formation  of  cicatricial  tissue.  The  atrophic  patches 
may,  in  time,  increase  in  size,  even  when  the  inflammatory  process  appears 
to  have  come  to  an  end,  owing,  apparently,  to  impairment  of  nutrition. 
The  whole  process  may  run  its  course  in  a  few  months  or  may  be  pro- 


CHRONIC  CPIOROIDITIS 


635 


longed  for  years.  Relapses  are  not  infrequent.  A  curious  feature, 
occasionally  met  with,  is  the  formation  of  plates  of  bone  as  a  result  of 
the  long-standing  irritation. 

Destruction  of  the  choriocapillaris  at  the  points  of  exudation  prob- 
ably always  takes  place.  This,  by  interfering  with  the  blood  supply 
of  the  retina,  leads  to  atrophy  of  that  membrane,  and,  if  extensive,  may, 
in  turn,  lead  to  partial  atrophy  of  the  optic  nerve.  Degenerative  changes, 
due  to  the  lack  of  nutrition,  may  also  take  place  in  the  vitreous  and 
the  lens. 

FIG.  175 


Formation  of  bone  in  the  choroid,  the  result  of  chronic  inflammation.     Zeiss  obj.  DD, 
without  ocular.     (From  the  collection  of  the  Royal  Victoria  Hospital.) 

Forster  has  described,  under  the  term  choroiditis  alveolaris,  an  affec- 
tion, usually  found  in  children,  which  appears  to  be  a  form  of  choroiditis 
disseminata.  This  begins  at  or  near  the  posterior  pole  of  the  eye  with 
the  formation  of  areas  of  pigmentation.  These  gradually  become 
lighter  in  the  centre  and  the  plaques  thin,  until  we  get  atrophic  areas 
bounded  by  fairly  dense  rings  of  pigment.  Such  patches  may  coalesce. 
There  may  be  only  two  or  three  patches  of  atrophy  and  pigmentation  in 
this  affection,  or  a  large  part  of  the  fundus  of  the  eye  may  be  involved. 
The  process  may  begin  and  remain  more  marked  at  the  periphery  of 
the  fundus,  or,  again,  may  involve  chiefly  the  central  portion  (choroiditis 
posterior). 

Choroiditis  diffusa  begins  gradually  and  progresses  in  a  somewhat 
sluggish  way.  It  begins  with  the  formation  in  the  choroid  of  large 
plaques  of  exudation,  of  a  pale  yellowish-pink  or  orange  color.  These 
are  not  pigmented.  The  overlying  retina  is  slightly  cedematous.  The 
patches  coalesce,  forming  large,  irregular,  map-like  areas.  The  chorio- 


636  THE  CHOROID 

capillaris  and  the  pigment  layer  of  the  retina  undergo  atrophy  and 
the  vessels  are  thickened  and  become  obliterated.  The  disk  and  optic 
nerve  may  occasionally  show  some  evidences  of  atrophy.  The  deeper 
layers  of  the  choroid  practically  always  escape. 

Syphilis. — Syphilis  of  the  choroid  may  take  the  form  of  a  serofibrinous 
choroiditis,  or,  again,  a  diffuse  or  disseminated  choroiditis.  Inasmuch 
as  the  retina  is  almost  always  involved,  it  would  be  more  strictly  correct 
to  speak  of  these  conditions  as  syphilitic  chorioretinitis.  In  one  form 
of  syphilitic  inflammation  the  vitreous  chiefly  appears  to  be  involved 
and  becomes  cloudy,  especially  in  the  axial  portion.  In  another  variety 
the  chorioretinitis  is  most  marked  in  the  neighborhood  of  the  posterior 
pole.  Atrophy  and  connective-tissue  formation  are  marked  features  of 
syphilis. 

Tuberculosis. — The  usual  form  of  choroidal  tuberculosis  is  the  miliary 
eruption,  although  massive  tuberculosis  is  not  unknown. 

Miliary  tuberculosis  of  the  choroid  is  but  one  manifestation  and  locali- 
zation of  a  general  miliary  infection.  The  tubercles,  which  are  similar 
in  all  respects  to  miliary  tubercles  elsewhere,  may  be  few  or  very 
numerous  and  are  situated  under  the  choriocapillaris.  The  retina  is  not 
affected,  save  that  it  may  be  elevated  somewhat  where  it  overlies  the 
milia.  The  vitreous  is  also  free.  The  ciliary  body  and  the  iris  are 
but  rarely  attacked  by  tuberculosis.  The  discovery  of  tubercles  in  the 
fundus  of  the  eye  by  ophthalmoscopic  examination  is  sometimes  a  valu- 
able aid  to  the  diagnosis  of  systemic  tuberculosis. 

In  the  massive  form  of  tuberculosis,  the  choroid  is  the  seat  of  larger 
nodules  or  tumor-like  masses,  which  caseate  in  the  centre.  The  process 
may  lead  to  perforation  of  the  sclera  and  the  extension  of  the  tuberculous 
process  to  structures  outside  the  globe. 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  may  be  the  result  of  choroiditis  or  hemorrhages 
into  the  membrane,  or,  again,  of  imperfect  blood  supply,  and  occurs 
in  small,  scattered  areas  or  in  larger  irregular  patches.  It  is  evidenced 
by  pallor  of  the  affected  part  of  the  choroid,  with  an  increase  in  the 
amount  of  pigment.  Atrophy  also  is  met  with  in  myopia. 

There  is,  also,  an  affection  called  "colloid  disease"  of  the  choroid  which 
may  be  discussed  under  this  heading.  Very  smal  nodules,  at  first  soft, 
later  becoming  hard  like  glass,  are  formed  in  the  thin  lamina  elastica. 
The  exact  nature  of  these  is  not  fully  known.  They  are  found  in  cases  of 
partial  atrophy  after  choroiditis  and  in  eyes  removed  for  old  inflammatory 
disturbance. 


TRAUMATISM  AND  ALLIED  CONDITIONS. 

Detachment. — Detachment  of  the  choroid  is  rare,  but  may  be  met 
with  as  a  result  of  hemorrhage  or  exudation  of  inflammatory  products 


ANEMIA  OF  THE  RETINA  637 

between  the  choroid  and  sclerotic.  It  may  also  be  caused  by  a  tumor 
and  may  accompany  degeneration  of  the  vitreous  humor  in  the  course  of 
irido-cyclo-choroiditis. 

Ruptures. — Ruptures  of  the  choroid,  either  single  or  multiple,  are 
occasionally  met  with  as  a  result  of  blows  upon  the  eye.  They  are  accom- 
panied by  more  or  less  hemorrhage  and  eventually  exudation,  which 
may  find  their  way  into  the  retina  and  the  vitreous.  When  the  extra- 
vasated  materials  are  absorbed  an  atrophic  patch  is  left.  The  rupture 
may  be  of  almost  any  shape,  but  is  usually  arranged  with  the  concavity 
toward  the  disk. 

Wounds. — Wounds  of  the  choroid  may  be  of  all  kinds  and  usually 
involve  other  structures.  If  exudation  take  place  into  the  vitreous, 
fibrous  adhesions  may  be  formed,  sometimes  leading  to  detachment  of 
the  retina.  Infected  wounds  may  bring  about  panophthalmitis. 

The  Retina. 

The  retina  is  the  highly  specialized  terminal  of  the  optic  nerve. 
Owing  to  its  preeminent  importance  in  the  visual  apparatus,  disorders 
which  would  elsewhere  be  of  no  consequence  are  here  of  the  greatest 
practical  moment.  The  retina  is  rarely  diseased  alone.  Owing  to  its 
close  proximity  to  the  choroidal  membrane  and  the  nature  of  its  blood 
supply,  inflammation  of  the  latter  membrane  and  disorders  of  its  vascular 
apparatus  are  particularly  liable  to  involve  the  retina  secondarily. 

Again,  retinal  disease  is  very  frequently  an  expression  of  some  general 
systemic  condition,  and  may  be  of  great  diagnostic  value.  Among  such 
conditions  may  be  mentioned  general  arteriosclerosis,  pernicious  anemia, 
leukemia,  Bright's  disease,  diabetes,  syphilis,  and  septicemia. 

Finally,  disorders  of  the  optic  nerve,  particularly  those  which,  like 
congestion,  cedema,  or  inflammation,  tend  to  hamper  the  blood  supply  of 
the  part,  often  lead  to  serious  disturbance  of  the  retina. 


CONGENITAL  ANOMALIES. 

The  retina  may  be  defective  in  the  condition  known  as  coloboma 
oculi  (q.  v.). 

CIRCULATORY  DISTURBANCES. 

Anemia. — Anemia  of  the  retina  is  of  great  practical  importance. 
It  may  be  due  to  extrinsic  causes,  such  as  general  systemic  anemia. or 
loss  of  blood,  or,  again,  to  some  local  disturbance  in  the  vessels,  leading 
to  a  deficient  supply  of  blood.  General  anemia  must  be  of  high  grade 
before  it  will  produce  noticeable  changes  in  the  retina.  The  papilla 
is  pale,  the  arteries  are  narrower  than  normal  and  imperfectly  filled, 
while  the  veins  are  also  somewhat  diminished  in  size,  although  occasion- 
ally they  may  be  overfilled. 


638  THE  RETINA 

The  anemia  of  the  retina  resulting  from  extensive  hemorrhage  may 
lead  to  atrophy  and  fatty  degeneration  of  the  membrane.  Blindness 
may  result  and  be  permanent. 

Total  anemia  of  the  retina  is  due  to  obstruction  of  the  central  artery, 
whether  by  embolism,  thrombosis,  spasm  of  the  muscular  coat,  hemor- 
rhage into  the  optic  sheath,  injury  to  the  artery  within  the  nerve,  or 
pressure  upon  it  by  a  new-growth. 

Embolism. — Embolism  of  the  central  artery  of  the  retina  is,  according 
to  recent  investigations,  considerably  more  uncommon  than  has  usually 
been  thought.  The  primary  cause  is  to  be  looked  for  in  endocarditis, 
aortic  aneurism,  or  arteriosclerosis.  The  embolus  usually  obstructs 
the  whole  vessel  before  its  bifurcation,  although  occasionally  one  of  the 
terminal  branches  is  alone  affected.  Instant  blindness  is  the  result. 

On  ophthalmoscopic  examination  a  short  time  after  the  embolism  has 
occurred,  the  retinal  arteries  are  almost  completely  empty,  the  smaller 
branches  being  nearly  invisible,  while  the  larger  ones  present  only  a 
fine  central  thread  of  blood.  The  veins  of  the  papilla  and  its  neighbor- 
hood are  also,  though  to  a  less  extent,  deficient  in  blood.  The  optic 
disk  is  pale,  with  sharp  edges.  In  course  of  time  a  marked  whitish 
turbidity  of  the  retina  becomes  manifest,  situated  round  about  the 
optic  nerve  and  fovea  centralis.  The  contour  of  the  papilla  is  thereby 
obscured  and  the  whole  of  the  macular  region  and  its  neighborhood 
becomes  cloudy.  In  the  centre  of  this  area  can  be  seen  a  reddish  spot 
corresponding  with,  though  somewhat  larger  than,  the  centre  of  the 
fovea.  The  affected  portion  of  the  retina  gradually  undergoes  atrophy, 
so  that  the  choroid  shows  through.  Small  hemorrhages  may  some- 
times also  be  seen  in  the  neighborhood  of  the  papilla.  Finally,  the 
cloudiness  disappears  and  the  papilla  and  the  retina  atrophy  and 
become  functionless. 

Thrombosis. — Thrombosis  of  the  arteria  centralis  retina?  gives  rise  to 
a  train  of  events  similar  to  those  in  the  case  of  embolism.  The  condi- 
tion is  rare  and  is  probably  in  all  cases  to  be  attributed  to  arterial  disease. 

Spasm. — Spasm  of  the  retinal  artery  and  its  branches  has  been 
observed  in  cases  of  migraine  (Wagenmann),  with  the  production  of 
temporary  blindness. 

Hyperemia. — Hyperemia  of  the  retina  may  be  arterial  or  venous. 

Arterial  Hyperemia. — Arterial  or  active  hyperemia  results  from  inflam- 
mation of  the  retina,  eyestrain,  irritation  of  the  eye,  from  keratitis,  iritis, 
choroiditis,  and  is  met  with  in  cases  of  meningitis,  Graves'  disease,  and 
neurasthenia.  The  arteries  in  this  condition  are  overdistended,  appar- 
ently lengthened,  and  varicose. 

Venous  Hyperemia. — Venous  hyperemia,  or  passive  congestion,  is  due 
to  some  interference  with  the  return  flow  of  blood  from  the  eye.  As 
a  rule,  the  obstruction  is  referable  to  some  diseased  condition  of  the  optic 
papilla,  such  as  optic  neuritis.  Here,  the  swelling  of  the  disk  leads  to 
compression  of  the  central  vein,  and  the  same  thing  may  be  produced 
by  glaucoma  and  by  disease  processes  in  the  orbit,  as,  for  example, 
tenonitis  and  orbital  cellulitis.  Occasionally,  meningitis,  intracranial 


HEMORRHAGE  639 

tumors,  or  thrombosis  of  the  cavernous  sinus  may  be  the  causative 
factors.  Congenital  heart  disease,  when  associated  with  general  cyan- 
osis, are  associated  with  noteworthy  congestion  of  the  retina.  Throm- 
bosis of  the  central  vein  is  one  of  the  rarest  causes  of  venous  hyperemia. 
It  is  generally  due  to  angiosclerosis,  but  occasionally  has  been  observed 
in  orbital  cellulitis. 

Venous  hyperemia  is  characterized  by  distention  and  tortuosity  of  the 
veins,  and  the  disks  appear  also  to  be  hyperemic.  In  many  cases  the 
arteries  look  somewhat  attenuated.  In  the  severer  forms,  hemorrhages 
into  the  fundus  may  occur.  Vision  is  not  usually  entirely  impaired, 
and  temporary  improvement  may  take  place.  Relapses  are,  however, 
common,  and  the  sight  may  ultimately  be  lost. 

Hemorrhage. — Retinal  hemorrhages  are  due  to  a  great  variety  of 
causes.  One  of  the  most  important  is  trauma.  They  are  often  also 
met  with  in  certain  constitutional  diseases,  especially  those  that  damage 
the  integrity  of  the  vessel  walls,  and  in  some  local  affections  of  the  eye 
itself. 

A  potent  factor  is  passive  congestion.  We  therefore  are  liable  to  get 
retinal  hemorrhages  in  cases  of  suffocation,  thrombosis  of  the  central 
vein  of  the  retina,  in  pressure  upon  the  vein,  such  as  may  be  produced 
by  optic  neuroretinitis  and  neuritis  or  glaucoma.  Occasionally,  hemor- 
rhages are  found  in  embolism  of  the  central  artery  of  the  retina  or 
infarction. 

Vascular  changes,  sclerosis  or  endarteritis,  predispose  strongly  to  hemor- 
rhage, and  are  met  with  in  conditions  such  as  general  arteriosclerosis, 
Bright's  disease,  diabetes,  gout,  pernicious  anemia,  leukemia,  scurvy, 
and  in  liver  affections  associated  with  jaundice.  Among  other  general 
causes  may  be  mentioned  septicemia,  malaria,  relapsing  fever,  extensive 
burns  of  the  skin,  and  poisoning  with  phosphorus  and  lead.  Among 
rarer  causes  are  mentioned  disorders  of  menstruation  and  vicarious 
menstruation.  Fatty,  hyaline,  and  amyloid  changes  in  the  vessel  walls 
may  on  occasion  lead  to  extravasation  of  blood. 

Retinal  hemorrhages  vary  considerably  in  number,  size,  shape,  and 
position.  The  patches  are  pale  red,  dark  red,  or  black,  according  to 
the  age,  and  frequently  assume  a  radiate  or  '*  flame-shaped"  appearance. 
This  is  owing  to  the  fact  that  the  extravasation  often  takes  place  into 
the  nerve-fiber  layer,  where  it  follows  the  course  of  the  fibers.  The 
larger  effusions  of  blood  may  force  their  way  into  the  vitreous,  which 
thereby  becomes  opaque,  or  between  the  choroid  and  the  retina.  Occa- 
sionally, the  blood  collects  beneath  the  hyaloid  membrane  (subhyaloid 
hemorrhage). 

The  blood  is  often  absorbed  rapidly  but  leaves  whitish  patches  in 
the  retina,  which  are  due  to  fatty  degeneration  and  atrophy  of  the 
membrane,  resulting  from  the  interference  with  the  nutrition.  Not  in- 
frequently, such  spots  become  pigmented,  usually  at  the  periphery,  and 
may  clear  toward  the  centre.  They  may  often,  however,  contain  scattered 
blotches  of  pigment.  In  cases  of  hemorrhage  into  the  vitreous  the  clots 
may  remain  attached  to  the  retina  and  become  organized,  forming 


640  THE  RETINA 

curious  tags.  This  is  believed  to  be  the  cause  of  the  so-called  retinitis 
proliferans. 

The  disturbances  of  vision  which  result  depend,  of  course,  on  the 
extent  and  localization  of  the  hemorrhages.  Extravasations  in  the  macula 
will  lead  to  serious  interference  with  the  sight.  Even  moderate  effusions 
of  blood  into  the  vitreous  will  produce  cloudiness  of  vision.  In  other 
cases  we  may  have  metamorphopsia,  less  often,  photopsia. 

Hemorrhage  into  the  sheath  of  the  optic  nerve  may  cause  pressure 
upon  the  central  artery  and  anemia.  It  has  been  known  to  follow 
trauma  to  the  eye  or  hemorrhage  at  the  base  of  the  brain,  the  blood  in 
the  latter  case  forcing  its  way  along  the  sheath  of  the  nerve. 

Aneurisms. — Aneurisms  of  the  retinal  arteries  are  very  rare.  They 
are  usually  miliary  in  size  and  multiple,  though  larger  single  ones  may 
occur.  Traumatic  arteriovenous  aneurism  has  been  described. 

Phlebectasia. — Phlebectasia  is  a  rare  condition  in  which  the  retinal 
veins  present  a  markedly  beaded  appearance,  due  to  alternate  constric- 
tions and  dilatations.  It  has  been  noted  in  connection  with  suppressed 
menstruation. 

INFLAMMATIONS. 

Retinitis. — Under  the  term  retinitis,  which,  strictly  speaking,  should 
be  employed  to  designate  inflammatory  conditions  of  the  retina  only,  are 
usually  classed  a  number  of  affections,  chiefly  of  a  degenerative  nature, 
that  are  only  more  or  less  doubtfully  related  to  inflammation.  Such  are 
certain  forms  of  fatty  degeneration,  atrophy,  cedema,  hemorrhage,  and 
pigmentation. 

Retinitis  may  exist  per  se,  but  is  usually  dependent  on  or  associated  with 
disease  of  the  neighboring  structures.  When  due  to  infection  it  may  be 
brought  about  directly  by  trauma,  or  may  be  secondary  to  some  disease 
process  in  a  distant  part.  According  to  the  type,  we  can  recognize 
suppurative  and  non-suppurative  forms. 

Suppurative  Retinitis. — Suppurative  retinitis  is,  in  a  large  proportion 
of  cases,  due  to  penetrating  wounds  of  the  bulb,  whereby  septic  micro- 
organisms are  imported  into  the  eye.  It  may,  for  example,  follow  an 
operation  for  cataract  extraction.  Metallic  substances,  particularly 
copper,  which  have  entered  the  eye  and  are  disintegrating  there,  some- 
times give  rise  to  a  mild  form  of  suppurative  inflammation.  In  other 
cases  the  infection  is  metastatic,  the  primary  condition  being  puerperal 
septicemia  or  some  acute  infectious  fever.  In  this  variety  the  condi- 
tion is  apt  to  be  a  chorioretinitis.  Where  the  inflammatory  process  has 
affected  mainly  the  vitreous  humor,  the  condition  may  give  rise,  in 
children,  to  the  appearance  known  clinically  as  pseudoglioma.  In  the 
milder  forms  of  septic  retinitis,  we  find  in  the  retina  hemorrhages  and 
white  spots,  not  unlike  those  found  in  albuminuric  retinitis  without 
any  marked  evidences  of  inflammation,  and  apparently  without  the 
presence  of  microorganisms,  while  the  severer  cases  go  on  to  suppuration 
and  exudation.  In  the  latter  class  of  cases  staphylococci  or  strepto- 
cocci may  be  found. 


ALBUMINURIC  RETINITIS  641 

In  the  earlier  stages  the  retina  appears  to  be  swollen  and  cloudy 
with  scattered  hemorrhages.  Later,  the  vitreous  humor  becomes  turbid 
from  exudation  to  such  an  extent  as  to  interfere  with  further  study  of 
the  case  by  ophthalmoscopic  methods.  Microscopically,  the  retina  is 
swollen  and  oedematous,  the  nerve-fiber  layer  and  later  the  ganglionic 
layer  are  infiltrated  with  leukocytes,  and  in  the  supporting  stroma  is 
a  granular  and  fibrinous  exudate,  with,  in  severe  cases,  hemorrhagic 
extravasations.  In  course  of  time  the  radial  fibers  hypertrophy  and 
elongate  in  the  direction  of  the  cornea.  Ultimately  the  rods  and  cones 
atrophy  and  disappear.  The  process  ends  finally  in  panophthalmitis 
and  phthisis  bulbi. 

Retinitis  Simplex. — The  mildest  form  of  non-suppurative  retinitis  is 
that  known  as  retinitis  simplex  (serous  retinitis;  oedema  retince).  The 
causes  are  not  entirely  clear.  Some  cases  are  attributed  to  the  effect  of 
eye-strain,  others,  again,  follow  blows  upon  the  eye  (commotio  retinae). 
The  condition  is  also  said  to  be  one  of  the  first  manifestations  of  sym- 
pathetic ophthalmia.  The  retina  is  found  to  be  congested  and  hazy, 
apparently  from  oedema,  which  may  be  either  patchy  or  diffuse. 

Albuminuric  Retinitis. — Of  much  more  practical  importance,  from 
the  standpoint  of  the  diagnostician,  is  albuminuric  retinitis.  In  this 
form  of  retinitis  the  optic  nerve  papilla  is  usually  involved  as  well 
(neuroretinitis).  The  changes,  when  well  marked,  are  almost  pathog- 
nomonic  of  nephritis,  and  it  not  infrequently  happens  that,  in  cases 
where  the  general  symptoms  are  somewhat  in  abeyance,  the  diagnosis  of 
Bright's  disease  is  first  made  by  the  ophthalmologist.  On  examination, 
the  optic  disk  is  found  to  be  reddened,  swollen,  and  somewhat  blurred 
at  the  margin,  while  in  the  neighborhood  are  numerous  rounded  or 
radially-disposed  streaks  of  hemorrhage,  together  with  larger  or  smaller, 
irregular,  white  patches,  which  may  coalesce  and  form  extensive  areas 
around  the  papilla.  The  retinal  vessels,  particularly  the  veins,  are  over- 
distended  and  tortuous.  In  the  region  of  the  macula  can  often  be  seen 
white  streaks  arranged  in  rows,  having  a  characteristic,  star-like  form. 
Both  eyes  are  usually  involved,  but  one  may  be  more  affected  than  the 
other. 

Microscopically,  we  find  abundant  lymph-corpuscles,  especially  along 
the  vessels,  with  fibrinous  exudate  into  the  tissue  spaces,  together  with 
hyperplasia  of  the  supporting  stroma.  There  is  a  widespread  arteritis 
and  capillaritis,  resulting  in  thickening  and  sclerosis  of  the  smaller  vessels. 
The  vascular  changes,  no  doubt,  account  for  the  numerous  small  hemor- 
rhages that  are  found  in  this  form  of  retinitis.  The  white  patches  above 
referred  to  are  produced  by  dense  accumulations  of  fatty  granular 
cells,  which  are  situated  within  and  between  the  granular  layers,  and  of 
hyaline  and  colloidal  masses  supposed  to  be  derived  from  degenerating 
blood-extravasations  and  nerve-substance.  The  whitish  streaks  in  the 
macular  region  are  due  to  fatty  degeneration  of  the  inner  ends  of  the 
radiating  nerve-fibers.  The  optic  papilla  shows  infiltration  with  lymph- 
cells,  degeneration  of  its  fibers,  and  hypertrophy  of  the  interstitial  sub- 
stance. 
41 


642  THE  RETINA 

In  albuminuric  retinitis  the  power  of  vision  is  rarely  lost  completely, 
and  this  peculiarity  is  one  of  the  most  important  means  of  distinguishing 
between  this  condition  and  the  optic  neuritis  resulting  from  brain  tumor 
in  which  the  sight  is  always  almost  lost.  The  fact  that  the  fovea  cen- 
tralis  is  rarely  affected  in  albuminuric  retinitis  accounts  for  the  fact  that 
central  vision  is  almost  constantly  preserved. 

Retinal  changes  somewhat  similar  to  those  occurring  in  Bright's 
disease  are  also  met  with  in  long-standing  cases  of  diabetes,  but  are 
much  rarer.  In  one  form  of  the  trouble,  multiple  small  hemorrhages 
are  to  be  seen  in  the  retina  and  nothing  more,  the  hemorrhagic  diabetic 
retinitis  of  Hirschberg.  This  condition  is  probably  not  inflammatory  in 
its  nature.  More  characteristic  is  central  punctate  retinitis.  Here,  the 
retina  presents  numerous  small,  bright,  shining  spots,  chiefly  in  the 
neighborhood  of  the  optic  disk  and  the  macula,  but  not  having  the  stellate 
arrangement  of  the  spots  in  albuminuric  retinitis.  With  these  are  to 
be  seen  multiple  scattered  hemorrhages.  The  retinal  vessels  seem  to  be 
normal  and  there  is  no  oedema  either  of  the  papilla  or  of  the  retina. 
Not  infrequently  hemorrhage  may  take  place  into  the  vitreous,  causing 
turbidity  of  that  medium  and  considerable  impairment  of  vision.  Glauc- 
oma, secondary  to  the  hemorrhage,  may  also  occur.  Vision  is  apt  to  be 
bad  in  diabetic  retinitis,  especially  in  the  central  portion  of  the  field, 
and  peripheral  vision  may  also  be  impaired. 

Chronic  Diffuse  Retinitis.— A  chronic  diffuse  retinitis  is  described, 
resulting  from  inflammation  of  the  uveal  tract.  It  is  marked  chiefly  by 
cellular  infiltration,  and,  later,  by  the  formation  of  new  connective  tissue 
in  the  deeper  layers  of  the  membrane.  The  radial  fibers,  together  with 
the  supporting  stroma  and  the  adventitia  of  the  vessels,  also  show 
thickening.  The  increase  in  length  of  the  radial  fibers  may  attain  such 
proportions  that  a  layer  of  reticulated  connective  tissue  is  formed  upon 
the  inner  surface  of  the  retina.  The  nerve-fibers  and  ganglia  ultimately 
atrophy  and  disappear,  while  the  rods  and  cones  are  similarly  affected, 
though  to  a  less  degree.  In  some  few  cases  the  rods  and  cones  are,  on 
the  contrary,  hypertrophied,  becoming  both  longer  and  thicker  than 
normal.  This  is  particularly  apt  to  be  the  case  when  detachment  of 
the  retina  has  taken  place. 

Disseminated  Retinitis. — Somewhat  akin  to  the  last-mentioned  form 
of  retinitis  is  disseminated  retinitis,  which  is  analogous  to  disseminated 
chorioretinitis,  above  described.  In  many  of  the  cases,  in  fact,  it  is  not 
always  easy  or  even  possible  to  say  whether  the  process  has  originated 
in  the  choroid  and  has  subsequently  spread  to  the  retina,  or  whether  it  is 
primary  in  the  latter  membrane.  Thin  patches  of  exudation  are  found 
between  the  choroid  and  the  retina,  together  with  circumscribed  destruc- 
tion of  the  pigment-epithelium  and  of  the  layer  of  rods  and  cones.  In 
some  parts  the  retinal  pigment  tends  to  accumulate,  so  that  it  may 
be  readily  recognized  on  ophthalmoscopic  examination.  Ultimately  the 
connective  tissue  of  the  outer  layers  of  the  retina  and  the  supporting 
fibers  proliferate  and  extend  in  the  direction  of  the  choroid.  In  this  new 
material  can  be  seen  more  or  less  altered  fragments  of  the  rods  and  cones, 


RETINITIS  PIGMENTOSA  643 

with  masses  of  pigment  and  larger  or  smaller  gland-like  excrescences, 
derived  from  the  lamina  vitrea  of  the  choroid.  The  connective-tissue 
formation  and  the  pigmentation  may  eventually  extend  to  the  innermost 
layers  of  the  retina,  where  the  pigment  is  seen  to  be  deposited  along  the 
course  of  the  vessels. 

Retinitis  Pigmentosa. — In  some  respects  comparable  to  the  last-men- 
tioned form  is  the  so-called  retinitis  pigmentosa,  an  affection  in  which 
pigmentation  is  an  early  and  characteristic  feature.  Inasmuch  as  the 
inflammatory  manifestations  are  of  the  slightest  description,  and,  in 
fact,  are  generally  lacking,  it  is  questionable  whether  the  process  is  not 
more  properly  to  be  classed  among  the  degenerations  than  among  the 
inflammations.  The  ophthalmoscopic  picture  is  characteristic.  There 
is  slight  atrophy  of  the  optic  papilla,  as  shown  by  its  yellowish-white 
appearance  and  sharp  contour;  the  vessels,  particularly  the  arteries,  are 
shrunken,  and  there  is  a  notable  deposit  of  pigment  in  a  zone  inter- 
mediate between  the  posterior  pole  and  the  equator  of  the  eye.  The 
patches  of  pigment,  which  are  more  or  less  numerous,  are  small,  irregularly 
indented,  and  arranged  in  large  part  along  the  course  of  the  vessels. 
Where  the  pigmentation  is  more  extreme  the  patches  may  coalesce  to 
form  large,  deep  black  clumps,  often  containing  rounded  spots  devoid 
of  coloring.  Hemorrhages  do  not  occur,  nor  are  the  clear  spots  resulting 
from  infiltration  of  the  retina  or  atrophy  of  the  choroid  to  be  seen. 

Histologically,  one  finds  hyaline  thickening  of  the  vessels,  with  obliter- 
ation of  their  smaller  branches,  atrophy  of  the  pigment  epithelium, 
with  the  new  formation  of  deeply-pigmented  cells  in  the  retina,  where 
they  become  located  in  the  vessel  sheaths,  and  marked  hypertrophy  of 
the  supporting  stroma  of  the  retina. 

The  disease  affects  both  eyes,  and  can  be  inherited. 

Among  the  rarer  forms  of  retinitis  may  be  mentioned  retinitis  circinata 
(Fuchs),  retinitis  striata  (Nagel),  retinitis  punctata  albescens  (Mooren), 
and  retinitis  Solaris. 

Retinitis  circinata  is  found  only  in  elderly  people  and  affects  one 
or  both  eyes.  On  examination,  a  number  of  small,  white  spots  can  be 
seen  arranged  about  the  macula  in  a  more  or  less  perfect  circle.  The 
macula  shows  a  grayish  opacity.  The  white  spots  are  deeper  than  the 
retinal  vessels  and  may  be  slightly  pigmented.  There  is  diminution 
in  central  vision,  contraction  of  the  visual  field,  and  a  small  central 
scotoma.  In  this  affection  one  or  both  eyes  may  be  involved. 

Retinitis  striata  derives  its  name  from  the  fact  that  there  are  a  number 
of  grayish  stripes  to  be  seen  in  the  retina  in  front  of  the  pigment  layer 
but  behind  the  vessels.  These  stripes  may  be  three  or  four  times  as 
wide  as  the  veins  and  may  radiate  from  the  disk  like  the  spokes  of  a 
wheel,  or,  again,  may  have  no  particular  arrangement.  The  retina 
may  also  show  pigmentation.  Vision  is  slightly  reduced,  but  blindness 
does  not  usually  result. 

In  retinitis  punctata  albescens  the  retina  is  studded  with  small,  white 
dots,  which  are  most  numerous  around  the  disk  and  in  the  macula.  The 
fovea,  however,  is  usually  unaffected.  Central  vision  is  reduced,  and 
there  are  sometimes  nyctalopia  and  reduction  of  the  peripheral  field. 


mi      .1     IIII 


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separation.  The  condition  m.i\  i  ome  on  without  obvious  cause,  When 
(he  separation  is  n<en(,  (he  retina  pro|<-«  I  .  forward  into  the  posterior 
chamber  as  .1  hrnnilon  ..  translucent,  ^'ravish  «  olored  ineinhranc.  lhro\\n 
in(o  folds,  over  which  (he  vessels  can  he  made  out  laKin^  an  irrc^nl.ir 
course.  The  separation  (ends  to  increase,  and  linallv  hecomes  complete, 
ihe  iciin.i  remaining  attached  at  die  disk  and  ora  serrata  in  (he  form  of 
a  plaited  funnel  Tin-  separated  ivthu  is  oh,  n  o-denuilous  and  m.i\ 


OLIOM  \ 


645 


un<]< •!•••«.   hyperpla  la      Calcification,  or,  rnon    nuvly,  o    in'ration,  r 

,r.      Tli.     |,|r, <,<!•.<  «  I      (iii;illv    iifi'l'  CO  r     :ni'l    ln-«  >,MM     llii'/in 

.      The  IMtrvoiJ     '  I'  MM  i,l     .-ilrophy  ;in«l  i-vi-iilu;illv  I.'  •  '.in-    in;.' •  i;.l«  (I 
p 


PROGRESSIVE  METAMORPHOSH, 

Tumors.  Tin-  only  I.'  HI-MI  ;'i',','.  llr-,  win- I.  hav<  L«  n  found  hi 
original*-  in  flu-  rH.in;i  Jin  MMflfltttttto  and  fibron  imor  ,  y,ln<li 

lic«-n  found  in  •  <  afh  i  c  niovw  I 

Glioma.  <ilioin;i  i,  lli«-  onlv  in;ili;'ii;i  hi  ;»rov/lh  \vlii'  h  i  |>iiruary 
in  flic  n -I  ina.  Sin'-'  it  i  Found  u  .nail  y  in  <  ai  I  v  inlan'  v  a  nd  n«  v<  t  lah  i 

l,s     I/', 


lj<f  twelfth 
.1    {>''':uli 
than  one  of    the 

,'/":       <  ' 


646  ?Hft  OPTIC  NERVE 

The  new-growth  begins  at  the  back  of  the  eye,  pushing  the  retina 
before  it  into  the  anterior  chamber.  Comparatively  early  it  invades  the 
sheath  of  the  optic  nerve.  It  gradually  spreads  to  the  ciliary  process 
and  iris,  and  may  eventually  infiltrate  the  whole  eye.  Iritis  and  ulcera- 
tion  of  the  cornea  occur,  and,  finally,  the  tumor  penetrates  the  globe 
and  appears  externally,  where  it  may  attain  relatively  enormous  pro- 
portions. It  forms  a  soft,  fungating  mass,  which  is  necrosed  in  parts 
and  tends  to  bleed  readily.  In  time  the  growth  may  involve  the  sclera, 
the  eyelids,  the  soft  parts  and  bones  of  the  face,  and  may  eventually 
reach  the  brain  by  way  of  the  sheath  of  the  optic  nerve.  Secondary 
growths  may  be  found  in  the  regional  nodes,  the  parotid  and  submaxillary 
glands,  in  the  liver,  and  other  organs. 

Histologically,  the  growth  does  not  differ  materially  from  the  glioma 
occurring  in  the  brain.  It  consists  of  numerous  closely  packed,  mono- 
nuclear  cells,  embedded  in  a  finely  granular  and  fibrillated  ground 
substance,  which  is  abundantly  provided  with  wide,  thin-walled  blood- 
vessels. The  glioma  cells  are  round  and  contain  a  single  nucleus  almost 
completely  filling  up  the  cell  body,  so  that  the  tumor  to  some  extent 
seems  to  be  made  up  of  granules,  recalling  the  granular  layer  of  the 
retina.  By  proper  methods  the  peculiar  spider-like  cells  of  gliomatous 
tissue  can  be  detected.  In  some  specimens  peculiar  rosettes  formed 
of  rods  and  cones  have  been  found  and  such  tumors  have  been  termed 
neuro-epitheliomata.  The  layer  of  rods  and  cones  is  homologous  with 
the  cells  lining  the  central  neural  canal.  Flexner  would,  therefore, 
term  such  tumors  ependymal  gliomata.  Glioma  usually  starts  from 
the  outer  portion  of  the  retina,  the  granular  layers,  but,  more  rarely, 
may  originate  from  the  nerve-fiber  layer. 


The  Optic  Nerve. 
CONGENITAL  ANOMALIES. 

Coloboma. — Coloboma  of  the  sheath  of  the  optic  nerve  has  already 
been  referred  to  (see  p.  608). 

CIRCULATORY  DISTURBANCES. 

(Edema  and  Congestion. — (Edema  and  congestion  of  the  optic  nerve, 
either  in  the  papilla  or  in  the  papilla  and  behind  the  bulb  as  well, 
are  found  in  the  early  stages  of  inflammation  of  the  nerve,  or  as  a  result 
of  the  pressure  of  tumors  or  inflammatory  exudates  upon  the  nerve 
trunk.  These  conditions  will  be  more  conveniently  treated  under  the 
heading  of  inflammation. 

INFLAMMATIONS. 

Inflammation  of  the  optic  nerve  may  affect  the  retrobulbar  portion  of 
the  trunk  (neuritis)  or  the  distal  extremity  (papillitis).  Inflammation 


PAPILLITIS  OR  CHOKED  DISK  647 

of  the  papilla— papillitis — -may  exist  per  se,  but  not  infrequently  is  due 
to  the  extension  of  an  inflammatory  process  from  the  retina  (neuro- 
retinitis),  from  the  nerve  trunk,  or  from  the  brain,  by  way  of  the  nerve 
trunk  (descending  optic  neuritis). 

Papillitis  or  Choked  Disk  (Stauungspapille). — Papillitis  is  com- 
monly the  result  of  meningitis,  sinus  thrombosis,  intracranial  tumors, 
and,  occasionally,  tumors  and  inflammatory  exudates  within  the  orbit. 
As  to  the  essential  nature  of  papillitis  opinions  vary.  Some  interpret 
it  as  the  result  of  hydrops  of  the  nerve  sheath;  others,  as  a  true  inflamma- 
tion of  the  disk.  It  is  a  fact,  however,  that  any  condition  which  leads  to 
an  increase  in  intracranial  pressure,  such  as,  for  example,  a  new-growth 
or  meningitis,  results  in  forcing  an  increased  amount  of  cerebrospinal 
fluid  into  the  intervaginal  space  of  the  optic  nerve.  This  induces  press- 
ure upon  the  central  artery  and  vein  of  the  retina  which  enter  the  nerve 
a  few  millimeters  behind  the  globe.  The  papilla  thus  becomes  swollen 
and  cedematous. 

The  ophthalmoscopic  examination  in  such  cases  shows  the  margin  of 
the  disk  to  be  blurred  and  the  retinal  zone  immediately  about  it  to  be 
dimmer  than  it  should  be.  The  papilla  itself  is  cloudy,  reddened,  and 
swollen,  particularly  in  the  nasal  portion.  The  veins  of  the  retina  are 
also  somewhat  congested.  In  the  more  intense  cases  of  papillitis  the 
infiltration  of  the  tissues  is  still  more  marked.  The  papilla  is  greatly 
swollen  and  so  cloudy  that  it  is  with  difficulty  recognizable.  The  retinal 
veins  are  also  more  indefinite,  while  numerous  small  extravasations  of 
blood,  arranged  in  radial  fashion,  are  to  be  seen  in  the  grayish-red  nerve 
substance.  In  purely  inflammatory  cases  the  turbidity  of  the  tissues 
is  the  most  striking  and  characteristic  feature;  in  the  hydropic  form  the 
turbidity  is  not  so  great,  but  the  disk  is  enormously  swollen  and  the 
retinal  veins  are  overfilled  and  distorted.  While  this  is  the  rule,  it  must 
be  admitted  however,  that  it  is  not  always  possible  to  determine  from 
the  ophthalmoscopic  examination  alone  the  true  nature  and  cause  of 
the  papillitis,  whether  due  to  pressure  or  to  inflammation.  The  form 
met  with  in  albuminuria  is  of  the  type  of  a  neuroretinitis  (see  p.  641). 
Papillitis,  when  of  mild  grade,  may  pass  away  leaving  the  disk  practically 
normal.  The  more  severe  or  more  prolonged  cases  usually  result  in 
loss  of  vision.  This  is  due  to  atrophy  of  the  nerve-fibers  from  the 
pressure  of  the  inflammatory  exudate  or  of  newly-formed  connective 
tissue. 

Histologically,  the  choked  disk  from  hydrops  presents  marked  oedema, 
which  is  the  sole  cause  of  the  great  swelling,  but  sooner  or  later  inflam- 
matory infiltration  is  superadded  and  a  picture  results  which  cannot  be 
differentiated  from  any  of  the  forms  of  true  papillitis.  The  tissues  are 
infiltrated  with  small  round  cells,  particularly  along  the  course  of  the 
vessels,  the  nerve-fibers  are  swollen  and  nodular,  and  there  are  minute 
extravasations  of  blood  and  a  deposit  of  finely  granular  detritus  between 
the  fibers.  In  the  most  advanced  stages,  there  is  a  considerable  increase 
of  connective  tissue  and  consecutive  atrophy,  more  or  less  complete,  of 
the  nerve-fibers.  In  the  latter  case,  the  papilla  is  sharply  defined,  on 


648  THE  OPTIC  NERVE 

ophthalmoscopic  examination,  of  a  dead  white  color,  perhaps  somewhat 
excavated,  and  the  retinal  vessels  are  shrunken  looking. 

Inflammation  of  the  trunk  of  the  optic  nerve  (retrobulbar  neuritis) 
may  affect  the  peripheral  portion  of  the  nerve  and  its  sheath  (peri- 
neuritis'),  the  axial  portion  (central  or  axial  neuritis),  or  various  areas 
scattered  through  the  nerve  (disseminated  neuritis).  The  parts  involved 
are  the  intervaginal  space  and  the  interstitial  fibrous  stroma  of  the  nerve. 
In  the  form  above  termed  perineuritis,  the  intervaginal  space  is  filled 
with  an  inflammatory  exudate,  consisting  of  serum,  round  cells,  and  often 
fibrin.  Later,  the  endothelial  cells,  covering  the  connecting  strands  that 
traverse  the  space,  proliferate.  Accompanying  this,  the  supporting 
stroma  of  the  nerve  may  be  found  infiltrated  with  similar  inflammatory 
products.  Later,  the  inflammatory  process  may  involve  the  nerve-fibers 
proper,  so  that,  as  the  combined  result  of  pressure  and  disintegration, 
they  atrophy  and  finally  disappear.  The  nerve-substance  may  thus  be 
represented  simply  by  masses  of  fragmenting  myelin,  fatty  granules,  and 
the  so-called  amyloid  bodies.  It  is  possible,  too,  that  the  inflammation 
and  disintegration  of  the  nerve-fibers  may  be  primary — neuritis  medul- 
laris  (Leber). 

Retrobulbar  neuritis  may  result  in  more  or  less  complete  loss  of  vision, 
according  to  the  amount  of  nerve-substance  that  is  destroyed.  Where 
there  is  partial  loss  of  vision,  the  condition  is  known  as  scotoma.  We 
may  have  peripheral,  axial,  or  disseminated  scotomas.  Perhaps  in  the 
majority  of  cases,  the  scotoma  is  situated  in  or  near  the  centre.  In  chronic 
tobacco  amblyopia  there  is  a  central  scotoma  with  also  some  limitation 
of  the  peripheral  field  of  vision. 

Neuritis  of  the  optic  nerve  trunk  is  often  due  to  inflammation  in  the 
orbit,  the  extension  of  a  basal  meningitis,  or  an  intracranial  new-growth. 
Occasionally,  it  can  be  traced  to  a  systemic  infection  or  intoxication. 
In  the  toxic  forms  it  is  perhaps  a  question  whether  the  inflammation  is 
primary  in  the  nerve  trunk  or  whether  the  process  begins  in  the  retina, 
degeneration  here  leading  to  secondary  ascending  atrophy  of  the  fibers. 

Retrobulbar  neuritis  may  disappear  without  serious  damage  being 
done,  but  not  infrequently  atrophy  supervenes  with  more  or  less  loss  of 
visual  power. 

Tuberculosis. — Miliary  tubercles  have  been  found  affecting  the  sheath 
of  the  nerve.  Occasionally,  also,  the  nerve  has  been  destroyed  by  a 
diffuse  infiltration  of  its  substance  with  tuberculous  granulation  tissue. 

Syphilis. — Syphilis  may  assume  the  form  of  a  simple  retrobulbar 
neuritis  or  a  gummatous  infiltration  of  the  nerve.  The  whole  trunk 
and  even  the  chiasm  may  be  involved.  Syphilitic  neuroretinitis  (q.  v.) 
is  also  a  well-recognized  condition. 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  optic  nerve  occurs  and  is  due  to  a  variety 
of  causes.     In  general,  it  may  be  said  that  any  condition  which  interferes 


TUMORS  649 

with  the  nutrition  of  the  nerve-fibers  or  brings  about  destruction  of  the 
neurocytes,  or  nutritive  nerve-centres,  is  competent  to  bring  about 
atrophy.  Pressure  upon  the  nerve-trunk,  as  from  inflammatory  exudates, 
connective-tissue  hyperplasia,  congested  vessels,  or  new-growths,  is  an 
important  cause.  Atrophy  of  the  neurocytes  in  the  retina,  from  inflam- 
mation, vascular  disturbances,  or  other  causes,  leads  secondarily  to 
wasting  of  the  nerve-fibers  proceeding  from  them.  Simple  atrophy  of 
the  optic  nerve  occurs  in  tabes  dorsalis  and  progressive  paralysis  of  the 
insane.  The  cause  is  held  by  some  to  be  a  primary  degeneration  of 
the  retinal  cells.  The  various  toxic  amblyopias,  notably  that  from 
quinine,  are  probably  due  to  a  similar  condition,  resulting  from  the 
induced  ischemia  of  the  retinal  vessels. 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — Tumors  of  the  optic  nerve  and  its  sheath  are  rare.  They 
may  be  primary  or  secondary.  The  secondary  new-growths,  which  are 
of  course  malignant,  usually  originate  in  other  parts  of  the  eyeball  or  in 
the  orbit  and  involve  the  nerve  by  direct  extension.  Thus,  glioma  of 
the  retina  and  sarcoma  of  the  choroid  may  invade  the  disk,  and  carcinoma 
and  sarcoma  of  the  orbital  cavity  may  attack  the  trunk  of  the  nerve. 
Metastatic  carcinoma  has  also  been  reported. 

The  primary  tumors  may,  with  Leber,1  be  classified  into  those  affecting 
the  intra- ocular,  the  intra-orbital,  and  intracranial  portions  of  the  optic 
nerve.  They  may  further  be  divided  into  those  that  spring  from 
the  dural  covering  or  the  parts  immediately  adjacent  to  it — extradural 
new-growths;  and  those  originating  from  structures  within  the  dural 
sheath  and  for  a  time,  at  least,  bounded  externally  by  it — intradural 
new-growths. 

The  vast  majority  of  primary  intradural  tumors  have  been  reported  as 
myxomas,  fibromas,  sarcomas,  and  various  combinations  of  these 
elemental  types,  occasionally  as  gliomas.  Many  of  these,  however, 
were  reported  before  microscopic  technique  was  as  perfect  as  it  is 
today,  so  that  Byers,  who  has  written  an  exhaustive  monograph  on  the 
subject,2  holds  that  nearly  all  of  these  growths  are  of  the  nature  of  fibroma, 
of  the  type  known  as  the  false  neuroma.  They  tend  to  involve  the  intra- 
cranial as  well  as  the  intra-orbital  portions  of  the  nerve,  extending  some- 
times into  the  chiasm.  Owing  to  the  wide  distribution  of  the  process, 
the  association  of  the  fibrosis  with  the  lymph-channels,  the  cedematous 
appearance  of  the  tissue  that  is  so  often  present,  Byers,  further,  compares 
the  condition  to  elephantiasis  and  suggests  the  term  fibromatosis  of  the 
optic  nerve  as  the  correct  designation  for  the  condition.  Such  tumors 

1  Die  Krankheiten  der  Netzhaut  und  des  Sehnerven.     Handb.  der  gesammten 
Augenheilkunde,  Graefe  and  Saemisch,  5:1877:910. 

2  The  Primary  Intradural  Tumors  of  the  Optic  Nerve.     Studies  from  the  Royal 
Victoria  Hospital,  Montreal,  1:  1901:  No.  1. 


650  THE  LENS 

occasionally  exhibit  a  limited  tendency  to  malignancy,  as  do  other  fibrous 
growths.  These  new-growths  occur  in  about  80  per  cent,  of  the  cases 
before  the  age  of  fifteen  years.  They  are  excessively  rare  after  twenty- 
five.  Females  are  slightly  more  often  attacked  than  males,  and  the  left 
eye  is  somewhat  more  often  affected  than  the  right. 

Fibromas  of  the  optic  nerve  start  from  the  arachnoidal  or  pial  covering, 
and  are  apt  to  extend  anteroposteriorly  rather  than  laterally.  They  tend 
to  compress  the  nerve  trunk,  which  early  undergoes  atrophy.  Loss  of 
vision  and  proptosis  of  the  eyeball  are  characteristic  symptoms. 

A  few  cases  of  endothelioma  have  been  recorded  (Alt,  Tailor,  Kalt). 
These  have  been  occasionally  recorded,  though  incorrectly,  as  carcinoma, 
alveolar  sarcoma,  or  fibrosarcoma.  Cells  of  flattened  appearance  and 
of  endothelial  type  are  found  aggregated  into  masses,  separated  one  from 
the  other  by  connective  tissue.  Occasionally,  the  characteristic  cells 
are  arranged  concentrically  or  in  whorls  about  a  central,  clear,  refractile 
body.  Such  forms  might,  therefore,  be  included  under  the  category  of 
psammoma. 

One  case  of  neuroma  has  been  reported,  consisting  both  of  medullated 
and  non-medullated  fibers. 

The  extradural  tumors  are  the  fibroma,  endothelioma,  and  sarcoma. 
They  originate  from  the  connective  tissue  of  the  structure  or  its 
endothelial  lining. 

The  Lens. 
CONGENITAL  ANOMALIES. 

Several  forms  of  partial  congenital  cataract  are  recognized.  The 
opacity  often  assumes  geometrical  forms  and  usually  involves  both 
lenses.  Remains  of  the  hyaloid  artery  may  be  found  in  the  form  of 
fibrous  strands  or  membranes  of  the  posterior  surface  of  the  lens  (posterior 
polar  cataract),  or  on  the  anterior  surface.  Other  anomalies  are  con- 
genital luxation  of  the  lens  and  coloboma. 


RETROGRESSIVE  METAMORPHOSES. 

The  anterior  surface  of  the  lens  is  covered  by  a  single  layer  of  epi- 
thelial cells  which  extend  backward  a  short  distance  behind  the  equator 
of  the  lens  and  then  gradually  become  converted  into  lens  fibers.  This 
epithelium  is  capable  of  overgrowth  and  may  undergo  a  thickening  known 
as  capsular  cataract.  The  transparent  fibers  of  the  lens  proper,  however, 
do  not  appear  to  be  endowed  with  the  same  vitality,  and  are  capable 
only  of  degeneration.  Degeneration  of  the  fibers  gives  rise  to  lenticular 
cataract. 

Capsular  Cataract. — Simple  capsular  cataract  is  usually  due  to  the 
pressure  of  the  lens  upon  the  cornea  in  cases  where  the  cornea  has 
been  perforated  and  the  aqueous  humor  has  drained  away.  We  see  it, 
therefore,  in  connection  with  ulceration  of  the  cornea  and  in  corneal 
staphyloma.  Occasionally,  a  central  capsular  cataract  is  found  in  con- 


LENTICULAR  CATARACT  651 

nection  with  ophthalmia  neonatorum,  even  where  there  is  no  corneal 
perforation.  Penetrating  injuries  of  the  lens  may  also  bring  about 
capsular  cataract,  but  this  is  usually  associated  with  lenticular  cataract. 

The  histological  appearances  indicate  that  the  epithelium  of  the  cap- 
sule, usually  about  the  centre  of  the  anterior  surface  of  the  lens,  under- 
goes proliferation,  gradually  becoming  stratified.  Eventually,  the  newly 
formed  cells  are  converted  into  spindles  and  later  into  fibers,  poor  in 
nuclei  and  resembling  connective-tissue  fibers. 

Lenticular  Cataract. — Lenticular  cataract  is  often  found  in  connec- 
tion with  affections  of  the  retina  and  choroid,  and  in  cyclitis.  Other 
well-known  forms  are  those  resulting  from  traumatism,  diabetes,  and 
old  age. 

In  injuries  to  the  lens  involving  penetration  of  its  capsule,  we  usually 
get  a  combined  form  of  cataract.  If  the  rupture  be  slight  it  may  be 
covered  by  the  iris  or  closed  by  the  formation  of  a  capsular  cataract. 
Thus,  a  small  localized  opacity  may  result  which  may  eventually  be 
absorbed.  In  more  extensive  injuries,  the  capsule  retracts  and  the 
aqueous  humor  soaks  into  the  substance  of  the  lens.  The  fibers  of  the 
lens  begin  to  swell,  become  opaque  and  protrude  through  the  opening. 
As  the  extruded  tissue  is  absorbed  or  detached,  other  fibers  in  their  turn 
prolapse,  and  this  process  goes  on  until,  if  the  nucleus  be  not  too  hard, 
the  whole  substance  of  the  lens  is  absorbed.  Leukocytes  may  pass  into 
the  lens,  also,  and,  later,  vascular  connective  tissue,  so  that  we  get  a 
fibrous  cataract,  in  which  calcareous  salts  are  often  deposited.'  A 
formation  of  bone  may  also  take  place. 

In  diabetes,  the  aqueous  humor  appears  to  undergo  changes  in  its 
chemical  composition  which  result  in  degeneration  of  the  lens  fibers. 
They  become  granular  and  disintegrate  and  the  subcapsular  epithelial 
cells  degenerate  and  atrophy. 

In  senile  cataract  it  is  believed  that  the  first  step  is  the  formation  of 
a  fissure  near  the  equator  of  the  lens  owing  to  traction  on  the  peripheral 
lamellae  and  shrinkage  of  the  nucleus.  The  clefts  fill  with  liquid  and 
the  cortical  fibers  begin  to  swell  up  and  become  vesicular  from  imbibi- 
tion. The  fibers  next  present  small  globules  in  their  substance,  and 
clear,  homogeneous,  myelin  masses  and  fatty  globules  are  found  in  the 
tissue  spaces.  Later,  the  superficial  fibers  become  detached  from  the 
capsule  and  the  cortex  is  converted  into  a  soft,  pulpy,  or  semifluid  mass. 
This  is  the  stage  of  tumefaction.  Next,  the  liquid  is  absorbed  and  the 
lens  returns  to  its  normal  size.  The  cataract  is  then  said  to  be  "ripe." 
The  degeneration  may  go  on  farther,  and  the  cataract  is  then  said  to 
be  "over-ripe."  The  whole  of  the  cortical  portion  may  eventually  be 
converted  into  a  milky  liquid  in  which  the  nucleus  floats  (Morgagnian 
cataract),  or,  again,  the  liquid  may  be  entirely  absorbed,  leaving  only 
the  nucleus  within  the  capsule  (membranous  cataract).  In  some  cases  of 
advanced  senile  cataract  a  capsular  cataract  develops  also. 

In  the  so-called  lamellar  cataract  there  is  an  opaque  zone  between 
the  cortex  and  the  nucleus.  The  condition  is  commonly  met  with  in 
rickety  children,  and  affects  both  eyes. 


654 


THE  ORBIT 


some  of  the  contiguous  structures.  The  benign  tumors  that  have  been 
observed  are  the  angioma,  lymphangioma,  osteoma,  and  various  forms 
of  teratoid  new  formations,  such  as  dermoid  cysts,  rhabdomyoma,  and 
myoneuroma. 

Cysts  of  various  kinds  are  also  found,  such  as  hygroma,  meliceris, 
fatty  and  oil  cysts,  atheromatous,  hematomatous,  and  steatomatous 
cysts,  many  of  which  are  congenital  and  perhaps  related  to  the  dermoids. 

The  most  common  primary  malignant  growth  of  the  orbit  is  the 
sarcoma,  usually  the  round-  or  spindle-celled  form.  Osteosarcoma,  myxo- 
sarcoma,  cylindroma,  and  myofibrosarcoma  are  also  described.  Ghloroma 
occurs  and  apparently  arises  from  the  periosteum.  Sarcomas  and 
endotheliomas  originating  in  the  antrum,  brain,  or  pituitary  gland  may 
involve  the  orbit  secondarily. 

FIG.  177 


Exophthalmos  from  an  intra-orbital  growth.     (From  the  Ophthalmological  Clinic, 
Montreal  General  Hospital.) 

Carcinoma  is  always  secondary  and  originates  from  the  eyelids,  the 
episcleral  tissue,  or  the  lacrymal  gland. 

New-growths  originating  in  the  orbit  may  lead  to  marked  dislocation 
of  the  eyeball  (proptosis;  exophthalmos). 

Injuries. — Extravasations  of  blood  may  take  place  into  the  orbital 
tissue  as  a  result  of  contusions,  wounds,  or  fractures.  Fracture  of  the 
inner  wall  of  the  orbit  opening  up  communication  with  the  nose  or  nasal 
duct  often  lead  to  emphysema  of  the  orbital  cellular  tissue. 


CHAPTER    XXX. 

THE  EAR. 
CONGENITAL  ANOMALIES. 

CONGENITAL  defects  of  the  ear  may  affect  the  whole  auditory  apparatus 
or  a  part  of  it.  Malformations  of  the  external  and  middle  ear  are  gener- 
ally unilateral,  rarely  bilateral,  and  are  often  associated  with  other 
developmental  anomalies,  such  as  harelip,  cleft  palate,  club-foot,  hernia, 
persistent  branchial  clefts,  and  facial  hemiatrophy. 

Complete  absence  of  the  auricles  is  exceedingly  rare.  It  is  said  that  in 
China  there  is  a  species  of  sheep,  called  the  Yung-ti,  in  which  this  pecu- 
liarity is  of  normal  occurrence.  In  the  case  of  the  auricle,  the  helix, 
antihelix,  or  tragus  may  be  defective,  or  the  whole  structure  is  stunted 
(microtia).  In  other  cases  the  auricle  is  exceedingly  large  (macrotia), 
either  as  a  whole  or  in  part;  or  there  may  be  supernumerary  auricles 
(polyotia).  These  additional  auricles  are  usually  unilateral,  and  may 
be  more  or  less  perfect.  As  a  rule,  they  are  pre-auricular  appendages, 
but  may  be  found  on  the  neck,  cheek,  or  shoulder.  Similar  peculiarities 
seem  to  have  been  known  from  remote  times,  for  there  is  in  the  British 
Museum  the  head  of  an  ^Egipan  with  an  accessory  auricle.  The  Satyrs 
are  also  represented  with  goat-like  ears.  Readers  of  Hawthorne's 
"Marble  Faun"  will  remember  the  interesting  way  in  which  the  story 
is  made  to  centre  around  an  inherited  anomaly  of  this  kind.  Not 
infrequently,  small,  scar-like  grooves,  or  fistulas,  discharging  a  creamy 
fluid,  are  found  on  the  ear,  remnants  of  the  primitive  gill-clefts  (fistula 
auris  congenital). 

Complete  absence  of  the  external  auditory  meatus  has  been  observed. 
It  is  generally  unilateral,  but  may  be  bilateral.  The  condition  is  not 
necessarily  associated  with  impairment  of  hearing.  The  site  of  the 
meatus  is  sometimes  indicated  by  a  shallow  groove,  but  even  this  may  be 
wanting.  The  atresia  may  be  due  to  bone  or  membrane.  Partial 
atresia,  the  presence  of  connective-tissue  bands  traversing  the  passage 
(Moos1),  hour-glass  constriction  of  the  cavity,  abnormal  wide  ness  or 
reduplication  of  the  passage  (Schwartze),  have  been  observed.  As  a 
rule,  these  conditions  are  associated  with  other  defects  of  the  auricles, 
membrana  tympani,  or  middle  ear. 

Congenital  anomalies  in  development,  configuration,  and  position  of 
the  membrana  are  fairly  common. 

Absence  of  the  membrana  is  only  met  with  together  with  defect  of  the 
external  auditory  meatus  and  tympanic  cavity.  Small  fissures  of  the 

1  Klinik  der  Ohreiikrankheiten,  85. 


656  THE  EXTERNAL  EAR 

membrana,  often  bilateral,  are  occasionally  met  with  as  a  result  of  non- 
closure  and  explain  how  certain  persons  are  able  to  emit  tobacco  smoke 
from  the  ear. 

The  middle  ear  with  its  contained  ossicles  may  be  completely  absent 
or  rudimentary.  Atresia  may  be  present,  partial  or  complete  absence  of 
the  foramen,  abnormal  wideness  or  reduplication  of  the  same.  The 
incus  and  stapes  have  been  found  to  be  fused.  In  such  cases  the  hearing 
may  be  practically  normal. 

The  Eustachian  tube  may  be  absent  in  cases  of  defect  of  the  external 
ears  and  rudimentary  development  of  the  tympanic  cavity  and  labyrinth. 
Kinking  of  the  tube  or  abnormal  position  of  its  pharyngeal  opening  are 
more  frequent.  Stenosis  is  rare. 

Congenital  malformations  of  the  internal  ear  are  occasionally  met 
with.  They  are  usually  bilateral,  and  may  be  associated,  though  not 
invariably  so,  with  other  developmental  anomalies  of  the  auditory 
apparatus.  The  labyrinth  may  be  wholly  or  partially  defective,  the 
cochlea  may  be  undeveloped,  or  the  modiolus  or  lamina  spiralis.  The 
aqueducts  may  be  dilated  or  reduplicated.  When  the  labyrinth  is 
defective,  the  auditory  nerve  may  also  be  absent  or  end  in  a  bulbous 
swelling  in  the  bone. 

The  External  Ear. 
ANOMALIES  OF  SECRETION. 

Anomalies  of  secretion  of  the  sebaceous  and  ceruminous  glands  are  not 
infrequently  met  with.  Secretion  may  be  scanty  with  marked  dryness 
of  the  surface  of  the  canal.  This  is  seen  in  association  with  certain 
affections  of  the  middle  ear  (chronic  sclerosing  otitis  media),  and  is 
apparently  trophic  in  character.  Hypersecretion,  with  its  numerous 
unpleasant  accompaniments,  is  of  frequent  occurrence  especially  in 
those  suffering  from  chronic  middle-ear  disease.  Under  ordinary  cir- 
cumstances, the  cerumen  is  gradually  removed  to  the  exterior  by  the 
movements  of  the  jaw  communicated  to  the  meatus,  and  by  the  natural 
exfoliation  of  the  epithelium.  In  certain  cases,  however,  favored  by 
excessive  production  of  wax,  collapse  of  the'  meatus,  or  change  in  the 
nature  of  the  secretion,  whereby  it  becomes  tough  and  viscid,  the  cerumen 
accumulates  and  tends  to  become  impacted.  The  hypersecretion  is 
sometimes  excited  by  persistent  hyperemia  of  the  meatus,  but  in  other 
cases  appears  to  be  a  trophic  disturbance.  Should  foreign  bodies  be 
present,  such  as  hair,  epithelial  plugs,  cotton  wool,  etc.,  the  secretion 
will  tend  to  remain  within  the  meatus  and  may  be  impacted  by  mis- 
directed efforts  to  remove  it.  The  pressure  of  impacted  wax  rarely,  if 
ever,  causes  any  serious  disorder  of  the  auditory  canal,  although  excoria- 
tion or  inflammation  of  the  canal  and  membrana  tympani  are  of  not 
infrequent  occurrence.  When  enlargement  of  the  meatus  or  destruction 
of  the  drum  membrane  is  present,  this  is  most  probably  to  be  attributed 
to  antecedent  suppurative  processes. 


OTHEMATOMA  657 

Keratosis  Obturans.— Keratosis  obturans  (Wreden;  Burnett)  is  a 
rather  rare  condition  in  which  the  meatus  is  obstructed  by  a  material 
at  first  sight  not  unlike  impacted  cerumen.  The  substance  is,  however, 
tough  and  adherent,  and  of  a  lighter  color  than  ordinary  wax.  After 
removal,  which  is  difficult,  the  meatus  is  always  found  to  be  more  or 
less  eroded.  The  material  in  question  is  composed  of  an  accumulation 
of  the  horny  elements  of  the  skin  lining  the  cavity  and  the  condition 
probably  results  from  a  chronic  otitis  externa.  The  condition  is  some- 
what similar  to,  if  not,  indeed,  identical  with,  the  so-called  cholesteatoma, 
which  originates  in  the  tympanic  cavity. 


CIRCULATORY  DISTURBANCES. 

Hyperemia. — Hyperemia  of  the  parts  may  be  due  to  inflammation 
or  trauma,  resulting  from  mechanical,  thermic,  or  chemical  insults. 
It  is  seen  also  in  paretic  or  paralytic  conditions  of  the  sympathetic 
nerve  and  the  vasomotor  fibers  of  the  cervical  plexus. 

Hemorrhage. — Hemorrhage,  in  the  form  of  small  ecchymoses  or 
blebs  in  the  skin,  occasionally  results  from  traumatism  and  occurs  in 
severe  inflammation  of  the  auricle  or  middle  ear.  »  ! 

Othematoma. — The  most  striking  form  is  the  extensive  extravasa- 
tion of  blood  known  as  othematoma,  or  haematoma  auris,  which  gives  rise 
to  a  bluish-red,  fluctuating,  tumor-like  swelling.  The  effusion  takes 
place  between  the  cartilage  and  the  perichondrium,  or  into  the  substance 
of  the  cartilage  itself.  Two  forms  are  known — the  traumatic,  due  to 
fracture  of  the  cartilage,  and  the  spontaneous.  The  latter  is  some- 
times bilateral  and  symmetrical,  and  is  found  with  most  frequency 
in  the  insane,  although  it  is  also  met  with  in  severe  blood  dyscrasias, 
such  as  leukemia.  Owing  to  the  fact  that  section  of  the  restiform 
bodies  in  experimental  animals  is  sometimes  followed  by  hematoma 
auris,  Brown-Sequard1  would  refer  the  occurrence  of  spontaneous 
hematoma  to  lesions  at  the  base  of  the  brain.  Degenerative  changes  in 
the  cartilage  and  the  formation  of  new  bloodvessels  is  supposed  to  pre- 
dispose to  the  condition.  Cysts  have  been  known  to  develop  in  the 
muscles  of  the  auricle  after  a  hematoma. 


RETROGRESSIVE  METAMORPHOSES. 

The  degenerative  disturbances  are  not  many,  nor  are  they  of  great 
importance.  In  gout,  small,  nodular  deposits  of  uratic  salts  (tophi) 
are  not  infrequently  found  in  the  auricle.  Fissuring,  softening,  necrosis, 
and  partial  calcification  are  also  met  with. 

1  Bull,  de  PAcad.  de  Med.,  34. 
42 


658  THE  EXTERNAL  EAR 


PROGRESSIVE  METAMORPHOSES. 

Exostoses  and  Hyperostoses. — The  progressive  metamorphoses  are 
exostoses  and  hyperostoses,  and  various  forms  of  tumors.  Exostoses 
and  hyperostoses  may  arise  in  any  part  of  the  external  auditory  meatus, 
but  frequently  grow  from  the  upper  or  posterior  part  of  the  canal  just 
in  front  of  the  drum-membrane.  They  form  single  or  multiple  growths, 
pedunculated  or  sessile,  and  of  a  spherical  or  conical  shape.  They  are 
composed  of  cancellous  or  compact  ivory-like  bone.  More  or  less  occlu- 
sion of  the  meatus  results.  These  growths  are  by  some  attributed  to 
preexisting  subperiosteal  abscesses  which  have  made  their  way  to  the 
meatus.  This  is  doubtful.  Others,  again,  think  that  they  are  dependent 
on  gout,  excessive  bathing,  or  hereditary  peculiarities.  It  is  possible 
also  that  they  are  due  to  anomalies  of  development. 

Polypi. — Polypi,  arising  from  exuberant  granulations,  are  rather 
common  in  the  meatus.  They  may  be  mucous,  fibrous,  myxomatous, 
and  angiomatous.  Some  contain  hairs;  others,  keratinous  masses  and 
giant  cells. 

Tumors. — Among  tumors  should  be  mentioned  the  fibroma,  lipoma, 
osteoma,  angioma,  chondroma,  sarcoma,  and  carcinoma.  The  sarcomas 
are  usually  spindle-celled,  occasionally  round-celled,  fibrosarcomas,  and 
osteosar comas.  The  chloroma,  a  rare  tumor  of  sarcomatous  type  and 
of  pale  green  color,  which  sometimes  originates  from  the  temporal  bone, 
may  invade  the  meatus. 

Sebaceous  and  Dermoid  Cysts. — Sebaceous  and  dermoid  cysts  have 
also  been  described.  Various  forms  of  warts  and  nevi  are  found  on  the 
auricle  but  are  rare. 

INFLAMMATIONS. 

Inflammation  of  the  auricle  may  be  primary  or  secondary  to  lesions 
in  the  neighborhood.  Practically  all  the  diseases  in  the  skin  may  be 
found  affecting  this  part.  Most  of  these  need  only  be  mentioned  here. 
They  are,  the  acute  exanthemata,  erysipelas,  erythema,  acne,  ecthyma, 
eczema,  herpes,  impetigo  contagiosa,  psoriasis,  pemphigus,  seborrhcea, 
pityriasis  alba,  lupus,  syphilis,  actinomycosis,  lepra,  ichthyosis,  ele- 
phantiasis, scleroderma,  and  gangrene. 

Common  affections  of  the  auricle  are  chilblain  and  frostbite  (pernio). 
Both  conditions  have  this  in  common,  that  profound  circulatory  dis- 
turbances are  induced  as  a  result  of  various  degrees  of  cold.  In  the 
milder  form  the  auricle  is  swollen,  bluish-red,  and  very  itchy.  When 
actual  freezing  has  taken  place,  the  organ  is  much  reddened  and  swollen, 
intensely  painful,  and  may  be  covered  with  blebs  containing  a  yellowish 
or  bloody  fluid.  In  more  severe  cases  ulceration  or  even  gangrene  may 
result,  and  more  or  less  deformity  remains. 

Gangrene. — Gangrene  is  seen,  rarely,  in  erysipelas  and  typhoid  fever. 
Noma  may  occur  in  young,  debilitated  infants.  Urbantschitsch  has 
recorded  a  case  of  Raynaud's  disease  of  the  ear. 


OTITIS  EXTERN  A  659 

Perichondritis. — Perichondritis  of  the  auricle  and  meatus  is  a  rare 
affection.  It  gives  rise  to  a  fluctuating  tumor  on  the  concavity  of 
the  auricle,  not  unlike  the  othematoma.  The  process  generally  begins 
in  the  external  auditory  canal  and  thence  extends  to  the  concha  and  other 
portions  of  the  auricle;  the  tragus  alone  escapes.  The  meatus  is  liable 
to  be  stenosed  from  inflammatory  oedema.  The  fluid  contained  within 
tin*  swelling  is  at  first  clear  and  serous,  subsequently  becoming  purulent. 
The  periosteum  is  stripped  from  the  underlying  cartilage,  and  necrosis  of 
this  structure  may  occur.  Complete  restitution  to  the  normal  may 
take  place,  but  there  is  usually  some  shrinkage.  Ossification  occurs, 
but  is  rare.  The  left  auricle  is  the  one  usually  involved.  Some  cases, 
recorded  by  Haug,  have  been  tuberculous. 

Besides  these  frankly  inflammatory  disturbances,  there  is  another 
affection  that  should  be  mentioned,  characterized  by  a  painless  and 
afebrile  collection  of  a  clear,  yellow  fluid  between  the  cartilages  and 
perichondrium.  After  evacuation  the  condition  readily  clears  up.  These 
cysts  are  attributed  by  Hartmann1  to  previous  degenerative  softening 
of  the  part  and  formation  of  local  collections  of  fluid. 

Otitis  Externa  Circumscripta;  Furunculosis ;  Follicular  Inflam- 
mation.— Otitis  externa  circumscripta  is  a  fairly  common  affection 
of  the  meatus  in  adults,  though  much  rarer  in  children.  It  is  usually 
caused  by  an  infection  of  the  sebaceous  and  ceruminous  follicles  with 
pyogenic  cocci,  but  occasionally  originates  in  the  cartilage  and  peri- 
chondrium. The  direct  exciting  factors  are  trauma  of  all  kinds,  chronic 
discharges  from  the  ear,  and  chronic  eczema.  Anemia,  gout,  diabetes 
mellitus,  and  disorders  of  menstruation  are  said  by  some  to  be  predis- 
posing causes.  Occasionally,  pathological  conditions  of  the  nerves  of 
the  meatus  cause  a  trophic  or  so-called  "sympathetic"  furunculosis 
of  the  opposite  ear.  This  affection  is  characterized  by  the  production  of 
furuncles  or  boils,  almost  invariably  in  the  cartilaginous  portion  of  the 
canal.  Several  furuncles  may  appear  together  or  may  develop  one 
after  the  other.  In  severe  cases  deep-seated  abscesses  may  be  formed, 
and  the  tissues  around  the  ear  are  reddened  and  swollen.  The  neighbor- 
ing lymph-glands  are  commonly  swollen  and  tender,  and  even  the  parotid 
may  be  involved.  Spontaneous  resolution  rarely  occurs,  and,  as  a  rule, 
the  abscess  breaks  externally.  Polypoid  granulations  frequently  form 
about  the  edges  of  the  ulcer. 

Otitis  Externa  Diffusa. — Otitis  externa  diffusa  may  be  acute  or 
chronic.  It  is  rarely,  if  ever,  idiopathic,  but  can  usually  be  traced  to 
various  traumatic  causes,  chief  among  which  is  the  instillation  of  irritating 
fluids.  It  occurs  also  in  erysipelas,  acute  and  chronic  exanthemata, 
and  suppurative  otitis  media.  The  affection  is  not  uncommon,  especially 
in  weakly  and  badly  nourished  children.  The  condition  should  always 
arouse  the  suspicion  of  deeper-seated  disease.  The  part  affected  is 
usually  the  osseous  portion  of  the  canal,  but  the  process  may  extend  to 
the  membrana  and  the  outer  parts.  The  lining  membrane  of  the  meatus 

*  Zeitschr.  f.  Ohrenh.,  15:  156;  and  18:  42. 


660  THE  EXTERNAL  EAR 

is  reddened,  swollen,  and  covered  with  discharge,  at  first  serous  but 
later  purulent.  Sometimes,  from  the  presence  of  secretion,  together 
with  exfoliated  cells,  a  kind  of  membrane  is  formed.  In  some  cases  the 
entire  epidermis  may  be  thrown  off  repeatedly,  leaving  each  time  a 
reddened,  moist  surface  which  quickly  becomes  covered  with  new 
epithelium  (otitis  externa  exfoliativa). 

Membranous  Otitis  Externa. — Membranous  otitis  externa  is  rare 
as  a  primary  affection.  It  usually  arises  from  an  angina,  such  as 
that  in  scarlatina,  which  has  extended  to  the  middle  ear.  The  osseous 
portion  of  the  canal  and  the  membrana  tympani  are  first  and  chiefly 
involved,  but  the  process  may  gradually  extend  outward.  The  parts  are 
covered  with  a  dirty  white,  firm,  and  adherent  membrane,  which,  when 
removed,  leaves  a  bleeding  excoriated  surface. 

Diphtheria. — True  diphtheria  of  the  external  auditory  meatus  is  also 
rare  as  a  primary  affection.  It  has  been  observed  in  the  course  of 
epidemics  of  diphtheria  of  the  throat,  but  in  some  cases  there  has  been 
some  previously  existing  inflammation  or  excoriation  of  the  parts  which 
predisposed  to  infection.  Much  more  commonly  the  disease  is  secondary 
to  diphtheria  of  the  throat  and  middle  ear. 

Phlegmonous  Inflammation. — Phlegmonous  inflammation  of  the 
meatus  arises  from  septic  infection  through  a  wound  or  abrasion  of  the 
surface. 

Periostitis  accompanies  all  forms  of  deep-seated  or  deeply  penetrating 
inflammation,  or  may  be  due  to  an  extension  of  inflammation  from  the 
middle  ear. 

Tuberculosis. — Tuberculosis  takes  the  form  of  a  nodular  perichon- 
dritis  or  a  miliary  tuberculosis  of  the  skin.  The  first  form  is  found  in 
the  concha,  the  soft  tissues  of  which  are  reddened  and  oedema tous. 
Small  nodules  form  resembling  fibromas,  which  grow  slowly  and  do 
not  tend  to  soften.  If,  however,  there  be  no  operative  intervention, 
after  a  considerable  time  tuberculous  ulcers  and  fistulse  form  upon  the 
skin  and  portions  of  the  cartilage  may  become  sequestrated.  The  neigh- 
boring glands  are  swollen  and  tender.  The  infection  is  said  by  Haug 
to  arise  from  the  practice  of  piercing  the  ears,  or  from  wearing  the  ear- 
rings of  a  tuberculous  person. 

Syphilis. — Primary  syphilis  of  the  auricle  is  rare,  but  has  been  de- 
scribed by  Zucker.  The  auricle  was  greatly  swollen  and  ulcers  were 
present  on  the  anterior  surface  of  the  tragus.  The  neighboring  glands 
were  enlarged.  Secondary  syphilitic  ulcers  are  much  more  common. 
They  form  by  preference  at  the  point  where  the  ear  is  pierced  for  an  ear- 
ring. Such  ulcers  are  deep,  crateriform,  with  sharp,  indurated  edges. 
In  tertiary  syphilis  gummas  form,  or  there  is  a  perichondritis. 

Parasites. — Otomycosis  is  a  parasitic  affection  of  the  external  audit- 
ory passage  produced  by  various  forms  of  moulds.  As  a  rule,  the  asper- 
gillus  is  the  infecting  agent.  About  60  cases  of  otomycosis  aspergillina 
have  been  recorded,  mostly  in  Germany  and  the  United  States.  It 
is  a  decidedly  rare  affection  in  Canada,  only  4  cases  having  been 
recorded  in  Montreal  in  many  years.  The  forms  usually  found  are  the 


,   OTOMYCOSIS   ASPERGILLINA  661 

Aspergillus  niger,  the  A.  fumigatus,  the  A.  flavus,  and,  exceedingly 
rarely,  the  A.  glaucus.  A  case  of  the  last-mentioned  form  of  the 
affection  has  been  recently  recorded  by  Dr.  H.  S.  Birkett  and  one  of  us 
(A.  G.  N.1). 

The  disease  is  most  common  in  adults,  and  is  liable  to  affect  those 
who  live  in  damp  houses  and  under  unfavorable  hygienic  conditions. 
Inasmuch  as  it  has  been  found  impossible  to  cultivate  these  organisms 
in  the  healthy  ear,  it  is  improbable  that  the  disease  is  ever  spontaneous, 
but  more  likely  that  some  disease  has  preexisted.  Moisture  and 
warmth  appear  to  be  the  two  essential  factors.  Maceration  and  loosen- 
ing of  the  superficial  epithelium,  with  the  formation  of  a  neutral 
or  slightly  acid  medium,  such  as  is  produced  by  dermatitis,  eczema, 

FIG.  178 


Aspergillus  nigricans  from  the  meatus.  A,  myceUum  covered  with  numerous  fallen  spores; 
B,  hypha ;  C,  sporangium  with  ripe  spores  ;  Bf,  hypha  ;  D,  receptaculum  :  E,  sterigmata 
with  spores.  (Politzer.) 

and  psoriasis,  so-called,  provide  the  most  favorable  conditions.  In- 
fection probably  takes  place  through  the  instillation  of  substances,  such 
as  oil,  which  contain  the  spores.  The  mycelium  of  the  fungus  grows 
in  the  rete  Malpighii  and  gradually  penetrates  the  deeper  structures, 
while  the  hyphse  project  into  the  cavity.  Owing  to  the  fungus  extending 
to  the  sensitive  parts,  or  possibly  from  the  presence  of  some  toxin,  irrita- 
tion and  a  certain  amount  of  inflammation  result. 

On  examination,  upon  the  walls  of  the  meatus,  or  even  on  the  drum- 
membrane  (myringomycosis  aspergillina,  Wreden),  can  be  seen  mem- 
branous patches,  or  the  meatus  is  filled  with  a  dirty  detritus  of  waxy 
consistence,  like  desquamating  epithelium,  covered  with  mould  of  a 
black-brown  or  dull  grayish-green  color,  according  to  the  nature  of 
the  fungus  present.  In  several  cases  recorded,  the  disease  has  spread 
to  the  middle  ear,  and  in  two  was  apparently  the  cause  of  perforation 

1  Birkett  and  Nicholls,  Otomycosis  Due  to  the  Aspergillus  Glaucus,  Montreal  Med. 
Journ.,  33:1904:338. 


662  THE  DRUM-MEMBRANE 

(Pollitzer,  Bezold).  Among  the  rarer  fungi  that  have  been  found  in  the 
ear  may  be  mentioned  the  Mucor  mucedo,  Mucor  corymbifer,  Tricho- 
theceum  roseum,  Ascophora  elegans,  Otomyces  purpureus,  Pityriasis 
versicolor,  and  the  O'idium  albicans. 

Foreign  Bodies. — A  great  variety  of  substances,  provided  only 
they  be  small  enough,  may  be  found  in  the  auditory  canal.  Such  are 
seeds,  peas,  nut-shells,  beads,  buttons,  cotton,  wool,  sand,  gravel. 
Matches  or  toothpicks,  which  have  been  inserted  to  relieve  itching, 
have  been  broken  off  and  portions  left  behind.  The  larvce  of  flies,  or 
even  a  living  fly,  have  been  found,  as  has  also  the  Acarus  folliculorum. 
Animal  parasites  are,  however,  much  more  common  in  the  lower  animals. 
Concretions  of  carbonate  or  phosphate  of  lime  are  also  met  with  somewhat 
frequently  in  the  lower  animals  but  rarely  in  man. 

Foreign  bodies  of  all  kinds  are  liable  to  excite  congestion  and  inflam- 
mation, and  may,  by  pressure  upon  the  sensitive  structures,  give  rise  to 
marked  symptoms.  Some  of  these  are  of  reflex  character  and  affect 
the  fibers  of  the  trigeminus  and  vagus  nerves.  Epileptiform  convulsions, 
vertigo,  mental  depression,  neuralgic  pains,  and  laryngeal  cough  have 
all  been  found  resulting  from  this  condition. 

The  Drum-membrane. 

The  drum  membrane  forms  the  boundary  between  the  external 
auditory  meatus  and  the  middle  ear.  On  the  outer  side  it  is  covered 
by  a  thin  layer  of  cutis  continuous  with  the  epidermis  of  the  meatus, 
and  on  the  inner  side,  by  the  mucous  membrane  of  the  tympanic  cavity. 
As  will  be  readily  understood,  from  its  position,  it  is  especially  liable 
to  be  involved  in  disease  of  either  of  the  before-mentioned  cavities. 
Primary  lesions  are  rare. 

CIRCULATORY  DISTURBANCES. 

Hyperemia. — Hyperemia  may  affect  the  external  epidermal  layer, 
the  mucous  surface,  or  both.  In  milder  grades  the  vessels  are  seen 
to  be  large  and  distended,  while  in  the  severer  forms  the  membrana  is 
diffusely  reddened. 

Hemorrhage. — Hemorrhage  into  the  membrana  occurs  on  either 
surface  and  takes  the  form  of  punctiform  or  linear  ecchymoses.  Those 
on  the  cuticular  surface  tend  to  spread  upward  and  posteriorly  toward 
the  periphery.  They  result  frequently  from  traumatism.  In  typhoid, 
variola,  endocarditis,  and  scurvy,  bluish-red,  sharply-defined  elevations 
may  be  found  on  the  mucous  covering. 

INFLAMMATIONS. 

Myringitis. — Inflammation  of  the  tympanic  membrane  (myringitis) 
is  acute  or  chronic.  The  membrane  is  rarely  involved  alone,  but  may 


MYRINGITIS  663 

be  inflamed  from  injury,  or  by  extension  from  the  middle  ear.  Sea- 
bathing, foreign  bodies,  and  careless  instrumentation  may  produce  it. 

In  the  acute  form  the  membrana  is  congested  and  swollen,  and  on  ex- 
amination the  handle  of  the  malleus  and  the  short  process  can  no  longer 
be  seen.  Later,  the  superficial  epithelium  becomes  macerated  and  is 
cast  off.  Microscopically,  all  the  layers  are  swollen,  cedematous,  and 
infiltrated  with  round  cells.  The  mucous  membrane  is  especially  con- 
gested and  greatly  thickened  from  the  exudation  of  inflammatory  prod- 
ucts. In  certain  cases  of  intense  inflammation,  localized  collections 
of  pus  are  found  in  the  cutis,  giving  rise  to  small,  yellowish  dots  and 
streaks  (interlamellar  abscesses). 

In  chronic  myringitis  the  membrana  is  swollen  and  infiltrated,  the 
vessels  much  congested  and  varicose,  while  the  surface  is  covered  with 
small  papillary  or  polypoid  granulations  (myringitis  villosd). 

Tuberculosis. — Myringitis  may  be  due  also  to  tuberculosis,  in  cases 
of  tuberculosis  of  the  middle  ear.  Small,  miliary  foci  may  be  seen  or  a 
more  rapidly  caseating  and  destructive  ulceration. 

Syphilis. — Condylomas  and  gummas  have  been  observed  on  the  drum- 
membrane.1  * 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  drum-membrane  may  result  from  severe 
and  long-continued  pressure  upon  it.  This  may  be  caused  by  foreign 
bodies  or  accumulations  of  detritus  in  the  external  auditory  canal,  or 
by  the  pressure  of  the  external  air  in  cases  of  obstruction  of  the  Eustachian 
canal.  The  part  chiefly  affected  is  the  lamina  propria.  The  membrane 
becomes  thin,  more  or  less  transparent,  and  sinks  inward,  or  projects 
externally  like  a  bladder. 


PROGRESSIVE  METAMORPHOSES. 

Among  the  progressive  disturbances  may  be  mentioned  the  polypoid 
outgrowths  that  sometimes  form  on  the  cuticular  or  mucous  surfaces. 
These  are  largely  the  result  of  chronic  inflammation.  Localized  over- 
growths of  the  epidermis  of  the  outer  surface  are  met  with  (cornua 
cutanea),  or  small,  pearly  nodules  the  size  of  a  pin-head. 

A  rare  and  interesting  growth  is  the  cholesteatoma,  which  is  found  on 
the  inner  surface  of  the  drum-membrane.  It  forms  a  tumor-like  mass 
composed  of  more  or  less  concentrically  arranged  scales  and  plates  sur- 
rounded by  a  thin,  vascular  capsule.  When  of  large  size  such  growths 
may  lead  to  atrophy  of  the  bony  parts,  so  that  the  auditory  canal, 
tympanic  cavity,  and  mastoid  cells  are  thrown  into  one  large  cavity. 
The  exact  nature  of  this  growth  is  still  in  dispute.  Virchow  considered 

1  Baratoux,  Bull,  et  m6m.  de  la  Soc.  d'Otol.,  2:2. 


664  THE  EUSTACHIAN  TUBE 

it  to  be  a  true  heterologous  tumor;  others  refer  it  to  a  developmental 
defect — branchiogenic  clefts.  Leutert  believes  that  it  is  a  retention-cyst, 
the  squamous  epithelium  of  the  meatus  or  tympanic  membrane  having 
passed  through  a  perforation  and  developed  into  an  encapsulated  mass. 
Rednew  believes  in  a  variable  etiology,  namely,  that  some  of  these  growths 
are  true  tumors,  while  others  are  epithelial  cysts,  retained  masses  of 
tissue,  or  hyperplastic  epidermis. 


TRAUMATISM. 

Rupture. — Rupture  of  the  drum-membrane  may  arise  from  direct 
or  indirect  violence,  as,  for  instance,  from  the  introduction  of  pointed 
instruments,  condensation  of  the  air,  concussion  and  fracture  of  the  skull. 
In  the  cases  due  to  the  first-mentioned  cause,  the  perforation  is  usually 
in  the  superior  posterior  quadrant.  In  those  due  to  condensation  of 
the  air,  as  from  boxing  the  ears,  concussions,  explosions,  working  in 
caissons,  pulling  the  auricle,  fractures  of  the  skull,  sneezing  or  coughing, 
the  rupture  is  in  the  anterior  quadrant.  Some  few  cases  are  dependent 
on  the  weakening  of  the  membrane  from  pressure  atrophy.  Perhaps 
the  most  frequent  cause  is  necrotic  inflammation  of  the  drum-membrane 
secondary  to  otitis  media.  Only  rarely  does  the  form  of  inflammation 
restricted  to  the  membrana  (interlamellar  abscess)  lead  to  perforation. 
The  membrana  becomes  eroded  and  infiltrated  and,  either  by  direct 
extension  of  the  destructive  process,  or  from  the  pressure  of  contained 
fluid,  or  both,  finally  gives  way.  Any  part  of  the  structure  may  be 
involved.  There  may  be  only  one  perforation  or  several,  varying  in 
size  from  one  extremely  minute  to  destruction  of  the  whole  structure. 
In  the  milder  cases,  the  tear  may  heal  without  any  obvious  alteration  in 
the  part,  but  if  extensive  a  scar  is  formed.  The  lamina  propria  in  such 
cases  is  not  restored.  Large  scars  may  sink  inward  and  become 
attached  to  the  wall  of  the  labyrinth.  Occasionally,  calcification  of 
the  membrana  results,  and  rarely,  ossification. 

The  Eustachian  Tube. 

Owing  to  the  close  relationship  existing  between  the  Eustachian  tube, 
the  nasopharynx,  and  the  tympanic  cavity,  affections,  especially  the 
inflammatory  ones,  of  these  cavities  are  particularly  liable  to  involve 
the  tube  by  extension.  Primary  involvement  of  the  tube  must  be  rare,  if 
indeed  it  occurs.  The  most  important  conditions  met  with  are  various 
alterations  in  the  lumen,  such  as  kinking,  stenosis,  and  dilatation. 

One  of  the  common  causes  of  obstruction  is  the  presence  of  the  naso- 
pharyngeal  or  Luschka's  tonsil.  The  presence  of  this  outgrowth  in  the 
young  child  may  prevent  the  proper  development  of  the  tube,  and  in  all 
individuals,  owing  to  its  close  proximity  to  the  nasopharyngeal  orifice 
of  the  tube,  may  grow  directly  over  it.  In  many  cases,  from  prolonged 
contact,  or  from  the  formation  of  inflammatory  adhesions,  union  takes 


HYPEREMIA  665 

place,  leading  to  traction  upon  the  tubal  lip  and  more  or  less  kinking. 
In  advanced  cases,  the  obstruction  may  become  even  more  extreme, 
inasmuch  as  the  traction  is  greatly  increased,  owing  to  the  fibroid  involu- 
ton  which  the  hypertrophied  tonsil  inevitably  undergoes.  Again,  any 
obstruction  in  the  nasal  cavities  which  limits  the  supply  of  air  to  the  naso- 
pharynx, will  tend  to  produce  a  partial  vacuum  in  that  cavity.  Chronic 
congestion  and  more  or  less  hyperplasia  of  the  mucous  membrane  in 
the  lower  portion  of  the  tube  is  thus  induced  and  brings  about  stenosis. 

Various  inflammatory  processes,  both  within  and  without  the  tube,  or 
the  presence  of  tumors,  may  lead  to  obstruction.  Vegetations  and  polypi 
in  the  middle  ear  may  press  upon  and  occlude  the  upper  end  of  the  tube. 

Where  stoppage  is  complete,  the  air  in  the  middle  ear  is  gradually 
absorbed,  the  tympanic  membrane  is  sucked  in  and  thus  rendered  more 
tense,  so  that  deafness  results.  In  other  cases,  the  tube  is  so  large  that 
its  lumen  is  constantly  patent,  with  the  result  that  passage  of  air  into 
the  middle  ear  produces  autophony,  and  sudden  concussions  of  the 
air  may  lead  to  deafness  and  even  rupture  of  the  membrana  tympani. 
Atrophy  of  the  mucous  membrane  is  a  chief  cause  of  this  state  of  affairs. 

Inflammation. — Catarrhal  and  purulent  inflammation  of  the  tube 
are  not  uncommon  as  a  result  of  nasopharyngeal  catarrh  and  otitis 
media  and  as  complications  of  certain  infectious  fevers,  prominent 
among  which  are  scarlatina,  measles,  diphtheria,  influenza,  and  typhoid. 
The  lining  membrane  of  the  tube  becomes  hyperemic  and  swollen,  so 
that  the  lumen  is  more  or  less  completely  obstructed  and  there  may 
be  abundant  exudation. 

Syphilis,  variola,  and  tuberculosis  may  also  produce  lesions  in  the  tube. 
Prolonged  inflammation  and  congestion  will,  in  time,  lead  to  hyper- 
plasia of  the  mucous  membrane  and  the  submucous  connective  tissue, 
and  stenosis. 

Foreign  Bodies. — Foreign  bodies  in  the  tube  may  also  cause  obstruc- 
tion. The  condition  is  rare.  A  bougie  may  break  off  in  the  course  of 
instrumentation.  Pins,  wire,  and  particles  of  food  have  also  been  known 
to  enter  the  tube. 

The  Middle  Ear. 

The  tympanic  cavity  is  so  closely  connected  by  contiguity  and  the 
anastomosis  of  blood  and  lymph-vessels  that  it  is  particularly  liable  to 
be  involved  in  any  disease  processes  affecting  the  neighboring  parts, 
such  as  the  external  auditory  meatus,  the  nasopharynx,  labyrinth,  and 
cranial  cavity. 

CIRCULATORY  DISTURBANCES. 

Hyperemia. — Active  hyperemia  of  the  mucosa  is  met  with  in  the  early 
stage  of  inflammation. 

Passive  hyperemia  may  be  found  in  general  systemic  congestion,  such 
as  may  occur  in  the  course  of  cardiac  and  pulmonary  disease,  or  as  a 


664  THE  EUSTACHIAN  TUBE 

it  to  be  a  true  heterologous  tumor;  others  refer  it  to  a  developmental 
defect — branchiogenic  clefts.  Leutert  believes  that  it  is  a  retention-cyst, 
the  squamous  epithelium  of  the  meatus  or  tympanic  membrane  having 
passed  through  a  perforation  and  developed  into  an  encapsulated  mass. 
Rednew  believes  in  a  variable  etiology,  namely,  that  some  of  these  growths 
are  true  tumors,  while  others  are  epithelial  cysts,  retained  masses  of 
tissue,  or  hyperplastic  epidermis. 


TRAUMATISM. 

Rupture. — Rupture  of  the  drum-membrane  may  arise  from  direct 
or  indirect  violence,  as,  for  instance,  from  the  introduction  of  pointed 
instruments,  condensation  of  the  air,  concussion  and  fracture  of  the  skull. 
In  the  cases  due  to  the  first-mentioned  cause,  the  perforation  is  usually 
in  the  superior  posterior  quadrant.  In  those  due  to  condensation  of 
the  air,  as  from  boxing  the  ears,  concussions,  explosions,  working  in 
caissons,  pulling  the  auricle,  fractures  of  the  skull,  sneezing  or  coughing, 
the  rupture  is  in  the  anterior  quadrant.  Some  few  cases  are  dependent 
on  the  weakening  of  the  membrane  from  pressure  atrophy.  Perhaps 
the  most  frequent  cause  is  necrotic  inflammation  of  the  drum-membrane 
secondary  to  otitis  media.  Only  rarely  does  the  form  of  inflammation 
restricted  to  the  membrana  (interlamellar  abscess)  lead  to  perforation. 
The  membrana  becomes  eroded  and  infiltrated  and,  either  by  direct 
extension  of  the  destructive  process,  or  from  the  pressure  of  contained 
fluid,  or  both,  finally  gives  way.  Any  part  of  the  structure  may  be 
involved.  There  may  be  only  one  perforation  or  several,  varying  in 
size  from  one  extremely  minute  to  destruction  of  the  whole  structure. 
In  the  milder  cases,  the  tear  may  heal  without  any  obvious  alteration  in 
the  part,  but  if  extensive  a  scar  is  formed.  The  lamina  propria  in  such 
cases  is  not  restored.  Large  scars  may  sink  inward  and  become 
attached  to  the  wall  of  the  labyrinth.  Occasionally,  calcification  of 
the  membrana  results,  and  rarely,  ossification. 

The  Eustachian  Tube. 

Owing  to  the  close  relationship  existing  between  the  Eustachian  tube, 
the  nasopharynx,  and  the  tympanic  cavity,  affections,  especially  the 
inflammatory  ones,  of  these  cavities  are  particularly  liable  to  involve 
the  tube  by  extension.  Primary  involvement  of  the  tube  must  be  rare,  if 
indeed  it  occurs.  The  most  important  conditions  met  with  are  various 
alterations  in  the  lumen,  such  as  kinking,  stenosis,  and  dilatation. 

One  of  the  common  causes  of  obstruction  is  the  presence  of  the  naso- 
pharyngeal  or  Luschka's  tonsil.  The  presence  of  this  outgrowth  in  the 
young  child  may  prevent  the  proper  development  of  the  tube,  and  in  all 
individuals,  owing  to  its  close  proximity  to  the  nasopharyngeal  orifice 
of  the  tube,  may  grow  directly  over  it.  In  many  cases,  from  prolonged 
contact,  or  from  the  formation  of  inflammatory  adhesions,  union  takes 


HYPEREMIA  665 

place,  leading  to  traction  upon  the  tubal  lip  and  more  or  less  kinking. 
In  advanced  cases,  the  obstruction  may  become  even  more  extreme, 
inasmuch  as  the  traction  is  greatly  increased,  owing  to  the  fibroid  involu- 
ton  which  the  hypertrophied  tonsil  inevitably  undergoes.  Again,  any 
obstruction  in  the  nasal  cavities  which  limits  the  supply  of  air  to  the  naso- 
pharynx, will  tend  to  produce  a  partial  vacuum  in  that  cavity.  Chronic 
congestion  and  more  or  less  hyperplasia  of  the  mucous  membrane  in 
the  lower  portion  of  the  tube  is  thus  induced  and  brings  about  stenosis. 

Various  inflammatory  processes,  both  within  and  without  the  tube,  or 
the  presence  of  tumors,  may  lead  to  obstruction.  Vegetations  and  polypi 
in  the  middle  ear  may  press  upon  and  occlude  the  upper  end  of  the  tube. 

Where  stoppage  is  complete,  the  air  in  the  middle  ear  is  gradually 
absorbed,  the  tympanic  membrane  is  sucked  in  and  thus  rendered  more 
tense,  so  that  deafness  results.  In  other  cases,  the  tube  is  so  large  that 
its  lumen  is  constantly  patent,  with  the  result  that  passage  of  air  into 
the  middle  ear  produces  autophony,  and  sudden  concussions  of  the 
air  may  lead  to  deafness  and  even  rupture  of  the  membrana  tympani. 
Atrophy  of  the  mucous  membrane  is  a  chief  cause  of  this  state  of  affairs. 

Inflammation. — Catarrhal  and  purulent  inflammation  of  the  tube 
are  not  uncommon  as  a  result  of  nasopharyngeal  catarrh  and  otitis 
media  and  as  complications  of  certain  infectious  fevers,  prominent 
among  which  are  scarlatina,  measles,  diphtheria,  influenza,  and  typhoid. 
The  lining  membrane  of  the  tube  becomes  hyperemic  and  swollen,  so 
that  the  lumen  is  more  or  less  completely  obstructed  and  there  may 
be  abundant  exudation. 

Syphilis,  variola,  and  tuberculosis  may  also  produce  lesions  in  the  tube. 
Prolonged  inflammation  and  congestion  will,  in  time,  lead  to  hyper- 
plasia of  the  mucous  membrane  and  the  submucous  connective  tissue, 
and  stenosis. 

Foreign  Bodies. — Foreign  bodies  in  the  tube  may  also  cause  obstruc- 
tion. The  condition  is  rare.  A  bougie  may  break  off  in  the  course  of 
instrumentation.  Pins,  wire,  and  particles  of  food  have  also  been  known 
to  enter  the  tube. 

The  Middle  Ear. 

The  tympanic  cavity  is  so  closely  connected  by  contiguity  and  the 
anastomosis  of  blood  and  lymph-vessels  that  it  is  particularly  liable  to 
be  involved  in  any  disease  processes  affecting  the  neighboring  parts, 
such  as  the  external  auditory  meatus,  the  nasopharynx,  labyrinth,  and 
cranial  cavity. 

CIRCULATORY  DISTURBANCES. 

Hyperemia. — Active  hyperemia  of  the  mucosa  is  met  with  in  the  early 
stage  of  inflammation. 

Passive  hyperemia  may  be  found  in  general  systemic  congestion,  such 
as  may  occur  in  the  course  of  cardiac  and  pulmonary  disease,  or  as  a 


666  THE  MIDDLE  EAR 

local  effect  of  pressure,  for  example,  in  the  case  of  tumors  about  the 
head  and  neck. 

Hemorrhages. — Hemorrhages,  either  petechial  in  character  or  a 
free  effusion  of  blood,  may  occur  spontaneously,  or  as  a  result  of  concus- 
sion or  fracture  of  the  skull.  They  may  also  be  caused  by  the  severer 
forms  of  otitis  media  arising  in  the  course  of  diphtheria,  Bright's  disease, 
or  leukemia,  and  by  embolism  of  the  stylomastoid  artery.  Hemorrhages, 
so  extreme  that  the  blood  escapes  externally  through  the  meatus  or  Eus- 
tachian  tube,  are  met  with  in  severe  traumatism  to  the  temporal  bone, 
the  spontaneous  separation  of  polyps  and  in  carious  processes  which 
cause  erosion  of  important  vessels,  such  as  the  carotid,  the  jugular  vein, 
the  transverse  and  superior  petrosal  sinuses. 


INFLAMMATIONS. 

Otitis  Media. — Otitis  media  is  of  fairly  frequent  occurrence  and 
arises  from  a  great  variety  of  causes.  It  may  be  divided  into  simple 
catarrhal  (non-suppurative),  suppurative,  and  specific,  or,  according  to 
its  course,  into  acute  and  chronic. 

Among  the  primary  causes  may  be  mentioned,  trauma,  climatic 
changes,  various  forms  of  inflammation,  and  circulatory  disturbances. 
Perhaps  in  the  majority  of  cases  microorganisms  are  at  work  as  well. 
The  chief  traumatic  causes  are  direct  or  indirect  injuries  to  the  drum- 
head, as  from  careless  instrumentation,  forcible  syringing,  concussion 
and  condensation  of  the  air,  local  irritants,  contusions,  and  fractures  of 
the  skull.  Exposure  to  wet  and  cold,  sea-bathing,  the  aspiration  of 
water,  medicinal  solutions,  or  infected  secretions  through  the  Eustachian 
tube  should  also  be  mentioned.  External  otitis  of  all  forms,  impacted 
wax,  and  foreign  bodies  may  cause  an  inflammation  that  may  extend  to 
the  middle  ear.  Infective  secretions  from  the  accessory  cavities  of  the 
nose  and  nasopharynx  may  reach  the  cavity  through  the  Eustachian 
tube.  The  pus  from  a  retropharyngeal  abscess  has  been  known  to 
burrow  its  way  along  the  sheath  of  the  tensor  tympani  and  infect  the 
cavity.  In  children  perhaps  the  most  frequent  predisposing  cause  is  the 
presence  of  adenoids.  Many  cases  also  are  dependent  on  certain  of 
the  infective  fevers,  such  as  scarlatina,  measles,  variola,  influenza, 
whooping-cough,  and  cerebrospinal  meningitis.  Gout,  syphilis,  and 
tuberculosis  are  also  of  importance.  Some  cases,  finally,  appear  to  be 
due  to  obscure  circulatory  disturbances,  probably  reflex  in  nature.  In 
the  vast  majority  of  instances  bacteria  are  present  from  the  beginning, 
or  make  their  appearance  sooner  or  later.  No  particular  germ  can  be 
regarded  as  the  specific  cause  of  otitis  media,  but  nearly  all  the  known 
pathogenic  bacteria  are  capable  of  exciting  it.  The  infection  may  be 
mixed  or,  again,  one  form  may  die  out  and  be  replaced  by  another. 

With  regard  to  the  frequency  of  the  various  forms  of  microorganisms 
Orne  Green's  analysis  of  101  cases  may  be  cited.  He  found: 


ACUTE  SUPPURATIVE  OTITIS  MEDIA  667 

Staphylococcus  (albus,  8;  aureus,  9;  not  specified,  19)      ...  36 

Streptococcus .19 

Pneumococcus 10 

Bacillus  pyocyaneus 3 

A  capsulated  bacillus 3 

Bacillus  diphtherias 2 

Mixed  infection 28 

In  many  cases  the  infective  agents  reach  the  middle  ear  through  the 
Kustachian  tube,  being  introduced  into  it  by  the  acts  of  coughing,  sneez- 
ing, or  gagging.  When  the  drum-head  is  perforated  the  organisms 
may  enter  from  the  external  auditory  meatus.  In  the  case  of  the  infect- 
ious fevers,  even  in  diphtheritic  and  scarlatinal  angina,  the  invasion  is 
most  probably  through  the  blood  stream. 

Acute  Catarrhal  Otitis  Media. — In  acute  catarrhal  otitis  media  the 
mucous  membrane  is  reddened  and  swollen,  while  the  submucous 
connective  tissue  is  infiltrated  with  inflammatory  products.  The 
cavity  is  more  or  less  filled  with  a  serous  or  more  viscid  mucinous 
secretion,  containing  desquamated  epithelial  cells  with  a  few  mucous 
cells  and  leukocytes.  Examination  of  the  drum-membrane  in  the  early 
stages  will  show  congestion  and  beginning  infiltration  at  the  periphery, 
especially  the  upper  part  and  near  the  manubrium  mallei.  Later  on, 
small  blebs  containing  serum  or  blood  may  be  seen  in  the  epidermis 
near  Shrapnell's  membrane.  In  other  cases  the  drum-membrane, 
particularly  the  posterior  portion,  may  bulge  considerably,  and  even 
perforation  may  occur. 

Chronic  Catarrhal  Otitis  Media. — In  the  chronic  form,  the  mucous 
membrane  is  thickened,  firmer,  of  a  grayish-white  color,  and  presents 
here  and  there  nodular  and  villous  granulations.  The  thickening 
may  affect  the  mucous  membrane  as.  a  whole,  or  may  be  confined  to 
certain  localities,  as  the  drum-membrane,  the  ossicles,  the  orifice  of  the 
Eustachian  tube,  or  the  labyrinth  fenestrum. 

Chronic  catarrhal  otitis  media  frequently  supervenes  upon  an  acute 
process,  or  may  be  chronic  from  the  start.  Most  of  the  causes  producing 
the  acute  form  are  competent  to  produce  the  chronic.  Some  cases  of 
the  chronic  disease,  however,  originate  independently  in  those  of  a  gouty 
or  rheumatic  tendency  or  who  have  syphilis.  The  constant  jarring 
from  loud  noises  may  also  produce  it.  In  such  cases  the  lesion  usually 
begins  in  the  neighborhood  of  the  tubal  orifice.  In  other  instances 
the  affection  begins  about  the  base  plate  of  the  stapes  and  apparently 
depends  on  vasomotor  conditions.  In  some  it  is  said  to  be  hereditary. 
Debilitating  diseases  play  a  part. 

Acute  Suppurative  Otitis  Media. — Acute  suppurative  otitis  media  occurs 
chiefly  in  the  course  of  the  acute  infective  fevers,  as  scarlatina,  measles, 
variola,  diphtheria  and  typhoid.  It  is  not  always  easy  to  draw  a  hard  and 
fast  line  between  the  catarrhal  and  the  suppurative  forms.  Undoubtedly 
many  cases  of  the  latter  originate  in  simple  catarrhal  inflammation. 

The  mucosa  is  greatly  swollen  and  congested,  while  the  cavity  is 


668  THE  MIDDLE  EAR 

filled  with  a  purulent  or  mucopurulent  exudation,  sometimes  mixed 
with  blood.  The  drum-membrane  becomes  infiltrated,  softened,  and 
eroded,  so  that  perforation  is  common.  It  is  not  usual  for  marked 
ulceration  of  the  mucous  membrane  to  occur  except  in  disease  of  the 
most  violent  septic  nature,  but  in  such  cases  the  ossicles  and  wall  of 
the  tympanic  cavity  may  become  necrosed  and  the  infection  extend  to 
the  cranial  cavity,  where  it  may  lead  to  extradural  abscess,  meningitis, 
and  thrombosinusitis.  This  is  particularly  liable  to  ocur  in  children  in 
whom  the  petrososquamosal  suture  not  infrequently  remains  patent  for 
a  long  time,  thus  exposing  the  dura,  which,  furthermore,  may  dip  down 
after  the  fashion  of  a  hernia  through  the  fissure.  The  inflammatory 
exudate  tends  to  gravitate  to  the  bottom  of  the  tympanum,  although  cases 
are  met  with  where,  owing  to  oedema  of  the  mucosa,  the  fluid  remains  pent 
up  in  the  attic.  Hence,  it  may  dissect  its  way  along  the  upper  wall  of 
the  meatus  and  make  its  appearance  externally  behind  the  ear.  In  such 
cases  the  mastoid  cells  are  almost  invariably  involved  as  well. 

Chronic  Suppurative  Otitis  Media. — Chronic  suppurative  otitis  media  is 
invariably  the  result  of  a  previous  attack  or  repeated  attacks  of  the  acute 
form.  Here,  the  thickness  of  the  lining  membrane  is  greatly  increased, 
owing  to  round-celled  infiltration,  congestion  of  the  vessels,  with  hyper- 
plasia  of  the  tissue,  chiefly  of  the  subepithelial  layer,  and  new-formation 
of  bloodvessels.  The  secretion  varies  in  amount  and  is  usually  foul- 
smelling,  mixed  with  blood,  and  of  a  dirty  brown  color.  When  the  secre- 
tion is  retained,  as  it  usually  is,  for  this  is  one  of  the  most  frequent  causes 
of  an  acute  becoming  a  chronic  process,  it  becomes  inspissated,  and,  being 
admixed  with  cast-off  epithelium,  forms  a  tough,  cheesy  mass,  possessing 
a  peculiar  laminated  structure,  known  as  cholesteatoma.  The  drum- 
head is  perforated,  more  or  less  destroyed  and  deformed,  and  what  re- 
mains of  it  may  be  greatly  thickened  and  studded  with  granulations.  In 
certain  cases  the  epidermal  layer  of  the  drum-membrane  penetrates 
through  the  perforation  and  tends  to  involve  the  cavity.  Where  the 
ciliated  epithelium  has  been  exfoliated,  the  exposed  surface  of  the  mem- 
brane is  dark  red  and  actively  secretes  pus,  while  here  and  there  it  is 
dotted  with  fungoid  or  villous-like  granulations.  These  may  proliferate 
to  such  an  extent  that  opposite  masses  of  granulations  may  fuse  and 
give  rise  to  cystic  cavities  or  loculi  lined  with  epithelium. 

Sooner  or  later,  the  destructive  inflammation,  which  has  led  to  erosion 
and  even  ulceration  of  the  mucosa,  extends  to  the  bony  parts,  which 
become  carious  and  necrosed.  The  incus  is  usually  first  attacked,  as  it  is 
somewhat  imperfectly  supplied  with  blood.  Next,  the  malleus  is  involved, 
and  later  that  portion  of  the  tympanic  ring  on  which  the  head  of  the 
malleus  abuts,  as  well  as  the  inner  end  of  the  Eustachian  tube.  When 
the  wall  of  the  tympanic  cavity  is  affected  the  course  is  usually  most 
marked  at  the  tegmen.  The  wall  of  the  labyrinth  may  be  invaded 
so  that  the  Fallopian  canal  and  the  cavity  of  the  labyrinth  are  opened 
up,  leading  to  paralysis  of  the  facial  nerve  and  sometimes  infection  of 
the  cranial  cavity.  The  lower  part  of  the  tympanic  cavity  and  the 
anterior  wall  of  the  labyrinth  are  not  so  often  involved. 


CHRONIC  SCLEROSING  OTITIS  MEDIA  669 

In  very  severe  forms  of  inflammation,  such  as  those  which  in  children 
complicate  the  infective  fevers,  extensive  necrosis  and  sequestration  of 
the  bone  may  take  place.  The  ossicles  may,  owing  to  necrosis  and 
loosening  of  their  attachments,  be  cast  off  and  appear  in  the  discharge 
from  the  meatus.  The  foot-plate  of  the  stapes  usually,  however,  resists 
the  process.  From  the  adjoining  necrosing  inflammation,  together  with 
the  presence  of  retained  secretions,  the  cavity  of  the  middle  ear  becomes 
considerably  enlarged.  In  course  of  time,  owing  to  the  stimulus  of  the 
inflammatory  process  on  the  periosteum  and  the  bone-marrow,  hyper- 
ostoses  and  exostoses  are  formed,  usually  on  the  promontory,  the 
fenestrum  rotundum,  or  the  eminentia  pyramidalis.  Much  more  rarely, 
they  form  upon  the  ossicles  and  orifice  of  the  Eustachian  tube.  In  this 
way  the  cavity  may  in  the  end  become  notably  diminished  in  size.  In 
some  cases  the  ossicles  and  other  bony  plates  become  sclerosed  rather 
than  necrosed.  Invasion  of  the  mastoid  antrum  is  not  infrequent  in 
cases  of  chronic  suppurative  otitis  media,  and  is  always  of  serious 
import,  as  it  may  lead  to  extensive  necrosis  of  the  bone  and  infection  of 
the  cranial  cavity,  or  to  septic  thrombosis  of  the  lateral  sinus.  If 
unrelieved,  or  if  operative  measures  be  undertaken  too  late,  the  jugular 
vein  may  become  thrombosed  for  a  considerable  distance  and  the  patient 
die  from  general  sepsis. 

Besides  the  simple  and  suppurative  forms  of  chronic  otitis  just 
described  there  are  others  of  great  practical  importance,  the  etiology  of 
which  is  not  quite  clear.  These  are  the  adhesive  and  sclerosing  forms. 

Chronic  Adhesive  Otitis  Media. — In  chronic  adhesive  otitis  media 
there  is  a  slowly  progressive  inflammation  which  leads  to  thickening  and 
proliferation  of  the  mucous  membrane  lining  the  tympanic  cavity,  and 
the  formation  of  bands,  membranes,  and  other  adhesions  binding  the 
drum,  the  ossicles,  and  the  tensor  tendon  together,  or  to  the  tympanic 
wall,  or,  again,  traversing  the  cavity.  The  adhesions  are  composed  of 
loose,  avascular  connective  tissue,  covered  with  epithelium. 

Chronic  Sclerosing  Otitis  Media. — Chronic  sclerosing  otitis  media  is 
perhaps  of  greater  importance.  In  some  cases  there  is  a  transformation 
of  the  infiltrated  and  hyperemic  mucosa  into  dense,  scar-like  tissue.  In 
other  cases  it  is  the  deeper  or  periosteal  portion  of  the  mucosa  that  is 
sclerosed.  In  still  other  cases  there  may  be  a  granular  deposit  of  lime 
salts  in  the  submucous  tissue  or  in  the  drum-membrane.  These  changes 
may  affect  the  whole  lining  membrane  of  the  cavity,  or  certain  structures 
may  be  more  particularly  affected,  such  as  the  drum-membrane,  the 
ossicles,  the  promontory,  and  the  window  of  the  labyrinth.  Naturally, 
when  these  lesions  become  pronounced,  ankylosis,  more  or  less  complete, 
takes  place  between  the  ossicles.  Most  frequently  the  attachment  of 
the  stapes  to  the  fenestrum  ovale  is  involved,  or,  more  rarely,  the  articu- 
lation between  the  malleus  and  incus.  Ankylosis  of  the  stapes,  which  in 
some  cases  is  congenital,  is  due  either  to  calcification  of  the  annular 
ligament  attaching  it  to  the  fenestrum  ovale,  or  to  cartilaginous  or  bony 
outgrowths.  The  ankylosis  leads  to  pressure  upon  the  endolymph  of 
the  labyrinth  and  serious  interference  with  the  circulation. 


670  THE  MIDDLE  EAR 

Diphtheritic  Otitis  Media. — Diphtheritic  otitis  media  is  usually 
secondary  to  diphtheria  of  the  nasopharynx.  It  probably  arises  by 
way  of  the  Eustachian  tube,  owing  to  the  aspiration  of  infective  material. 

Tuberculosis. — Tuberculosis  of  the  middle  ear  is  now  a  well-recog- 
nized affection.  It  is  .probably  never  primary,  but  usually  occurs  late 
in  the  course  of  tuberculosis  in  some  other  part  of  the  body.  It  is  thought 
by  some  that  infection  takes  place  through  the  Eustachian  tube,  although 
it  is  not  improbable  that  some  cases  are  hematogenous.  It  is  character- 
istic of  the  affection  that  It  is  insidious  in  its  onset  and  of  slow  progression, 
so  that  the  disease  may,  exist  for  some  time  before  symptoms  arise  to 
draw  attention  to  it.  The  first  sign  is  a  discharge  from  the  meatus, 
and  in  a  short  space  of  time  the  membrane  is  completely  destroyed. 
Then  the  cavity  of  the  tympanum  is  found  to  be  lined  with  an  exuberant 
granulation  tissue,  secreting  acrid  pus,  a  condition  which  may  persist 
for  months.  In  other  cases,  when  the  patient  lives  long  enough,  or  the 
disease  is  more  severe,  the  ossicles  are  loosened  from  their  attachments 
and  cast  off  in  the  discharge,  while  large  portions  of  the  bony  wall 
become  carious,  or  even  large  sequestra  may  be  formed.  The  micro- 
scopic appearances  of  the  diseased  parts  do  not  differ  materially  from 
those  in  other  forms  of  destructive  inflammation,  save  that  in  the  deeper 
layers  of  the  mucous  membrane,  giant  cells  and  patches  of  caseation 
with  bacilli  can  be  seen.  It  is,  however,  a  curious  circumstance  that 
tubercle  bacilli  frequently  cannot  be  discovered  in  the  discharge.  How- 
ever, the  fact  that  there  are  advanced  tuberculous  lesions  in  other  parts 
of  the  body,  the  insidious  nature  of  the  otitis,  and  the  rapid  destruction 
of  the  tissues,  is  practically  conclusive  of  the  nature  of  the  disease.  In 
the  severe  forms  where  there  is  much  destruction  of  bone,  meningitis 
may  develop,  or,  rarely,  a  local  brain-abscess. 

Syphilis. — Syphilis  gives  rise  to  forms  of  acute  and  chronic  otitis 
closely  resembling  those  arising  from  non-specific  causes.  Neuralgia 
of  the  tympanic  cavity  is  also,  rarely,  observed.  Most  authorities  appear 
to  be  agreed  that  in  syphilis  there  is  a  special  tendency  to  implication 
of  the  auditory  nerve. 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — Hyperplastic  outgrowths  and  tumors  are  found  more 
frequently  in  the  middle  ear  than  in  other  parts  of  the  auditory 
apparatus.  They  may  arise  from  any  part  of  the  tympanic  cavity, 
but  generally  spring  from  the  wall  of  the  labyrinth,  the  attic,  or,  occasion- 
ally, from  the  membrana.  The  majority  of  these  form  polypoid  masses, 
either  pedunculated  or  sessile,  of  a  globular  or  nodular  shape,  and  having 
a  smooth  or  papillary  surface.  They  may  attain  such  a  size  as  to  com- 
pletely fill  the  tympanic  cavity  and  even  appear  externally.  They  are 
of  importance,  since  they  tend  to  perpetuate  any  suppurative  process 
that  may  be  going  on  and  to  retain  the  secretion.  Occasionally,  a  growth 
of  this  kind  may  be  separated  from  its  base  and  give  rise  to  serious  and 
even  fatal  hemorrhage. 


TUMORS  671 

These  polyps  are  composed  of  a  central  core  of  varying  nature,  covered 
by  ciliated  epithelium,  stratified  columnar  or  squamous  cells.  Many 
of  them  are  of  the  nature  of  inflammatory  hyperplastic  new-formations, 
while  others  are  more  truly  tumors.  Among  the  various  forms  may 
be  mentioned  fibromas,  angiofibromas,  mucous  and  adenomatous  polyps, 
angiomas,  and  myxomas,  which  are  rare. 

Mucous  polyps  are  soft,  vascular  growths,  usually  lobulated,  resemb- 
ling the  mucous  membrane  in  structure,  but  more  cellular  and  containing 
gland-tubules. 

Angiomas  form  red,  slightly  vascular  growths,  which  may  pulsate  in 
accord  with  the  radial  pulse. 

Myxomas  are  supposed  to  be  derived  from  the  remains  of  the  mucoid 
substance  which  normally  fills  the  cavity  of  the  ear  in  the  fcetal  state. 

Cholesteatoma  has  already  been  referred  to  (p.  663). 

Sarcoma,  osteosarcoma,  and  carcinoma  have  been  described,  but  are  rare. 
Most  frequently  they  are  secondary  to  malignant  disease  elsewhere. 
Malignant  growths  from  the  brain  or  dura  may  invade  the  ear.  They 
may,  however,  be  primary,  and  when  this  is  the  case,  they  can  be  traced 
to  a  chronic  or  neglected  suppurative  process  in  the  tympanic  cavity, 
or  to  necroses  in  the  temporal  bone.  Large  portions  of  the  temporal 
bone  may  be  destroyed.  Meningeal  and  brain  symptoms  may  result, 
or  hemorrhage.  In  advanced  cases  there  may  be  paralysis  of  the  facial, 
abducens,  and  the  first  division  of  the  fifth  nerve. 


The  Internal  Ear. 
CIRCULATORY  DISTURBANCES. 

Anemia. — Anemia  of  the  inner  ear  may  be  present  in  cases  of 
general  anemia,  and  in  all  conditions  which  lead  to  an  imperfect  supply 
of  blood  to  the  parts,  such  as  endarteritis  or  embolism  of  the  arteria  audi- 
tiva  interna,  tumors  pressing  on  it,  or  aneurisms  of  the  basilar  artery. 

Hyperemia. — Active  hyperemia  is  found  in  cases  of  early  inflamma- 
tion of  the  parts  and  inflammation  of  the  associated  cavities. 

Passive  hyperemia  arises  from  generalized  blood  stasis  in  the  head,  as 
from  heart  and  lung  diseases,  or  struma.  Local  causes,  such  as  tumor 
of  the  base  of  the  brain  or  skull  and  sinus  thrombosis,  may  also  lead  to 
the  condition,  owing  to  interference  with  the  free  outflow  of  blood. 

Hemorrhages. — Hemorrhages,  either  punctiform  or  extensive,  readily 
follow  upon  hyperemia  and  inflammation.  Large  doses  of  quinine  and 
salicylic  acid  lead  to  hyperemia  and  hemorrhages  in  experimental  animals 
(Grunert;  Kirchner1). 

These  minute  hemorrhages  may  be  confined  to  certain  parts  of  the 
cochlea  or  vestibule,  or  may  be  more  extensive.  They  are  seen  particu- 
larly in  severe  otitis  media  and  many  of  the  infective  fevers,  such  as  typhoid, 

1  Berlin,  klin.  Woch.,  1881:  49;  725;  u.  Monatssch.  f.  Ohrenheilk.,  1883:  5. 


672  THE  INTERNAL  EAR 

variola,  septicemia,  acute  tuberculosis,  and  mumps.  They  may  also  be 
caused  by  intracranial  affections,  such  as  meningitis  and  hemorrhagic 
pachymeningitis,  and  occur  in  severe  blood  dyscrasias,  such  as  per- 
nicious anemia  and  leukemia.  The  more  copious  extravasations  are 
due  to  trauma,  as,  for  example,  concussion  and  fractures  of  the  skull. 
Such  extravasations  may  become  completely  absorbed  in  time,  or 
become  organized  or  infiltrated  with  lime  salts.  The  resulting  structure 
is  usually  colored  by  altered  blood  pigments.  Even  moderate  hemor- 
rhages may  lead  to  deafness,  notwithstanding  that  in  time  they  become 
more  or  less  absorbed.  This  is  due  to  the  fact  that  inflammation 
frequently  supervenes,  leading  to  atrophy  and  degeneration  of  the  con- 
nective tissue  and  nerve  filaments.  If  infection  occur,  suppurative 
inflammation  may  set  in  and  be  communicated  to  the  cranial  cavity. 

Inflammation. — Inflammation  of  the  labyrinth  is  rarely  primary, 
being  much  more  commonly  derived  by  extension  from  the  middle  ear 
or  the  cranial  cavity.  From  the  middle  ear  the  infective  agents  invade 
the  parts  by  means  of  the  bloodvessels  in  the  wall  of  the  labyrinth, 
through  fistulous  openings,  or  through  the  fenestrum.  In  the  earlier 
stages  we  see  congestion  and  oedema  of  the  labyrinth  with  round-celled 
infiltration.  Later  on,  in  the  more  severe  infections,  the  cavity  is  filled 
with  pus  and  the  membranous  structures  are  destroyed.  In  time  the  pus 
may  become  inspissated  and  the  process  be  limited  through  the  forma- 
tion of  adhesions  in  the  porus  acusticus  internus;  or  the  pus  may 
burrow  its  way  along  the  sheath  of  the  acoustic  nerve  to  the  brain 
cavity. 

In  cases  originating  in  the  cranial  cavity  the  inflammatory  process 
extends  along  the  sheath  of  the  auditory  nerve,  or  through  the  aqueduct 
of  the  cochlea,  or  both.  Epidemic  cerebrospinal  meningitis  plays  an 
important  role  in  this  form. 

•  Here,  in  the  earlier  stages  there  is  hyperemia  and  hemorrhage  into  the 
labyrinth  with  more  or  less  necrosis  of  the  membranous  structures. 
Later  on,  the  softer  parts  are  completely  destroyed;  the  periosteum 
is  stripped  from  the  underlying  bone,  and  the  cavity  is  filled  with  pus 
and  granulation  tissue.  Should  the  patient  survive,  this,  in  time,  be- 
comes organized  into  connective  tissue  or  even,  in  parts,  into  bone. 
The  intensity  of  the  inflammatory  process  is  most  marked  in  the  vestibule 
and  semicircular  canals  and  becomes  less  extreme  toward  the  cochlea. 
As  a  result  of  the  process  we  get  in  addition,  atrophy  and  degeneration 
of  the  membranous  structures  and  of  the  nerve-fibers,  cells,  and  ganglia, 
while  the  cavity  is  filled  with  altered  pus,  detritus,  cholesterin,  and  pig- 
ment. Thus,  the  whole  auditory  apparatus  may  become  disorganized. 

The  productive  or  hyperplastic  type  of  labyrinthitis  is  frequently 
associated  with  syphilis. 

In  Meniere's  disease,  an  affection  in  which  there  is  deafness,  and 
severe  vertigo,  there  is  a  hemorrhagic  exudation  into  the  semicircular 
canals  and  vestibule,  but  it  is  not  quite  clear  whether  here  we  have  to 
do  with  simple  blood  extravasation  or  with  inflammation. 


TUMORS  673 

Deaf-mutism  is,  by  some,  attributed  to  an  internal  otitis  in  early  life, 
originating  in  meningitis  which  has  extended  along  the  aqueduct. 
Others,  again,  associate  it  with  defects  of  the  auricle,  atresia  of  the 
nu'utus,  immobility  of  the  tympanic  fenestra,  and  acquired  lesions  of 
the  acoustic  nerve. 

The  Acoustic  Nerve. 

Atrophy. — Atrophy  of  the  nerve  is  frequently  the  result  of  pressure 
exerted  upon  it,  as  by  internal  hydrocephalus,  tumors  of  the  brain  or  base 
of  the  skull,  fractures  of  the  petrous  bone,  or  hyperostosis  about  the 
porus  acusticus  internus.  It  may  also  be  caused  by  hemorrhage 
and  inflammation  (neuritis).  Cerebral  causes,  such  as  apoplexy  and 
encephalitis,  involving  the  nucleus  of  the  nerve,  may  also  be  at  work. 
According  to  Erb,  atrophy  occurs  also  in  tabes  dorsalis.  Atrophy  from 
inhibition  of  function  (atrophy  of  inactivity)  seems  to  be  rather  rare. 

Tumors. — Primary  tumors  of  the  acoustic  nerve  are  excessively  rare, 
and  our  knowledge  of  them  is  strictly  limited.  According  to  Virchow, 
however,  this  nerve  is  more  frequently  the  site  of  tumor  growth  than 
any  other  cranial  nerve. 

Fibroma,  myxoma,  psammoma,  and  sarcoma  have  been  recorded. 

As  a  rule,  the  malignant  growths  originate  in  some  of  the  neighboring 
parts,  usually  the  brain  or  dura.  Pollitzer  reports  a  case  of  secondary 
carcinoma  originating  in  the  mastoid  region.  Burkhardt-Merian1  reports 
a  fibro sarcoma  originating  in  the  inferior  petrosal  sinus,  and  Moos,2  a 
spindle-celled  sarcoma  connected  with  the  cerebellum  which  had  invaded 
the  acoustic  nerve. 

1  Arch.  f.  Ohrenheilk.,  12.  2  Ibid ^  4 


43 


SECTION   V. 
THE  DUCTLESS  GLANDS. 


CHAPTER    XXXI. 

THE  FUNCTIONS  OF  THE  DUCTLESS  GLANDS  AND  THEIR 
DISTURBANCES. 

THE  DUCTLESS  GLANDS. 

IN  our  first  volume  we  have  discussed,  to  some  extent,  the  subject  of 
the  internal  secretions,  and  the  influence  exerted  by  increase  or  diminu- 
tion of  the  same  upon  the  organism.  It  is  not  necessary,  therefore,  to 
bring  forward  at  this  point  the  evidence  that  has  been  accumulated 
demonstrating  the  existence  of  internal  secretions.  Such  secretions,  it 
may  be  recalled,  are  afforded  both  by  typical  glandular  organs  pro- 
vided with  ducts,  such  as  the  liver  and  pancreas  (although  in  connection 
with  the  latter,  there  are  those  who  hold  that  the  specific  internal  secre- 
tion is  derived  from  cell-masses  unprovided  with  ducts — the  islands  of 
Langerhans),  and  again  by  atypical  and  ductless  glandular  organs — by 
organs  which,  having  no  ducts,  must,  if  they  be  functionally  active, 
abstract  certain  substances  from  the  circulating  blood  and  lymph,  and, 
as  the  result  of  their  metabolism,  must  discharge  the  products  of  their 
activity  into  the  same.  The  organs  usually  classified  under  the  heading 
of  ductless  glands  may,  from  histological  considerations,  be  classed  under 
three  headings:  (1)  Those  possessing  alveoli  or  cell-masses  of  the 
accepted  glandular  type,  namely,  collections  of  cells  of  fair  size  and  rela- 
tively abundant  cytoplasm,  of  a  cubical  or  polyhedral  form,  recalling 
those  of  the  salivary  glands,  liver,  etc.;  (2)  those  possessing  chromaffin 
cells  and  other  elements  which  we  now  regard  as  derived  from  the 
nervous  system;  and  (3)  those  composed  in  the  main  of  lymphoid  ele- 
ments. 

Such  a  classification  is  unsatisfactory  because  not  a  few  of  these 
ductless  glands  contain  elements  of  more  than  one  of  these  categories. 
The  cortex  of  the  adrenal  belongs  to  the  first  type,  the  medulla  to  the 
second;  the  pituitary  body  likewise  contains  elements  belonging  to  both 
of  the  two  first  categories;  the  thymus,  while  mainly  formed  of  the  third, 
contains  in  its  HassaU's  corpuscles,  elements,  rudimentary,  it  is  true,  of 
epithelial  origin.  It  is,  however,  difficult  to  suggest  any  other  classifica- 


676  THE  THYROID  AND  PARATHYROIDS 

tion,  or  perhaps  it  is  more  accurate  to  say  that  we  here  group  together 
organs  so  widely  different  histologically  that  they  defy  classification, 
save  as  a  group  possessing  the  one  common  feature  of  being  ductless. 
At  most,  Gaskell,  in  his  remarkable  and  strikingly  suggestive  work  on 
the  Origin  of  the  Vertebrates,1  traces  the  whole  group  of  these  ductless 
glands — pituitary,  tonsils,  thyroid,  thymus,  lymphatic  glands,  and  adrenals 
— to  a  common  origin,  as  one  and  all  modified  representatives  of  what 
were  primarily  duct-bearing,  "coxal"  glands,  or  excretory  organs, 
situated  at  the  bases  of  the  segmental  appendages  of  the  protostracan 
forms,  which,  he  holds,  gave  origin  to  the  vertebrates  as  well  as  to  the 
crustaceans  and  arachnids.  While  we  gladly  accept  the  results  of  his 
long-continued  studies,  as  throwing  light  upon  the  peculiar  structure  and 
components  of  the  pituitary  body,  adrenals,  and  thyroid,  we  confess  to 
a  difficulty  in  regarding  the  thymus  and  lymph-glands  in  general  as 
metamorphosed  "lepidic"  structures,  and  that  because  lymphoid 
tissue  is  so  widely  and  irregularly  distributed  throughout  the  organism 
that  it  seems  impossible  to  regard  it  as  the  representative  of  an  original 
chain  of  small,  paired  organs  situated  in  each  segment  of  the  body.  In 
fact,  the  tendency  nowadays  is  to  deny  to  the  spleen  and  lymph-follicles 
the  right  to  be  termed  glands.  Their  cells  are  neither  of  the  accepted 
"  glandular"  form,  nor  do  they  show  any  trace  of  the  characteristic  gland- 
ular arrangement  into  clusters  of  the  lepidic  type.  Conformably  with 
this  tendency,  we  have  treated  them  apart  and  in  connection  with  the 
vascular  system.  Yet  another  organ,  this  time  of  the  true  ductless  gland 
type,  namely,  the  corpus  luteum,  we  discuss  elsewhere;  it  is  both  more 
reasonable  and  more  natural  to  deal  with  this  in  connection  with  the 
genital  system. 

Let  us,  then,  as  briefly  as  possible,  sum  up  what  we  know  regarding  the 
functions  of  the  remaining  "ductless  glands,"  and  of  the  effects  upon  the 
organism  of  disturbances  of  these  functions. 


THE  THYROID  AND  PARATHYROIDS. 

Embryology. — We  have  here  full  evidence  that  the  thyroid  originates 
as  a  median  and  two  lateral  (ducted)  depressions  from  the  floor  of  the 
embryonic  mouth,  and  in  the  frequent  persistence  of  a  processus  pyrami- 
dalis  passing  upward  from  the  gland  toward  the  hyoid  bone,  and  the 
rarer  appearance  of  a  persistent  thyrolingual  canal  or  fistula  leading 
down  from  the  foramen  cecum  of  the  tongue,  as  again  of  thyroid  cysts 
in  the  substance  of  the  tongue  and  median  line  of  the  neck,  see  remnants 
of  this  order  of  development. 

Function. — The  vesicles  are  lined  by  an  epithelium  of  cubical  gland- 
ular type,  and  are  surrounded  by  an  abundant  network  both  of  blood 
capillaries  and  lymph-vessels.  The  cells  discharge  their  secretion  into 
the  vesicle,  and  this  tends  there  to  become  inspissated  (colloid).  But 

London,  Longmans,  Green  &  Co.,  1908:  418. 


THYROID  INCOMPETENCE  677 

apparently  there  is  also  external  discharge,  for  although  its  significance 
here  is  disputed,  colloid  matter  has  been  detected  in  the  surrounding 
capillaries  and  lymphatics.  There  is  evidence  that  this  colloid  material 
contains  the  specific  secretion  of  the  gland.  From  the  organ,  Baumann 
and  others  have  isolated  a  globulin  having  iodine  in  direct  combination 
iodothyrin).  This  body  is  by  most  held  to  be  the  active  principle  of  the 
uland.  It  is,  however,  to  be  noted  that  the  amount  of  iodine  present 
in  the  organ  shows  wide  variations  and  that  the  activity  of  the  gland 
extract  has  not  been  clearly  proved  to  be  related  to  the  amount  of  iodine 
present,  although  this  view  has  been  strongly  supported  by  Oswald  and 
Reid  Hunt.1  It  is  quite  likely  that  this  is  not  the  only  active  principle; 
in  treatment  iodothyrin  has  been  found  less  effective  than  the  dried  gland. 
The  simplest  hypothesis  to  account  for  this  peculiar  histological  arrange- 
ment is  that  the  thyroid  cells  are  capable  of  a  reversible  action,  and  so 
normally  regulate  the  amount  of  active  principle  present  in  the  circulation; 
that  when  the  active  principle  of  the  gland  or  its  precursors  are  present 
in  the  blood  in  excess,  these  are  taken  up  by  the  thyroid  cells  and  stored 
in  the  vesicles  in  a  converted,  less  soluble  state;  when,  on  the  contrary, 
these  have  become  used  up  in  the  blood,  and  there  is  defect,  now  the 
cells  absorb  the  active  principle  from  the  vesicles,  and  discharge  it  into 
the  blood.  On  the  one  hand,  we  may  find  the  contents  of  the  vesicles 
small  in  amount  and  distinctly  fluid,  with  extensive  congestion  of  the 
organ,  suggesting  that  the  specific  secretion  is  being  discharged  into  the 
blood  rather  than  into  the  vesicles — as  in  ordinary  exophthalmic  goitre, 
or  the  regenerative  hyperplasia  that  follows  removal  of  part  of  the  gland ; 
on  the  other,  as  in  colloid  goitre,  the  vesicles  may  be  found  hugely 
distended,  with  dense,  solid  colloid,  suggesting  that  the  cells  are  dis- 
charging into  the  vesicles  with  little  reverse  passage  into  the  blood.  It  is 
interesting  to  note  that  in  advanced  stages  of  this  condition  we  are 
apt  to  get  symptoms  of  myxcedema — of  absence  of  thyroid  secretion 
(  ?  through  compression  atrophy  of  the  vascular  epithelium),  and  contrari- 
wise, when  by  operative  handling  of  such  an  enlarged  thyroid,  a  con- 
gestion of  the  gland  is  induced,  symptoms  of  exophthalmic  goitre  are 
apt  to  show  themselves.  It  is  eminently  probable  that,  as  indicated  by 
Lucke,  the  activity  of  the  secretion  is  controlled  by  the  nervous  system. 

Effects  of  Ablation  and  of  Atrophy. — These  are  well  known  and 
have  already  been  discussed.  In  the  former  case  a  condition  of  cachexia 
strumipriva  may  supervene  (Reverdin,  Kocher)  identical  in  character 
with  the  myxoedema  which  Ord  had  noted  as  associated  with  atrophy 
of  the  gland  and  with  Gull's  cretinoid  cachexia,  Gull  noting  the  resem- 
blance between  the  symptoms  of  the  atrophy  of  the  thyroid  in  adult  life, 
and  those  of  cretinism  in  the  young,  associated  with  congenital  lack  of 
function  of  the  gland.  We  shall  revert  to  the  effects  of  ablation  when 
discussing  the  function  of  the  parathyroids. 

Thyroid  Incompetence. —  Congenital  atrophy  of  the  gland,  or  other 
disease  associated  with  arrest  of  function,  shows  itself,  as  in  cretins, 

1  Bulletin  47,  Hygienic  Laboratory,  Washington,  1908. 


678 

more  especially  by  delaved  growth  of  the  bones  and  (issues  in  general, 
b\  HOD  developinen!  of  the  geiiilalia  al  puberty,  sterility  in  adult  life,  and 
by  arrested  mental  development.  Occurring  in  later  life  the  ineoin 

pelenee    is  a  ee«  n  1 1  pa  n  led    b\    depressed    met  a  bo|  i  a  n  ,   I  ica  I    pio<  1 1  ie|  joi  i ,  a  I  id 

•M  icon  .  interchange,  slowed  mentality,  and  h  illessness.     The  hair  lends 
to  drop  out,  the  skin  is  thickened  as  though  infiltrated  by  a  linn  <  edema 
KM'nr.l.H  ol'  A(liniiiiiil.ral,ion  <i»  '1  liyroid  Kxl.rurl.       Melaholi  an  is  markedly 

accelerated  by  giving;  raw  th\  roid  of  the  domestic  ai al  .  by  the  month, 

or  administering  e.xlracl  of  Ihe  gland.    The  heart  rate  is  increased,  there 

i  ;i  tendency  to  nervoii:  exriteinent  with  mii.eiilar  tremors.  The  I  a  I 
of  ihe  organism  become  used  up,  ami  eventually  there  is  evidence  of 
increased  breaking  down  of  the  proteins.1  This  is  m  sinking;  eonh-a  i 
to  the  lowered  metabolism  and  nervous  depression  of  the  myx<rdeinatoua 
slate.  That  tin-  lowered  melaboh  .m  i.  due  to  the  lack  ol  thyroid  dis- 
charge is  demonstrated  b\  the  disappearance  of  the  mvxu'dematous  and 
eiehnoid  condition,  \\hrn  thyroid  extract  r;  administered. 

The  Parathyroids,- The  <p  lest  ion   of  greatest    present    interest    in   this 
<  ounce  lion    is   the   relation  ship  of   the   parathyroids   to   the   thyroid    and   to 

morbid  .1  He  of  ihe  organism.  And  here  there  is  wide  diversity  of 
opinion  There  is,  on  the  one  hand,  evidence  that  in  a  certain  number 

of  i  .1       ,,  both  i in  and  in  ihe  animals  of  the  laboralorv.  if  ihe  thyroid 

be  removed  and  the  pa  rat  h\  roid  s  be  left,  few  ill  results  ensue,  \\hcrea 
if  both  th\roid  and  p  n  a  t  h  \  roids  be  removed,  s\  mplonis  of  lelaiiy 
are  liable  l.»  supervene,  either  rapidl\  or  within  a  few  days,  with  fatal 
result.  This  tclanv  ma\  be  induced  if  (he  parathyroids  alone  be  removed. 
There  is,  ihns.  a  tendency  lo  regard  the  parathyroids  as  all  important, 
the  ihyroid  as  of  subsidiary  importance  al  least  m  regard  lo  (he  pie 
\cnlion  of  lelaii\  and  rapidl\  fatal  ell'ecls.  Hut,  on  the  other  hand,  il 
is  (o  be  noted  thai  nol  all  of  i;<  \.idm  and  Koeher's  cases,  in  which  ihe\ 
extirpated  the  whole  of  the  thyroids  and  the  attached  parathyroids, 
\\i-ie  followed  b\  lelany  and  death  wiihin  a  short  period.  ( )m  « •olleagne. 
IM  Shepherd,  who  has  a  large  experience  in  the  operative  treatment 
of  various  form,  of  goitre,  assures  n.  that  he  has  repeatedly  removed 
the  whole  organ  \\ilhoul  leaving  the  parathyroids,  and  never  once  has 
he  seen  telany  supervene.  Il  has  been  urged  ihat  in  such  CM  les  acces- 
sory detached  parathyroids  have  been  present  and  have  been  left  behind, 
\o\\,  it  is  true,  as  we  have  observed  from  (he  studies  of  Dr.  I'Ycedman 
m  our  laboratory  at  (he  Koval  Victoria  Hospital,  (hat  there  is  a  \<r\ 
\\idc  \arialion  bolh  in  the  number  of  para  thyroids  m  normal  relation 
hip  to  the  larger  gland,  and  in  these  accessory  parathyroids,  Swale 
Vincent.'  \\ho  records  a  remarkable  lack  of  aficr  elVcels  from  removal 
of  the  thyroids  and  paralhvroids  in  monkc\  .  (contrary  to  llorslcy, 
Murray  and  Kdmnnds,  he  never  obtained  m\  xicdema  in  these  animal  .1. 
states,  ho\\.-\er.  that  he  made  a  more  careful  search  for  acces-orv 
parathyroids  in  his  animals,  and  never  encountered  them.  We  have. 

1    Sell.  .HI, I.  .I'll.       I'llllV..'!1    .        \l,'l.    .      I.,  I  V>,  '-'I    - 

.•[.•nc,-   I'IO.M,-        ;    .IMIIUMI  \  . 


OLOGH   i  /    CONSIDER  \  /'/O.VN  07<) 

in      ln.ll,   \\illi    the    |>a  ra  I  li\  I  on  I    .   mudi    (In       .nnr    pmlilrin    Mi.H   ii.nln.nl 

n      in  r <  lli.n    \\  Illi    (lie    I    l.i  ml      nf    I  , ;ill^erlia  I  IS   in   I  lie   paliclca  s.        I  ii  ill  i 

h;i\c   ;ili    clllli  I  \onic,    linpi  lie,   ||\    ( |r  \  r|i,|  .r,  I  ,   nl     l.lldl!    ;i  ppe:i  I  a  I  K  c          \\  illi 

I  M  ill  i  tht*rt»  is  evidence  pointing  to  the  a    iiin|iiion  under  certain  condit 

of  .-in  adult-developed  I.M'  lln  human  parathyroids  in '|m  nil-  in 
l»l.i«i  nf  MM  "imiji  of  cell.  (resembling  Mi"  <  en  tiered  through  llir 
.iMnll  thyroid  hetAVeen  llir  vesicles)  lli.il  are  lln  i  i  linn  n  in  l«.ilnn  nf  lln 
bodies,  exhibit  \\ell  formed  vesicles  tilled  \\ilh  colloid;  and  in  aiinn;il 

IV \\liich    (he   lliNioid    ha,   Keen    removed,    the   parathyroid     ;i     mm 

llnroid    charadci  i shcs.      This,    however,    i      denied     by    the    nplioldi  i 

of   the      [MI   ilicil\    of   film  lion   of    MIC   c    In. die:,    u  In.   ;d    n   ic-'ard     lln     (  nlli.nl 

li      encountered   in  rerlain  apparent!)    normal   panillivroid     :i     dr 
tinguishcMl  from  those  of  the  thyroid  propei       Save  for  (he     mall     i.<    ..I 

lln     former,   \\  r    personally    have    Keen    Illialilc    lo    recoyin/e   an\     III    lnl<>"l<  ;d 

difference  Ketween  the  I  \\  n.1 

Without  denying  specific  I  nnd  mn  In  I  In    |  »a  i  .1  Mi  •  i  nu  I    ,  "  •    an    i  n<  lined 

10  demand    clearer    and     more    rnlaiii    evidence    Kelol'e    accepting    that 
Mn  •     arc    aK  <>lnlrl\    distinctive    nr."an.       Amid    lln     m.in       contradictor 
1. 1.  million    ,    we   are,    however,    nn|.i«      <<l    l.\    the    fail    lli:il    lln    <•    I  .«.•  I  n 

MI    r    IV an   <nil(l(/{'  (the    rpillirlnim   nf    llir    Mind   and     I'mnlli    Kralirln.M 

<  I.  ll     i   closel^    allied    fo,    Kill     n.   M   illn   !•          Ml    Inn  I    linm    lln     Intend    M'     K.ld 

11  ii  !<!(/<•  ii ,  a.  ,  a  pi  ill,  llial  I  lie     \  ni|»lom     of  Ida  n  \  ,  a      ,lio\\  n  K  v    \lac<  'a  limn  , 
i  .Mi    I  ir    ;i  mi   lim  ;i  li  1 1    K\     III  (Cctill^  Cllllll    imi      nl     lln      1 1.1  i  ;i  I  li  \  I  ( in  I 

The     inn    !     Irrrnl     U'orl    .    ll     m.i,     In      iim.ill'd,     III     rot  n  1 1 1    lol  I  f  stj'OH^Iy 

jioini  .    lo    llir   importance  of   llir    parathyroids    in    re^ulatin^   <alrium 

liielalioll     in 

THE  ADRENALS, 

Embryological  Considerations,     Tin-  emKryo^Miy  of  the  ndrenal 

i      iml    a    hhl.     i.  niaikaKle;   lln    cortex    and    (lie   medulla    have    u  holly  ilif- 

fcnnl    nii"in    ,   and,    in    fad,    in   ccrlain    ln\\i  r    \  d  id  >i  ;i  l<       '  •  l.i    inoKl'anch 
(l    In      i    i  nil    llhllr    di    IIIK   I    nl":ili  Tin          i|""i     I       dr  i   I     ll\     of    fnn<  linn 

e\en     if    (lie    evenllial     fir  inn        ll""(     I        ;d    n     Mia!     flic     I  U  n    :ii<      i  1 1 !  1 1 1 1.1  I  •  I 

dependent    the  mn    mi   iln    other,     'I  In    <  mh      111  Mill<  M  nl   animals  is, 

in    lad,  derived    Irom    the    mesoliephric   or  prolieplnn     excreton    oi".in 
I  lie   medulla    IM      .1      i  |  ..M  .1  li     m  i"i  n    from    lln        •  n  i|  o  ll  n  I  i<-    111  i  •  mi         •     hin 
While   (In    corl<        i      Iniinrd   nf   rnltlinii     of  rr||     of    .'d;iinlnl.M    I     |  n      m    inn 

male  a  ocialion  with  a  tern  of  capillarii  o  inliinal<'  thai  in  j)lar< 
accordilifj  I"  nnn-  aiiflinnln  ,  lln  'ill  adnalK  aKnl  nnmi  llir  Klood 
In  .MII  \'.  illi  no  ml'  i  ..inn"  i  ndnlln  linm  I  he  nn  dull.i  i  <  |I.M;M  l<  i  i/<  d  Ky 
an  al.rii'i  nf  ;_dand-cc||  |noper  and  pi<  <n<<  of  certaJII  i  •  ma  i  l.aKIc 
di  nuiKi  Iji //  cells.  Such  chroma  din  cell  ;m  •,•.  nli  I  di  hiKnIed  through- 
out the  Kodv.  They  owe  their  name  lo  (heir  allinit'  Im  neutral  .Mi  «l 
rlnmnir  arid,  a  t  iimin;'  with  the  .  ;,  IM.II^  yellow  m  Kmwnisli  color. 

1     \  n  :nllinr:ili|r   |,n     ctllnl  jiMI  of  I  In     <  :>    <     IMI    I  IK     «li    I  I  lirl  H  Ml   I  H'l  U  cell    I  In     I  \\  <  i  i 
di    \t\     I  iMrl      in    <  •    |.  i         MM.IIIM    \|I.|ICIIK       ,.      IIKM>:.'{S2. 


680  THE  ADRENALS 

They  are'derived  from  the  nervous  system,  and  from  one  portion  of  this, 
the  sympathetic  system.  They  are  to  be  found  in  the  sympathetic  gang- 
lia, in  the  pituitary,  the  carotid  glands,  and  the  organ  of  Zuckerkandl, 
situated  at  the  origin  of  the  superior  mesenteric  artery,  and,  according 
to  some  authorities,  in  the  coccygeal  gland,  but  are  present  in  greatest 
abundance  in  the  adrenal  medulla.1 

Function.  —  The  Medulla. — The  observations  of  recent  years  have 
demonstrated  that,  judged  from  the  effects  of  their  extract,  these  cells, 
wherever  present,  have  the  same  properties.  That  extract  has  a  powerful 
action  upon  the  arteries  and  arterioles,  leading  to  contraction  of  the  same, 
and  temporary  pronounced  rise  of  the  blood  pressure.  These  properties, 
as  first  noted  by  Oliver  and  Schafer,  are  particularly  marked  in  connec- 
tion with  extracts  of  the  adrenal  medulla.  The  active  principle  having 
these  effects  has  been  isolated  in  a  crystalline  form  by  Takamine  and 
Aldrich  independently,  and  by  Abel.  It  is  generally  known  as  adrenalin, 
the  name  given  to  it  by  Takamine,  but  as  his  substance  has  become 
proprietary,  Schafer  proposes  adrenin  as  a  more  ethical  name.  It  has 
an  action  upon  skeletal  and  cardiac  muscle  as  well  as  upon  the  plain 
muscles  of  arteries.  Langley2  has  enunciated  the  view  that  in  all  cells 
a  chief  substance  is  present,  to  which  is  owing  the  chief  function  of  the 
cell,  and  a  more  unstable  intermediate  or  receptive  body,  which  sets 
the  chief  substance  in  action  when  itself  acted  upon  by  nervous  and 
other  stimuli.  The  action  of  adrenin  appears  to  be  identical  with  that 
of  the  sympathetic  nerves,  and  Langley  suggests  that  adrenin,  taken  up 
by  the  cells,  has  the  same  effect  on  the  receptive  substance  as  have  stimuli 
reaching  it  through  the  sympathetic  nerve  endings.  Stoltz  and  others 
have  prepared  a  synthetic  adrenin  having  the  same  composition  and 
effects  as  adrenin,  but  optically  inactive,  instead  of  being  levorotatory, 
and,  according  to  Gushing,  not  so  powerful  as  the  natural  substance. 
Adrenin  is  soluble  in  water,  and  dialyzable,  unaffected  by  boiling,  but 
insoluble  in  alcohol,  and  non-acted  upon  by  the  gastric  juice  and  by 
acids. 

The  Cortex. — Very  little  definite  has  been  determined  regarding  the 
functions  of  the  cortex,  though  there  have  been  not  a  few  theories. 
Rolleston3  brings  forward  some  evidence  in  favor  of  a  relationship 
between  the  cortex  and  the  sexual  powers.  The  abundance  of  lecithin 
and  bodies  of  the  nature  of  myelins  is  suggestive  in  connection  with 
recent  studies  upon  the  importance  of  these  bodies  in  relationship  to 
the  blood  serum'  and  the  production  of  immunity. 

The  Relationship  between  Adrenal  Function  and  Disease.— 
Adrenal  Incompetency.- — To  repeat  what  was  stated  in  our  first  volume, 

1  For  a  study  of  the  "  Chromaffin  System,"  see  Gierke,  Lubarsch-Ostertag's  Ergeb. 
der  allg.  PathoL,  Jahrg.  10:  1904  to  1905:  502;  and  Wiesel,  Internal.  Clinics,  2: 1905: 
288. 

2  Jour,  of  Physiol.,  33:  1905  to  1906: 374. 

3  Montreal  Med.  Jour.,  1907 ;  or  Lancet,  London,  2 : 1907 : 875 ;  see  also  Schafer.    For 
the  fullest  collection  of  data  bearing  upon  adrenal  function,  see  Sajous,  The  Internal 
Secretions,  1:  1902,  and  2:  1907. 


RELATIONSHIP  BETWEEN  ADRENAL  FUNCTION  AND  DISEASE     681 

extensive  disease  of  the  adrenals,  or  atrophy  of  the  same,  is  accom- 
panied by  the  symptoms  of  Addison's  disease — great  muscular  weakness, 
with  low  blood-pressure  and  soft  pulse,  anorexia,  with  gastric  discom- 
fort, and  occasional  vomiting,  cerebral  disturbances  of  a  mild  type,  and 
above  all,  pigmentation.  The  vascular  symptoms  and  lack  of  muscular 
tone  are  the  very  opposite  of  the  result  of  injections  of  adrenin,  and 
thus  the  generally  accepted  view  is  that  normally  the  adrenal  medulla 
affords  the  active  principle  to  the  blood  whereby  the  vascular  and 
muscular  tone  of  the  body  is  maintained.  The  French  school,  under 
Abelous  and  Langlois,  uphold  rather  a  theory  of  auto-intoxication, 
finding  the  blood  of  animals  deprived  of  their  capsules  to  possess  curare- 
like  properties,  they  regard  the  gland  as  removing  or  neutralizing  this 
substance.  These  two  theories  are  not,  it  may  be  noted,  absolutely 
opposed ;  it  is  quite  possible  that  adrenin  may  both  neutralize  the  paretic 
action  of  such  a  substance,  and  itself  act  directly  upon  the  muscle. 

What  has  until  now  been  a  difficulty  in  understanding  the  relationship 
between  the  adrenals  and  Addison's  disease,  is  the  existence  of  a  certain 
small  number  of  cases  in  which  either  one  or  both  adrenals  and  their 
medullas  are  apparently  unaffected;  and  of  another  series  of  cases  in 
which  there  might  be  complete  replacement  of  the  adrenals  by  new- 
growth  with  no  signs  of  Addison's  disease.  Many  years  ago  it  was 
suggested  by  Rolleston  and  others  that  in  these  cases  not  the  glands,  but 
the  nearby  semilunar  ganglia  and  solar  plexus,  were  involved.  Very 
numerous  nerves  pass  from  these  to  the  adrenals — and,  indeed,  besides 
chromaffin  cells,  the  medulla  contains  ordinary  sympathetic  nerve  cells. 
Thus  some  have  held  that  nervous  disturbances  might  lead  to  arrest  of 
-adrenal  function.  The  recent  studies  upon  the  chromaffin  cells  have 
afforded  a  possible  explanation.  Here,  Wiesel's  observations  are  of 
distinct  importance.  In  seven  cases  of  the  disease  he  has  examined, 
not  merely  the  adrenals,  but  the  whole  chromaffin  system — adrenals, 
the  chains  of  sympathetic  ganglia  and  plexuses,  Zuckerkandl's  organ, 
etc.,  and  he  has  found  a  general  absence  of  chromaffin  cells,  although 
now,  as  a  compensatory  process,  the  sympathetic  ganglion  cells  may 
give  the  chromaffin  reaction.  If,  as  in  the  cases  of  cancer  above  men- 
tioned, the  adrenals  alone  are  destroyed,  the  chromaffin  cells  elsewhere 
may  be  adequate  to  prevent  disease,  or  there  may  be  extensive  destruc- 
tion of  the  chromaffin  cells  in  other  areas,  leading  to  symptoms  of  the 
disease,  without  extensive  adrenal  disease.  As  a  matter  of  fact,  Beitzke 
has  reported  a  case  of  cancer  involving  the  adrenals,  without  Addison's 
disease,  in  which  he  found  the  chromaffin  cells  intact  elsewhere.  Fur- 
ther confirmation  is  needed  of  these  most  suggestive  observations. 

But  neither  Wiesel's  nor  other  studies  upon  ablation  of  the  medulla 
have  thrown  light  upon  the  specific  pigmentation  seen  in  Addison's 
disease.  In  the  first  volume  (p.  893)  one  of  us  brought  together  the 
evidence  that  cell-pigment  of  the  nature  of  melanin  is  a  product  of  the 

'Zeit.  f.  Heilk.,  24:  1903;  Pathol.  Anat.,  Abt.  No.  4;  and  Internal.  Clinics,  2: 
1905:  loc.  cit. 


682  THE  ADRENALS 

disintegration  of  proteins,  and  is  an  oxidation  product  of  bodies,  like 
tyrosin,  of  the  aromatic  series,  and  Halle's  observation  that  in  the  adrenal 
tyrosin  is  converted  into  adrenin  through  the  action  of  an  enzyme. 
Now,  as  extracts  of  all  the  tissues  containing  chromaffin  cells  have  like 
effects  upon  the  vessels,  we  may  assume  that  adrenin  is  present  in  all, 
and  is  similarly  produced.  Thus,  if  Halle  be  correct,  the  absence  of 
chromafrin  tissue  must  tend  to  be  accompanied  by  an  accumulation 
in  the  tissues  of  members  of  the  aromatic  series,  which,  undergoing 
oxidation,  become  pigmented  bodies  of  the  nature  of  melanin,  instead 
of  undergoing  conversion  into  adrenin.  Along  these  lines  of  chro- 
maffin inadequacy,  with  heaping  up  of  members  of  the  aromatic  series, 
the  pigmentation  gains  a  plausible  explanation. 

There  is  still  confessedly  much  to  be  accomplished  before  we  have 
a  thorough  understanding  of  the  bearing  of  adrenal  changes  upon  the 
whole  series  of  symptoms  of  Addison's  disease.  Yet  the  last  few  years 
appear  to  have  carried  us  forward;  the  theory  of  adrenal  or,  more  accu- 
rately, of  chromafrin  cell  inadequacy  would  seem  to  be  establishing  itself. 

Excessive  Adrenal  Function. — We  said,  in  connection  with  the  thyroid, 
that  excessive  production  and  discharge  of  the  thyroid  secretion  led  to 
a  very  definite  syndrome.  Is  a  like  condition  of  excessive  production 
of  the  adrenal  secretion  to  be  determined?  We  observed  there  that 
feeding  or  injecting  thyroid  extract  brought  about  a  series  of  symptoms 
(hyperthyroidism)  resembling  in  many  respects,  though  not  in  all,  the 
symptoms  of  Graves'  disease  (vol.  i,  p.  325).  Is  there  any  morbid  state 
resembling  in  its  symptoms  the  results  of  administering  adrenalin  or 
adrenin  to  man  or  the  lower  animals?  As  we  have  stated,  the  most 
striking  feature  of  such  administration  is  rise  of  blood  pressure.  As 
a  matter  of  fact,  hyperpiesis,  or  pronounced  and  continued  rise  of  blood 
pressure,  is  not  uncommon.  Is  there  any  change  to  be  observed  in  the 
adrenals  in  this  condition?  It  may  be  objected  that  the  administration 
of  adrenin  leads  but  to  a  transient  rise  of  blood  pressure;  but,  on  the 
other  hand,  we  must  take  into  account  that  a  difference  is  to  be  expected 
between  the  effects  of  experimental  inoculation  from  time  to  time,  and 
the  steady  outpouring  of  the  active  principle  from  an  overactive  gland; 
further,  if  experimentally  we  give  repeated  doses,  each  is  followed  by 
its  rise;  there  is  no  accustomance.  It  is  in  connection  with  chronic 
interstitial  nephritis  that  we  most  frequently  encounter  this  condition 
of  hyperpiesis,  and  here  Vacquez  and  Aubertin,  Aschoff  and  Pearce,1 
have  put  on  record  the  observation  that  in  a  series  of  autopsies  upon 
arteriosclerosis,  more  particularly  when  accompanied  by  chronic  inter- 
stitial nephritis  and  hypertrophy  of  the  left  ventricle,  the  adrenals  are 
distinctly  larger  than  normal,  with  distinct  increase  in  the  size  of  the 
medulla.  Dr.  Klotz,  in  our  laboratory,  has  called  our  attention  to 
the  same  noticeable  hypertrophy.  Whether  this  is  primary  or  second- 
ary must,  for  the  present,  be  left  an  open  question,  as  also  it  is  not 
determined  whether  both  states  are  to  be  encountered;  a  series  in  which 

1  Jour,  of  Exp.  Med.,  10:  1908:  735  (with  bibliography). 


EMBRYOLOGICAL  CONSIDERATIONS  083 

the  hypertrophy  and  increased  secretion  is  the  primary  disturbance; 
another,  in  which,  either  through  the  agency  of  the  nervous  system,  or 
through  the  absorption  of  foods,  etc.,  acting  by  their  disintegration 
products,  increased  production  of  adrenin  is  brought  about.  The  rarity 
of  high  blood  pressure  and  of  hypertrophy  of  the  medulla  in  early  life  is 
somewhat  against  the  former.  It  has  still  to  be  determined  whether  this 
enlargement  of  the  glands  is  purely  associated  with  the  arteriosclerotic 
or  with  all  types  of  chronic  interstitial  nephritis,  as  also  how  far  it  is 
related  to  arteriosclerotic  states  in  general.  It  will  be  recalled  that 
adrenin  in  itself  leads  to  arteriosclerotic  manifestations,  and  also  that  not 
all  cases  of  this  condition  are  associated  with  high  blood  pressure.  It 
will  thus  be  seen  that  much  has  still  to  be  garnered  before  sure  conclusions 
can  be  laid  down.  We  have  thought  it  worth  while  to  mention  these 
matters  as  an  indication  that  the  study  of  possible  conditions  of  excessive 
adrenal  activity  is  not  being  wholly  neglected. 

THE  PITUITARY  BODY  (HYPOPHYSIS  CEREBRI). 

This,  like  the  adrenal,  is  formed  of  a  combination  of  glandular  and 
nervous  elements,  the  former  approximating  in  type  to  what  is  seen  in 
the  thyroid. 

Embryological  Considerations.— The  infundibulum,  to  whose  apex 
is  attached  the  pituitary,  represents  the  old  channel  of  communication 
between  the  neural  canal  and  the  mouth.  If  Gaskell  be  right — and 
it  is  difficult  to  controvert  the  extraordinary  volume  of  evidence  he 
has  brought  forward  in  favor  of  his  contention — that  neural  canal  repre- 
sents the  original  alimentary  channel  of  the  invertebrate  ancestors  of 
the  vertebrata.  Like  the  thyroid,  embryological  studies  (Bela  Haller) 
show  that  the  glandular  portion  is  originally  of  a  tubular  type;  Gaskell 
holds  that  these  tubules  represent  the  coxal  glands  situated  at  the  bases 
of  the  appendages  (endognaths)  originally  present  around  the  original 
mouth.  In  the  process  of  reduction,  the  nervous  elements  of  the  old 
cesophageal  tube  and  the  surrounding  ring  of  gland-substance  become 
intimately  intermixed.  In  the  cat  the  infundibular  process  retains  its 
central  cavity  and  is  lined  by  ependyma  cells.  The  central  canal  dis- 
appears in  other  higher  vertebrates. 

Thus,  in  the  fully  developed  pituitary  we  recognize  three  areas:  (1) 
An  anterior,  glandular,  containing  granular  cells  in  solid  columns,  with 
relatively  large  intervening  blood  capillaries;  (2)  a  "pars  intermedia" 
containing  clear  cells,  intermingled  with  neuroglia  fibrils.  These  cells 
are  of  epithelial  type,  and  discharge  a  colloid  material,  which,  as  shown 
by  Schafer  and  Herring,  passed  into  the  lymph-spaces  or  channels,  and 
thence  backward  into  the  cavity  of  the  infundibulum,  and  so  into  the 
cerebrospinal  fluid;  (3)  the  posterior  nervous  portion  is  made  up  of 
neuroglia  cells  and  fibers,  without  true  nerve-cells,  though  there  are 
indications  that  sympathetic  fibers  pass  in  along  the  bloodvessels.  There 
may  be  occasional  islets  of  epithelial  cells  which  have  grown  back  into 
this  region. 


684  THE  PITUITARY  BODY 

Functions. — No  effects  have  been  noted  as  following  injections  of 
extracts  of  the  anterior  glandular  portion,  but  the  intermediary  and 
nervous  portions  have  been  discovered  to  contain  a  body  resembling  in 
action  adrenin.  There  are  indications,  indeed,  of  the  presence  of  more 
than  one  active  principle.  Thus,  Schafer  and  Herring1  found  that  aque- 
ous extracts  had  opposite  effects  upon  the  bloodvessels.  In  the  first 
injections,  the  pressor  or  vasoconstrictor  effects  predominate;  in  subse- 
quent injections,  the  depressor  effects  become  manifest.2  So,  also,  the 
same  observers  have  discovered  that  from  the  posterior  portion  a  sub- 
stance can  be  obtained,  soluble  in  water,  and  uninfluenced  by  boiling, 
having  a  specific  effect  upon  the  kidney,  exercising,  indeed,  a  diuretic 
effect  more  powerful  than  that  of  any  known  substance. 

Relationship  of  Morbid  Disturbance  to  Disease. — It  is,  however, 
difficult  to  associate  these  experimental  results  with  the  data  of  disease; 
in  fact,  we  are  but  at  the  beginning  of  a  knowledge  of  the  function  of  the 
organ.  One  outstanding  fact  there  is,  that  the  remarkable  condition 
of  acromegaly  is  intimately  associated  with  overgrowth,  either  simple  or 
adenomatous,  affecting  more  particularly  the  glandular  portion  of  the 
organ. 

Briefly,  the  morbid  changes  in  this  rare  disease  are  that  it  shows  itself 
in  the  second,  or  more  often  in  the  third,  decade,  less  often  in  the  fourth, 
as  a  progressive  enlargement  of  the  bony  skeleton.  All  the  bones  are 
affected,  but  most  strikingly  those  of  the  extremities  and  cranium,  and 
more  especially  the  lower  jaw,  which  becomes  enlarged  in  all  directions, 
while  the  sella  turcica,  in  which  the  enlarged  pituitary  is  lodged,  under- 
goes atrophy  of  its  bony  walls  and  great  increase  in  its  cavity.  Along 
with  this  there  is  a  thickening  of  the  subcutaneous  tissues,  which,  in  a 
case  of  pituitary  tumor  described  by  one  of  us,  was  the  most  marked 
feature,  the  condition  approximating  more  to  myxoedema  than  to  acro- 
megaly. The  liver  and  spleen  are  often  noticeably  enlarged.  The 
testes,  ovaries,  and  uterus  often  exhibit  atrophy  or  hypoplasia,  although 
the  external  genitalia  may  be  hypertrophied. 

The  pituitary  in  the  majority  of  cases  is  greatly  enlarged,  either  from 
a  process  of  simple  hypertrophy,  or  by  neoplasia  of  the  glandular  portion, 
simple  or  malignant  adenoma,  or  even,  according  to  some  authorities,  a 
reversion  to  a  sarcomatous  type  of  growth.  Benda,  in  four  cases,  records 
a  marked  increase  in  chromaffin  cells.  With  these  changes  there  may 
be  nervous  symptoms  (blindness;  paralysis  of  the  oculomotor  muscles; 
deafness,  due,  apparently,  to  pressure  effects  in  the  cranial  cavity;  and 
others  that  cannot  so  surely  be  ascribed  to  pressure,  such  as  depression, 
loss  of  memory,  homicidal  insanity,  etc.).  Glycosuria  is  not  uncommon. 
(This  has  been  observed  in  connection  with  other  tumors  of  the  base  of 
the  brain  and  cerebellum.)  Polyuria,  with  or  without  glycosuria,  may 
be  a  prominent  symptom,  a  fact  which  is  suggestive  in  connection  with 

1  Phil.  Trans.  Roy.  Soc.  Lond.,  1896,  B. 

2  The  studies  of  Professor  and  Miss  Meltzer  point  to  the  existence  of  similar 
depressor  substances  in  thyroid  extract. 


THE  THYMUS  685 

the  existence  above  noted  of  an  active  principle  promoting  diuresis  in 
thr  normal  organ.  We  here  would  seem  to  see  obscurely,  as  in  connec- 
tion with  both  the  thyroid  and  the  adrenal,  some  relationship  between 
the  activity  of  the  epithelial  elements  of  the  organ  and  metabolism,  and 
more  particularly  the  development  of  the  proper  organs  of  sex.  Here, 
again,  as  in  connection  with  the  adrenal,  it  cannot  be  said  that  the 
exhibition  of  the  active  extract  of  the  gland  has  brought  about  any 
uniform  results.  That  occasionally,  both  in  Addison's  disease  and  acro- 
megaly,  it  is  followed  by  marked  amelioration  of  symptoms,  indicates 
as  suggested  by  Sajous,  that  we  have  not  as  yet  conquered  the  right 
mode  of  dosage  and  administration. 


THE  CAROTID  BODIES,  COCCYGEAL  GLAND  AND  ZUCKERKANDL'S 

ORGAN. 

These  may  be  dismissed  briefly.  All  are  organs  of  insignificant  size, 
intimately  associated  with  the  vascular  system  on  the  one  hand,  with  an 
abundant  sympathetic  network  on  the  other,  and  exhibiting  columns  or 
clusters  of  cells  bordering  upon  the  walls  of  an  abundant  capillary 
network.  These  cells  in  the  case  of  the  first  and  third  are  certainly  of 
the  chromaffin  type;  as  regards  the  coccygeal  gland,  the  observations 
are  not  so  clear.  The  carotid  gland  in  man  is  situated  in  the  posterior 
aspect  of  the  bifurcation  of  the  common  carotid  artery;  ZuckerkandPs 
organ  is  closely  attached  to  the  adventitia  of  the  origin  of  the  superior 
mesenteric  artery;  and  the  coccygeal  gland  lies  close  to  the  tip  of  the 
coccyx.  Little  is  to  be  said  in  addition,  save  that  these  bodies  may  be 
the  seat  of  tumors,  growths  of  peritheliomatous  type  (see  vol.  i,  Fig.  272, 
p.  759,  and  Fig.  277,  p.  767). 


THE  THYMUS. 

It  is  so  usual  to  consider  the  thyroid  and  the  thymus  in  succession, 
that,  although  personally  we  regard  the  latter  organ  as  belonging  to  the 
lymphatic  system,  we  have  not  ventured  to  depart  from  custom,  and, 
therefore,  must  here  note  what  is  known  regarding  its  function. 

Embryological  Considerations. — From  their  embryogeny  there  is 
undoubtedly  ground  for  discussing  these  two  glands  together.  Both 
originate  as  segmental  organs,  as  downgrowths  of  tubular  type  from 
the  mouth,  or,  more  accurately,  from  the  branchiae,  from  the  epithelium 
lining  the  original  gill-clefts.  Originating  thus,  the  thymus  undergoes 
a  modification  very  similar  to  that  seen  in  the  faucial  tonsils,  which, 
according  to  Gaskell,  are  of  like  origin,  the  epithelial  cell-nests  (HassalPs 
corpuscles)  being  the  remains  of  the  original  epithelial  downgrowths. 
The  matter  of  the  origin  of  the  vertebrate  leukocytes  is  still  in  dispute. 
That  Beard  was  too  restricted  in  regarding  them  as  derived  primarily 
and  essentially  from  the  thymus  epithelium,  must,  we  think,  be  generally 


686  THE  THYMUS 

accepted.  Gaskell1  gathers  together  much  evidence  showing  that  in 
lower  forms  the  segmental  tubes  (nephridia)  throughout  the  body 
may  become  modified  into  a  lymphoid  and  phagocytic  tissue.  On  the 
other  hand,  the  view  is  very  widely  held  that  lymphoid  tissue  is  essentially 
mesodermal  and  not  hypoblastic  or  epiblastic.  The  matter  must  be 
left  open. 

Certain  it  is  that  the  thymus,  when  fully  developed,  prior  to  the  end  of 
the  second  year,  is  essentially  a  lymph-glandular  organ,  and  that,  so  far, 
no  specific  active  principle  has  been  isolated  from  it,  or  recognized  as 
existing.  Later,  the  cells  undergo  a  characteristic  fatty  change,  and 
in  addition  come  to  contain  hyaline  droplets;  and  with  this  there  is  a  slow 
progressive  atrophy,  until  the  gland  is  represented  merely  by  fatty  tissue 
with  occasional  small  collections  of  lymph-cells. 

It  is  interesting  to  note  that  there  is  an  increasing  tendency  to  ascribe 
the  origin  of  the  not  uncommon  mediastinal  sarcomas,  or  lymphosarcomas, 
to  overgrowth  of  this  lymphoid  tissue  of  the  thymus.  Weigert,  in  addi- 
tion, has  promulgated  the  hypothesis  that  the  condition  of  myasthenia 
gravis  is  intimately  related  to  neoplasia  of  the  thymus.  In  an  autopsy 
upon  a  case  of  this  remarkable  state  of  progressively  increasing  muscular 
weakness,  he  found  present  lymphosarcoma  of  the  thymus,  and,  with 
this,  scattered  accumulations  of  lymphoid  cells  between  and  within  the 
skeletal  muscle  fibers.  These  he  regarded  as  metastases.  Recent 
workers  have  confirmed  the  frequent  presence  of  these  "  lymphorrhages" 
in  the  muscles  and  other  organs  in  myasthenia,  but  only  in  10  out  of 
180  cases  of  the  disease  have  disturbances  of  the  thymus  been  reported. 
Obviously,  therefore,  thymus  neoplasia  has  no  necessary  connection  with 
the  condition,  although  it  may  be  present  along  with  other  changes  in  the 
lymph-glandular  system. 

Thymic  Asthma. — The  one  severe  condition  in  which  we  may  regard 
the  thymus  as  primarily  at  fault  is  thymic  asthma,  a  condition  of  grave, 
rapidly  progressive,  and  fatal  dyspnoea  in  children,  associated  with 
hypertrophy  and  congestion  of  this  organ.  To  this  we  have  already 
referred  (p.  242).  But  here  if  the  gland  be  at  fault — which  some  still 
strenuously  deny — it  is  not  by  any  internal  secretion,  but  by  the  physical 
agency  of  its  enlarged  state  that  symptoms  are  produced.  Wiesel  points 
out  that  in  two  cases  examined  by  him,  in  one  of  which  (an  adult)  the 
thymus  was  the  size  of  an  apple,  there  was  an  accompanying  hypoplasia 
of  the  chromaffin  system  in  the  adrenal  medulla  and  elsewhere,  and 
to  this  rather  than  to  thymic  enlargement  he  is  inclined  to  ascribe  the 
sudden  death. 

1  Loc.  cit.,  p.  425. 


CHAPTER    XXXII. 

THE  THYROID  AND  THYMUS  GLANDS. 
THE  THYROID  GLAND. 

THE  thyroid  gland  is  composed  of  two  lateral  lobes,  situated  one  on 
each  side  of  the  larynx,  and  connected  by  an  isthmus.  The  average 
weight  in  the  adult  varies  between  twenty-five  and  sixty  grams. 

The  organ  is  enveloped  in  a  fibrous  capsule  sending  prolongations 
inward  to  form  the  stroma,  which  contains  numerous  bloodvessels, 
lymphatics,  and  nerves.  The  vascular  anastomoses  are  very  abundant 
and  the  larger  lymphatics  have  valves  like  the  veins.  Embedded  in  this 
stroma  are  numerous  acini  that  vary  somewhat  in  appearance.  Accord- 
ing to  AYolfler,  a  cortical  and  a  medullary  zone  are  to  be  differentiated. 
The  former  contains  solid  bands  and  groups  of  cells;  the  latter  is  made 
up  of  closed  vesicles  lined  by  a  single  layer  of  cubical  or  cylindrical  cells, 
and  filled  with  a  homogeneous  gluey  substance  or  colloid.  The  cells 
may  contain  minute  drops  of  colloid  or  larger  masses  that  force  the 
nucleus  to  one  side. 

The  colloid  substance  seems  to  be  a  secretion  of  the  specific  cells, 
but  in  some  instances  the  cells  seem  to  be  transformed  into  colloid, 
apparently  as  a  process  of  degeneration.  Colloid  occasionally  also  has 
been  observed  in  the  stroma  and  lymphatics.  It  is  by  no  means  certain 
how  the  colloid  enters  the  lymphatic  vessels.  Some  believe  that  the 
follicles  open  directly  into  the  lymphatic  vessels,  while  others  think 
that  there  is  no  direct  connection,  but  that  the  fluid  parts  alone  reach  the 
lymphatics. 

Between  the  follicles  containing  the  colloid  material  may  be  observed 
groups  and  rows  of  epithelial  cells  which  are  considered  by  Wolfler1 
to  be  embryonic  "cell-rests"  which  have  not  developed  into  the  normal 
acini.  They  are  especially  common  in  the  newborn.  Embryonic  cells 
may  also  be  present  in  the  capsule. 

A  word  or  two  should  be  said  about  the  so-called  parathyroids  of 
Sandstrom2  or  "  epithelkorperchen"  of  Kohn.3  According  to  Kohn, 
there  are  four  of  these,  an  outer  epithelial  body,  at  the  side  of  each 
lateral  lobe  and  an  inner  one  on  each  mesial  surface.  Functionally, 
these  bodies  are  to  some  extent  distinct  from  the  thyroid  gland,  since, 
in  cases  of  atrophy,  as,  for  instance,  in  myxoedema,  the  epithelial  bodies 


.  f.  klin.  Chir.,  29:  1885. 

2  Upsala  Lakertoreningens  Forhandlingar,  1880, 

3  Arch.  f.  mikrosk.  Anat.,  44:  1895:  366. 


688  THE  THYROID  GLAND 

remain  intact.  Apparently  they  have  to  do  with  the  control  of  calciun 
metabolism,  as  MacCallum  and  Voegtlin1  have  shown  recently. 

Microscopically,  they  consist  of  a  network  of  broader  or  smaller  row; 
of  cells  or  of  fairly  regular  lobules,  which,  however,  bear  ordinariiy  little 
resemblance  to  the  colloid-producing  cells  of  the  thyroid  proper. 

Embryologically,  the  thyroid  is  developed  from  three  germinal  centres 
two  lateral  and  one  medial.  The  median  portion  takes  its  origin  in  i 
diverticulum  from  the  floor  of  the  pharynx  between  the  bases  of  the  firs 
and  second  branchial  clefts.  The  lateral  portions  develop  as  evagination: 
from  the  posterior  aspect  of  the  fourth  branchial  arches.  The  fusion  o: 
the  three  parts  usually  occurs  at  about  the  seventh  week/  Originally,  th( 
middle  lobe  was  connected  with  the  pharynx  by  a  duct  called  the  thyro 
glossal  duct.  As' a  rule,  this  disappears  after  the  eighth  week,  but  maj 
persist  more  or  less  completely  for  many  years  in  the  form  of  a  fibrou; 
band  or  a  small  cyst  which  may  discharge  externally  so  as  to  form  i 
median  fistula.  G.  E.  Armstrong2  and  A.  T.  Bazin3  have  both  describee 
cases  of  this  nature  in  young  boys  that  were  successfully  operated  upon 
Usually  all  that  remains  of  the  duct  is  a  small  depression  on  the  surface 
of  the  tongue,  known  as  the  foramen  caecum.  Not  infrequently,  however 
judging  from  our  postmortem  experience,  there  is  a  pyramidal  prolonga- 
tion upward  of  the  middle  lobe  representing  an  accumulation  of  thyroic 
substance  about  the  former  duct. 

The  most  common  and  important  condition  that  we  have  to  deal  witt 
in  case  of  the  thyroid  is  the  so-called  strama  or  goitre.  These  terms,  o: 
course,  strictly  speaking,  apply  solely  to  increase  in  size,  but  hav< 
been  so  loosely  employed  that  much  confusion  has  resulted.  The  wore 
"  goitre"  has  been  used  indiscriminately  for  any  enlargement  of  th< 
gland,  whether  due  to  hyperemia,  hypertrophy,  cystic  dilatation,  fibroic 
induration,  hyperplasia,  or  tumor-formation.  Thus,  anatomically 
speaking,  the  term  is  objectionable,  for  a  great  variety  of  etiologicall) 
differing  conditions  are  arbitrarily  grouped  together,  nor  is  it  better  fronc 
a  clinical  standpoint,  since  widely  differing  symptoms  are  associated 
with  enlarged  thyroid.  The  size  of  the  thyroid  is  the  least  important  ol 
its  characteristics  and  is  only  of  significance  in  those  few  cases  where 
mechanical  pressure  is  exerted  on  the  air  passages.  It  would,  therefore, 
contribute  to  accuracy  if  the  terms  "goitre"  and  "struma"  could  be 
dropped  from  our  nomenclature.  For,  it  is  certainly  more  scientific  tc 
discuss  enlargements  of  the  glands  in  the  light  of  the  etiological  causes 
or  anatomical  peculiarities. 


CONGENITAL  ANOMALIES. 

Complete  or  unilateral  defect  of  the  thyroid  is  rare.     More  frequently 
there  is  absence  of  the   isthmus.      Occasionally,  abnormal  tabulation 

1  Johns  Hopkins  Hosp.  Bull,  19:  1908:  191. 

2  Mont.  Med.  Jour.,  28 : 1899 :  853.  8  Ibid.,  855. 


HYPEREMIA 


689 


FIG.  179 


is  observed  and  the  organ  may  be  divided  into  several  parts  held  together 
by  bloodvessels  and  connective  tissue.  Very  rarely,  the  isthmus  passes 
between  the  oesophagus  and  the  trachea.  Accessory  thyroids  are  not 
infrequently  met  with,  and  may  be  found  at  a  considerable  distance 
from  the  parent  gland,  viz.,  near  the  hyoid  bone,  behind  the  pharynx, 
within  the  larynx  or  trachea,  at  the  superior  clavicular  groove,  and  at 
the  aorta.  Accessory  thyroids  in  the  base  of  the  tongue,  situated  along 
the  course  of  the  thyroglossal 
duct,  have  given  rise  to 
tumors.1  Osier  mentions  hav- 
ing found  accessory  thyroids 
in  the  pleura. 

Premature  atrophy,  or  pos- 
sibly hypoplasia,  is  the  card- 
inal feature  of  cretinism. 

Congenital  enlargement2  is 
important  as  it  may  lead  to 
death  from  pressure  on  the 
air  passages.  Not  only  may 
the  normally-situated  gland 
undergo  this  increase,  but  also 
the  accessories.  The  causes 
are  very  various,  and  include 
hyperemia,  hypertrophy,  tele- 
angiectasis,  cysts,  fibrous  pro- 
liferation, and  adenoma. 

A  strange  anomaly  is  one 
mentioned  by  Wolfler,  who 
found  striated  muscle  in  an 
otherwise  normal  gland. 


CIRCULATORY  DISTURB- 
ANCES. 

Hyperemia.— Owing  to  the 
great  vascularity  of  the   thy- 
roid, circulatory  disturbances 
are  apt  to   be   both  frequent 
and    profound.       Hyperemia 
may  lead  to  a  surprising  en- 
largement of  the  gland.     Passive  congestion  is  met  with  in  valvular  and 
other  heart  affections,  in  suffocation,  and  in  obstruction   of  the  veins  of 
the  neck  from  whatever  cause. 


Exophthalmic  goitre,  or  Graves'  disease.     (From, 
the  Medical  Clinic  of  the  Montreal  General  Hospital.) 


1  J.  C.  Warren,  Amer.  Jour.  Med.  Sci.,  104:  1892:  377. 

2  For  the  literature  on  congenital  struma,  see  Demme,  Gerhardt's  Handbuch  der 
Kinderk.,  3:2. 

44 


690  THE  THYROID  GLAND 

Of  much  interest  is  the  congestive  hyperemia  of  neuropathic  origin, 
that  is  supposed  by  many  to  be  the  essential  lesion  of  Graves'  disease. 
The  enlargement  of  the  thyroid  that  occurs  in  females  at  puberty,  during 
menstruation,  and  pregnancy  is  probably  to  be  placed  in  the  same 
category  (struma  hyperemicd). 

Dilatation  of  the  bloodvessels  (struma  vasculosa)  is  met  with  in  two 
forms,  an  aneurismal  and  a  varicose.  In  the  former  the  arteries,  not 
only  within  the  gland  but  on  its  surface,  are  dilated  and  tortuous,  resemb- 
ling a  cirsoid  aneurism;  in  the  latter  the  veins  and  capillaries  are  affected. 

Hemorrhage. — Hemorrhage  is  frequent,  especially  in  cysts  and  tumors, 
or  when  the  vessels  are  dilated.  It  may  also  be  due  to  trauma. 


INFLAMMATIONS. 

Inflammation  may  affect  the  otherwise  normal  thyroid  (thyroiditis), 
or  one  that  is  chronically  enlarged  (strumitis).  The  latter  event  is  the 
more  common.  The  whole  gland  or  any  part  of  it  may  be  involved. 
Both  exudative  and  productive  forms  are  recognized. 

Acute  Exudative  Inflammation. — Acute  exudative  inflammation  is 
rarely  primary,  but  is  due  to  disease  elsewhere.  It  follows  traumatism, 
or  is  a  complication  of  affections  like  puerperal  infection,  typhoid  fever, 
angina,  septicemia,  Bright's  disease,  pneumonia,  ulcerative  endocarditis, 
and  acute  rheumatism.  The  bacteria  found  include  the  streptococcus 
and  Staphylococcus  pyogenes,  the  Diplococcus  pneumonise  and  the  B. 
typhi  abdominalis. 

The  affected  gland  is  swollen,  hard,  and  painful.  Resolution  may 
rapidly  take  place  or  the  condition  may  go  on  to  abscess-formation. 
Large  areas  may  undergo  purulent  softening,  and  if  cysts  be  present 
they  may  fill  with  pus. 

On  account  of  the  proximity  of  the  large  veins  of  the  neck  there 
is  great  danger  of  thrombophlebitis  and  general  septicemia.  The 
abscesses  may  rupture  into  the  mediastinum,  the  most  frequent  event, 
or  into  the  larynx,  trachea,  or  oesophagus.  When  healing  takes  place 
fibrous  scars  may  result,  or  the  abscess  may  become  encapsulated,  the 
contents  inspissated  and  infiltrated  with  calcareous  salts. 

Chronic  Productive  Inflammation. — Chronic  productive  inflammation, 
or  interstitial  fibrous  hyperplasia,  is  quite  rare,  except  in  the  form  that 
attacks  a  previously  hyperplastic  thyroid  (struma  fibrosa).  Fibrosis 
is,  however,  a  more  common  event  as  a  sequel  of  diffuse  exudative 
thyroiditis  or  abscess. 

Tuberculosis. — Tuberculosis  of  the  normal  or  enlarged  thyroid  is 
invariably  secondary  and  is  due  to  hematogenous  infection.  The  affec- 
tion is  more  common  than  has  been  supposed.  According  to  E.  Fraenkel1 
it  is  usually  present  in  general  miliary  tuberculosis.  Both  miliary  and 
caseonodular  forms  are  described. 

'Virchow's  Archiv,  104:  1886:  58. 


PROGRESSIVE  METAMORPHOSES  691 

Syphilis. — Syphilis  is  excessively  rare. 

Parasites. — Echinococcus  cysts  are  rare.  They  may  discharge  into 
the  trachea. 

Actinomycosis  of  the  thyroid  due  to  extension  of  the  disease  from  the 
neck  has  been  observed.  Occasionally,  it  is  caused  by  metastasis. 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Simple  atrophy  is  a  common  condition  in  old  age.  Here, 
the  acini  are  wasted,  the  interstitial  tissue  is  relatively  increased,  and  the 
vessels  are  sclerosed.  In  some  cases  the  atrophy  is  unilateral. 

Two  special  forms  of  atrophy  call  for  mention,  namely,  that  due  to 
the  continued  exhibition  of  small  doses  of  iodine  and  that  found  in  myx- 
oedema.  The  explanation  is  by  no  means  clear.  In  the  former  case, 
it  would  seem  that  iodine  interferes  in  some  way  with  the  nutrition  of 
the  cells,  so  that  they  become  unable  to  assimilate  foodstuffs  and  thus 
undergo  atrophy.  With  regard  to  myxoedema  there  are  not  wanting 
those  who  look  upon  this  disease  as  primarily  a  neurosis,  for,  as  is  well 
known,  there  is  a  close  relationship  between  the  nervous  system  and 
the  thyroid  gland.  It  is,  however,  difficult  as  yet  to  decide  whether 
the  atrophy  in  this  case  is  a  primary  or  secondary  manifestation. 

Degenerations. — The  various  forms  of  degeneration  affect  the  thyroid 
but  are  most  commonly  found  in  association  with  other  pathological 
conditions  of  the  gland.  Among  them  may  be  mentioned  fatty  degenera- 
tion of  the  glandular  epithelium,  coagulation  necrosis,  hyaline  degeneration, 
and  calcification. 

Amyloid  disease  is  met  with  under  the  usual  conditions,  but  it  is 
interesting  that  it  may  affect  " goitrous"  nodules  to  a  greater  extent  than 
the  rest  of  the  gland. 

Colloid  degeneration  is  described  as  occurring  in  the  thyroids  of  old 
people.  In  some  cases  it  appears  to  be  a  true  colloid  degeneration  of 
the  cells  which  are  small  and  tend  to  disappear,  but  in  others  it  is  simply 
an  arrest  in  the  development  of  a  colloid  struma. 


PROGRESSIVE  METAMORPHOSES. 

In  this  category  we  place  for  convenience  tumors  and  all  those  enlarge- 
ments of  the  thyroid  commonly  known  as  "goitre,"  "struma,"  and 
"  bronchocele,"  with  the  exception  of  those  due  to  simple  hyperemia. 

Inasmuch  as  our  knowledge  of  the  growth  and  overgrowth  of  the 
gland  is  still  somewhat  defective,  it  is  impossible  to  adopt  a  classifica- 
tion of  the  progressive  metamorphoses  that  is  entirely  free  from  objec- 
tion. That  adopted  here  must,  therefore,  be  regarded  as  merely  tenta- 
tive. The  subject  is  still  further  complicated  by  the  conflicting  views 
of  the  different  pathologists,  as  well  as  by  their  loose  use  of  terms.  The 
German  school  for  instance,  speak  of  "benign"  and  malignant"  struma, 


692 


THE  THYROID  GLAND 


meaning  by  the  former  all  simple  forms  of  enlargement,  whether  due  to 
hyperemia,  hyperplasia,  or  glandular  excess,  while  under  the  latter  they 
include  carcinoma  and  sarcoma.  It  would  be  better,  however,  in  dis- 
cussing "struma,"  to  keep  in  our  minds,  when  possible,  the  various 
etiological  factors,  and  draw  a  clear  distinction  between  vascular  dis- 
turbances, hyperplasia  and  hypertrophy,  and  true  tumor-formation. 

Goitre. — In  attempting  any  classification  of  the  goitrous  enlargements 
of  the  thyroid,  we  are  met  at  the  outset  with  the  old  difficulty  of  deciding 
what  enlargements  are  tumors  (adenomata  et  al.)  and  what  are  merely 
hyperplastic  overgrowths.  Wolfler,  in  his  classical  studies  on  this  sub- 

FIG.  180 


Parenchymatous  goitre.     (Dr.  Shepherd's  case,  Montreal  General  Hospital.) 

ject,  attempts,  and  rightly  so,  to  make  this  distinction,  but  his  classifica- 
tion is  in  several  points  open  to  criticism.  He  divides  "goitre"  into 
hypertrophic  and  adenomatous  forms.  In  the  first  group,  which  would 
be  more  correctly  styled  "hyperplastic,"  he  puts  all  those  cases  of  en- 
largement due  to  increase  of  the  specific  glandular  elements  and  vesicles 
or  to  increase  in  their  contents ;  in  the  second,  those  forms  where  there  are 
long,  branching,  cellular  processes  of  embryonal  type,  which  he  regards 
as  epithelial  new-formations.  His  cardinal  point  is  that  the  adenomas 
are  derived  from  the  activity  of  the  interalveolar  embryonic  cells. 

It  is  certainly  incorrect  to  call  a  simple  collection  of  colloid  within  the 


HYPERPLASIA 


693 


FIG.  181 


vesicles  an  hypertrophy,  as  Wolfler  does  (hypertrophia  gelatinosa). 
Again,  it  is  by  no  means  certain  that  we  are  justified  in  making  the  wide 
generalization  that  adenomas  are  always  derived  from  embryonic  cells. 
More  recent  observers,  notably  Hitzig,1  believe  that  new-formations 
resembling  adult  tissue  are  to  be  referred  to  the  overgrowth  of  previously 
existing  adult  cells. 

Hyperplasia. — Hyperplasia  and  regeneration  of  tissue  are  not  infre- 
quently found  in  the  thyroid.  When  a  portion  of  the  gland  is  removed 
or  is  functionally  useless,  compensatory  hyperplasia  occurs.  In  such 
cases,  as  Halstead2  has  shown  experimentally,  there  is  metamorphosis 
of  the  lining  epithelium  of  the  acini 
into  cylindrical  cells  that  tend  to 
assume  a  papillary  arrangement, 
while  the  colloid  material  becomes 
more  mucoid. 

Hyperplasia  may  affect  the  gland- 
ular elements,  the  stroma,  or  both, 
and  leads  to  considerable  enlarge- 
ment of  the  organ.  How  to  class 
this  form  of  goitre  is  difficult,  for 
it  is  not  always  possible  to  draw  a 
hard  and  fast  line  between  hyper- 
plasia and  adenoma.  The  over- 
growth may  be  diffuse  or  nodular; 
possibly  the  latter  variety  is  more 
correctly  to  be  placed  with  the 
adenomas. 

The  first  form  to  be  noticed  is  that 
called  by  Virchow  struma  hyper- 
plastica  parenchymatosa,  where  the 
overgrowth  is  confined  to  the  acini. 
The  growth  is  often  nodular, 
fairly  well  defined,  and  of  a  soft, 
yellowish-gray  appearance. 

Microscopically,  it  is  composed  of 
round,  oval,  and  elongated,  branch- 
ing alveoli,  often  containing  colloid. 
In  other  cases  there  is  an  increase  of  the  fibrous  stroma  with  atrophy  and 
fatty  degeneration  of  the  secreting  cells — struma  hyperplastica  fibrosa. 

Colloid  Goitre. — Another  and  one  of  the  most  frequent  and  important 
forms,  is  the  struma  gelatinosa  or  colloid  goitre.  Of  this  there  are  two 
sorts.  One  is  said  to  be  due  to  the  simple  accumulation  of  colloid  within 
the  vesicles  which  are  not  abnormal,  except  from  pressure.  The  exact 
mode  of  origin  is  doubtful.  The  affection  might  perhaps  be  correctly 
regarded  as  a  form  of  retention-cyst  It  is  significant  that  in  other  cases 


Colloid  struma.  The  thyroid  is  divided  ver- 
tically and  the  anterior  portion  turned  upward. 
(From  the  Pathological  Museum  of  McGill  Uni- 
versity.) 


1Arch.  f.  klin.  Chir.,  47:  1894:464. 

2  Johns  Hopkins  Hosp.  Rep.,  1:  1896:  373. 


694  THE  THYROID  GLAND 

vascular  changes  have  been  described.  The  arteries  show  thickening  of 
the  intima  and  degeneration  of  the  elastic  fibrillae.  In  some  instances  the 
lymphatics  do  not  contain  colloid,  and  it  has,  therefore,  been  suggested 
that  obstruction  of  the  lymphatics  is  a  main  cause.  Given  vascular 
obstruction,  it  is  not  difficult  to  see  how  atrophy  of  the  stroma  will  take 
place,  especially  as  the  condition  is  not  improved  by  pressure  of  the 
colloid  material.  The  walls  of  the  vesicles  may  rupture  and  large  cysts 
filled  with  colloid  result. 

FIG.  182 


Colloid  struma  of  the  thyroid  gland.     The  acini  are  greatly  dilated  and  filled  with  colloid. 
Leitz  obj.  No.  7,  without  ocular.     (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

Tumors. — More  frequently,  however,  colloid  goitre  originates  in  adeno- 
matous  growth.  In  this  case,  nodular  swellings  in  the  thyroid  are  met 
with  that  on  section  appear  to  be  composed  of  dilated  vesicles  contain- 
ing a  firm,  gummy  substance. 

Microscopically,  one  sees  vesicles  of  all  sizes,  from  the  small  acinus  of 
the  normal  gland  to  large  cystic  cavities  lined  with  flattened  epithelium. 
For  the  most  part  the  connective  tissue  between  the  vesicles  is  scanty. 
Owing  to  the  pressure  of  the  accumulated  colloid,  the  vesicular  walls  and 
the  fibrous  stroma  are  atrophic,  and  there  is  a  tendency  for  the  cavities 
to  coalesce,  so  that  a  multilocular  or  even  a  unilocular  cyst  is  the  result. 
In  the  larger  cysts,  the  colloid  is  converted  into  a  slippery,  albuminous 
fluid.  In  another  form  of  colloid  struma,  there  is  a  uniform  enlarge- 
ment of  the  thyroid,  which  on  examination  presents  abundant  colloid 
formation,  cystic  degeneration,  and  fibroid  induration  (interacinous 
adenoma  of  Wolfler).  In  still  a  third  variety,  both  lobes  of  the  gland 
are  enlarged  and  on  the  surface  soft,  rounded  elevations  can  be  felt. 


SARCOMA  695 

On  section,  it  is  a  colloid  goitre,  within  the  cavities  of  which  there  are 
large  papillary  outgrowths  (papillary  cystadenoma). 

In  how  far  these  different  types  are  due  to  the  proliferation  of  embry- 
onic "rests"  must  be  regarded  as  undecided,  but  there  is  one  form,  at  all 
events,  the  so-called  foetal  adenoma,  in  which  Wolfler's  view  is  probably 
correct.  This  growth  is  met  with  at  any  time  from  birth  to  the  age  of 
puberty,  and  its  occurrence  in  young  children  argues  for  its  origin  in 
embryonic  cell-inclusions.  It  forms  multiple  circumscribed  nodules  that 
are  often  extremely  vascular. 

FIG.  183 


Foetal  adenoma  of  the  thyroid.     Winckel  obj.  No.  3,  without  ocular.     (From    the 
collection  of  Dr.  A.  G.  Nicholls.) 

A  rare  form  of  tumor  that  should  be  mentioned  is  the  cylindrical- 
celled  adenoma  which  is  found  both  in  the  normal  and  in  the  strumous 
thyroid. 

Among  the  benign  tumors  may  also  be  mentioned  the  fibroma,  an 
example  of  which  has  been  recorded  by  Wolfler,  and  osteoid  chondroma. 
Whether  chondroma  and  osteoma  occur  is  perhaps  doubtful.  It  would 
be  better  to  restrict  the  term  adenoma  to  those  cases  where  there  is 
a  more  or  less  definite  overgrowth  of  the  glandular  elements,  which  is 
nodular  and  sharply  defined  from  the  rest  of  the  thyroid  substance. 

Sarcoma. — Sarcoma  is  the  most  common  of  the  mesoblastic  tumors, 
and  is  more  liable  to  be  found  in  cases  where  goitrous  enlargement  of 
the  gland  has  preexisted.  It  may  be  round-celled,  spindle-celled,  giant- 
celled,  alveolar,  or  angiomatous.  Rarely,  in  such  tumors  striated  muscle 
fibers  have  been  found  (Wolfler).  Sarcomas  form  nodular  tumors  that 
occupy  more  or  less  of  the  organ,  but  rarely  the  whole. 


696  THE  THYROID  GLAND 

On  section,  the  consistence  varies  and  the  surface  is  smooth  and 
somewhat  intersected  by  fibrous  bands.  The  color  is  white  or  gray,  or, 
again,  admixed  with  red,  according  to  the  amount  of  blood  present. 
The  tumor  grows  rapidly  and  may  penetrate  the  trachea  or  jugular  vein, 
so  that  widespread  metastases  are  quite  common. 

Carcinoma. — Primary  carcinoma  usually  takes  the  form  of  carcinoma 
simplex  or  carcinoma  medullare  and  produces  tumors  varying  in  size 
from  that  of  a  hen's  egg  to  that  of  a  child's  head.  Billroth,  however, 
has  described  cases  where  the  thyroid  was  not  enlarged.  Carcinoma 
develops,  as  a  rule,  in  glands  previously  enlarged  and  forms  either 
grayish-white  nodules  surrounded  by  connective  tissue,  or  a  uniform, 
more  or  less  diffuse,  infiltration  with  only  slightly  altered  parenchyma 
between  the  areas  of  new-growth.  The  nodular  form  is  regarded  by 
Wolfler  as  developing  in  a  follicular-fibrous  goitre,  and  the  diffuse 
variety  from  the  interacinar  embryonal  cells.  Cylindrical-celled  carci- 
noma is  described  as  well  as  papillary  cystic  carcinoma.  In  certain 
cancers  the  stroma  may  undergo  myxomatous  degeneration  (carcinoma 
myxomatodes). 

Squamous-celled  epitheliomas  have  been  met  with,  due  to  the  inclusion 
of  epidermal  cells  during  foetal  life,  but  the  vast  majority  are  examples 
of  secondary  growths. 

A  few  instances  of  mixed  sarcomatous  and  carcinomatous  growths  have 
been  described.  They  are  more  common  in  the  thyroid  than  elsewhere.1 

An  extremely  rare  and  interesting  tumor  is  a  mixed  form  of  carcinoma 
and  perithelial  angiosarcoma,  of  which  an  example  has  been  reported  by 
Woolley.2  Only  four  of  them  are  on  record,  one  of  them  in  a  dog  (Wells3). 

Inasmuch  as  many  carcinomas  contain  colloid  material,  the  secondary 
growths  frequently  produce  the  same  substance.  Any  tumor  containing 
colloid,  particularly  if  found  in  bone,  should  arouse  the  suspicion  of 
a  primary  growth  in  the  thyroid.  According  to  Kolisko,  perithelial 
angiosarcomas  of  the  thyroid  may  give  rise  to  pulsating  secondary  growths. 
Growths  in  the  gland  produce  serious  effects  not  only  from  extension,  but 
also  from  pressure.  The  trachea  may  be  compressed  and  the  cartilages 
eroded,  or  paralysis  may  ensue  from  involvement  of  the  recurrent  laryn- 
geal  nerve.  Invasion  of  the  great  vessels  of  the  neck  may  lead  to  stasis, 
thrombosis,  embolism,  hemorrhage,  and  secondary  growth. 

A  point  of  some  importance  in  regard  to  tumors  is  that  they  are  apt 
to  be  considerably  altered  in  appearance  by  secondary  changes.  The 
connective  tissue  is  frequently  increased  either  diffusely,  or  about  the 
nodules  and  cysts.  It  may  also  show  hyaline  or  mucoid  degeneration. 
Cystic  metamorphosis  is  not  uncommon,  due  to  the  overdistension  and 
rupture  of  adjacent  follicles  or  from  colliquative  necrosis. 

A  special  form  of  cyst  is  the  hemorrhagic  cyst,  formed  by  rupture  of 

1  Leo  Loeb,  Mixed  Tumors  of  the  Thyroid  Gland,  Amer.  Jour.  Med.  Sci.,  125: 
1903:243. 

2  American  Medicine,  4:  No.  9: 1902:  331. 

3  Jour,  of  Path,  and  Bact.,  June,  1901. 


THE  THYMUS  697 

vessels  and  the  discharge  of  blood  into  the  cavities.  Rokitansky1  was 
probably  the  first  to  point  out  that  hemorrhages  occurred  for  the  most 
part  only  in  neoplastic  growths  of  the  thyroid.  This  observation  has 
been  confirmed  here  by  Archibald.2  There  are  two  forms  of  hemor- 
rhagic  cysts:  one  in  which  hemorrhage  takes  place  into  an  ordinary 
colloid  retention-cyst,  and  another  where  extensive  extravasation  both 
into  the  vesicles  and  into  the  interstitial  connective  tissue  occurs.  The 
latter  form  has  been  more  especially  studied  by  Bradley.3 

Calcification  of  the  stroma  and  even  of  the  vesicular  contents  has  been 
observed.  Amyloid  and  fatty  changes,  as  well  as  inflammation,  may 
also  take  place. 

THE  THYMUS. 

The  thymus  is  one  of  the  ductless  glands,  and  its  function  is  as  yet 
by  no  means  thoroughly  understood.  Watney4  has  suggested  that  it 
participates  in  the  formation  of  the  red  and  white  cells.  It  is  possible, 
too,  that  the  gland  elaborates  an  internal  secretion.  Svehla5  has  shown 
that  when  large  doses  of  a  watery  extract  of  the  thymus  are  injected 
into  dogs,  death  with  dyspnoea  takes  place.  This  is  suggestive  in  con- 
nection with  the  subject  of  "thymic  asthma."  Small  amounts  of  an 
iodine-containing  substance  have  been  found  by  Baumann. 

The  thymus  lies  in  the  upper  part  of  the  anterior  mediastinum,  ex- 
tending from  the  pericardium  almost  to  the  thyroid,  and  is  composed  of 
two  long,  flat  lobes,  more  or  less  intimately  united  along  their  median 
aspects.  The  organ  is  enclosed  in  a  connective- tissue  capsule  that  sends 
in  trabeculse  dividing  it  into  lobes  and  again  into  lobules. 

Microscopically,  the  lobules  are  composed  of  acini  that  bear  a  close 
resemblance  to  those  of  the  lymphatic  glands.  In  the  peripheral  zone, 
the  connective  tissue  is  richer  and  the  lymphoid  cells  more  numerous, 
so  that  the  lobules  may  be  divided  into  a  cortical  and  a  medullary  portion. 
A  striking  feature  of  the  picture  is  the  so-called  HassalPs  corpuscles  which 
lie  in  the  middle  of  the  follicles  and  are  composed  of  homogeneous 
and,  toward  the  periphery,  concentrically  arranged  epithelial  elements. 
These  sometimes  calcify. 

The  thymus  is  originally  an  epithelial  structure  derived  from  the  hypo- 
blast  of  the  third  gill  clefts.  Gradually,  however,  the  epithelial  elements 
undergo  atrophy,  the  sole  representative  of  their  existence  being  the 
Hassall's  bodies  which  are  said  to  be  due  to  the  coalescence  of  the  epi- 
thelial remnants,  and  the  structure  is  substituted  by  vascular  connective 
tissue  from  which  the  lymphoid  elements  are  derived. 

The  thymus  weighs  about  twenty-four  grams  at  birth,  and  slightly 

1  Zur  Anat.  des  Kropfes,  Wien,  1849. 

2  Montreal  Med.  Jour.,  25:1897:780. 

3  Jour.  Exp.  Med.,  1:  1896:  401. 

4  Phil.  Trans,  of  the  Royal  Soc.,  3 :  1882. 

5  Wien.  med.  Blatter,  1896. 


698  THE  THYMUS 

increases  in  size  until  the  end  of  the  second  year.  It  then  remains 
stationary  until  the  age  of  puberty,  and  after  that  undergoes  gradual 
involution.  At  the  end  of  the  twentieth  year  it  is  almost  completely 
substituted  by  fat.  According  to  Waldeyer/  remains  of  the  lymphoid 
structure  and  of  the  Hassall's  bodies  are  to  be  recognized  throughout  life. 
Involution  is,  therefore,  not  always  constant,  and  the  gland  in  a  more 
or  less  complete  form  may  persist  into  old  age. 


CONGENITAL  ANOMALIES. 

Complete  absence  of  the  thymus  has  been  observed  in  the  case  of 
monsters,  and,  rarely,  in  otherwise  normal  children.  Accessory  glands 
are  not  uncommon  and  are  usually  found  just  above  the  main  thymus 
and  near  the  thyroid.  Irregularity  in  the  shape  and  lobulation  are  not 
rare.  Enormous  enlargement  is  sometimes  met  with. 


CIRCULATORY  DISTURBANCES. 

These  occur  in  death  from  asphyxia  and  in  the  hemorrhagic  diathesis. 

INFLAMMATIONS. 

Primary  inflammation  is  rare,  if  indeed  it  occur  at  all.  Generally,  the 
affection  is  due  to  extension  from  the  neighboring  organs. 

Suppurative  inflammation  is  found  more  particularly  in  septicemia. 
Care  should  be  taken  not  to  regard  the  yellowish  cellular  juice  of  the 
normal  gland  as  pus,  which  it  much  resembles.  Multiple  abscesses  are 
met  with  and,  also,  diffuse  purulent  infiltration. 

Tuberculosis. — Tuberculosis  is  somewhat  rare,  and  is  found  in 
miliary  form  and  caseous  masses. 

Syphilis. — Syphilis  takes  the  form  of  gummas  or  a  diffuse,  fibroid 
induration. 

RETROGRESSIVE  METAMORPHOSES. 

Focal  necroses  have  been  described  by  Jacobi2  in  connection  with 
diphtheria. 

Degeneration  Cysts. — Degeneration  cysts  containing  puriform 
matter,  which  has  led  to  their  being  mistaken  for  abscesses  (Dubois), 
have  been  described  by  Chiari.3  They  are  due  to  the  ingrowing  of  the 
thymus  tissue  into  the  Hassall's  corpuscles,  and  are  said  to  be  character- 
istic of  congenital  syphilis. 

1  Ruckbildung  der  Thymus,  Centralbl.  f.  d.  med.  Wiss.,  1890. 

2  Trans.  Assoc.  of  Amer.  Phys.,  3:  1888:  297. 

3  Ueber  Cystenbi Idling  in  der  Thymus,  Zeit.  f.  Heilk.,  4 :  1894. 


TUMORS  699 

PROGRESSIVE  METAMORPHOSES. 

Hyperplasia. — The  thymus  may  participate  in  the  general  lymphatic 
enlargement  that  occurs  in  leukemia,  pseudoleukemia,  and  the  "  status 
lymphaticus." 

Hyperplasia  may  occur  after  birth  and  has  been  noted  in  connection 
with  epilepsy  (Ohlmacher1),  exophthalmic  goitre  (Hektoen2)  in  acro- 
megaly,  myxoedema,  and  Addison's  disease.  It  is  most  commonly 
associated  with  a  general  lymphoid  hyperplasia,  and  is  of  interest  on 
account  of  its  relationship  to  cases  of  sudden  death.  The  exact  mechan- 
ism is  not  as  yet  determined.  Some  think  that  death  is  due  to  irritation 
of  the  inferior  laryngeal  nerves,  or  to  pressure  on  the  vagi  and  trachea. 
As  before  suggested  it  may  be  toxic.  There  have  only  been  one  or  two 
cases  in  which  compression  of  the  trachea  has  been  discovered  post 
mortem,  so  that  mechanical  pressure  does  not  appear  to  be  an  important 
factor.  This  much  should  be  said,  however,  that  it  is  not  impossible, 
or  even  unlikely  that  the  organ  may  be  subject  to  sudden  hyperemic 
enlargement,  a  condition  that  at  times  might  pass  off  before  the  case 
came  to  autopsy. 

Epithelial  transformation  of  the  thymus  has  been  described  by 
Lochte.3 

Tumors. — The  thymus  is  the  favorite  site  of  origin  of  mediastinal 
growths.  Of  the  benign  tumors  may  be  mentioned  angioma,  of  which 
an  example  has  been  described  by  Osier,  and  dermoid  cysts.  The  latter 
originate  in  persistent  epithelial  "  rests"  and  contain  yellowish-white, 
fatty  and  granular  material,  together  with  hair. 

The  most  frequent  tumor  is  sarcoma  in  its  various  forms.4  Lympho- 
sarcoma  may  be  recognized  by  its  smooth  homogeneous  appearance, 
conforming  to  some  extent  to  the  normal  outlines  of  the  gland,  in  contra- 
distinction to  the  more  irregular  and  nodular  arrangement  of  sarcoma 
originating  in  the  lymphatic  glands. 

Carcinoma  may  be  developed  from  the  epithelial  structures. 

Tumors  of  the  thymus  are  important,  since  they  may  grow  rapidly 
and  encroach  upon  vital  structures  like  the  heart,  lungs,  and  great 
vessels. 

1  Bull.  Ohio  Hosp.  for  Epileptics,  1898  and  1899. 

2  Internat.  Med.  Magaz.,  1896. 

d  Centralbl.  f.  allg.  Path.  u.  path.,  Anat.,  10:  1890:  1. 

4  Schneider,  Fibrosarcoma,  Inaug.  Diss.,  Greifswald,  1892. 


CHAPTER    XXXIII. 

THE  SUPRARENAL  GLAND,  PITUITARY,  PINEAL  AND  CAROTID 
BODIES,  AND  COCCYGEAL  GLAND. 

THE  SUPRARENALS. 

THE  suprarenals,  or  adrenals,  are  a  pair  of  organs  situated  at  the 
upper  end  of  the  kidneys,  with  which  they  lie  in  close  apposition. 
They  originate  in  the  same  embryological  elements  as  the  kidneys. 

In  shape  the  suprarenal  gland  is  generally  compared  to  a  cocked  hat, 
and  consists  of  a  cortex  and  medulla,  enclosed  in  a  fibrous  capsule,  which 
sends  prolongations  into  the  interior  of  the  structure.  The  cortex  is 
composed  of  three  zones.  The  outer,  or  zona  glomerulosa,  consists 
of  numerous  spherical  or  oval  masses  of  cells,  of  cylindrical  or  polyhedral 
shape,  containing  a  spherical  or  oval  nucleus.  The  middle  zone,  zona 
fasciculata,  is  composed  of  vertical  columns  of  polygonal  epithelial  cells, 
having  a  spherical  nucleus.  The  protoplasm  is  clear  and  pale,  and 
the  cell-bodies  are  usually  loaded  with  fat.  Between  the  columns  are 
fibrous  septa  containing  blood  capillaries.  The  innermost  layer,  the  zona 
reticularis,  is  formed  of  irregular  masses  of  polyhedral  cells,  the  various 
clusters  of  which  anastomose  one  with  the  other.  The  cells  are  somewhat 
larger  than  those  of  the  zona  fasciculata,  and  are  often  slightly  pigmented. 
The  medulla  consists  in  cylindrical  clusters  of  transparent  cells,  which 
are  polyhedral,  columnar,  or  branching.  The  cell-groups  here  also 
anastomose  with  each  other.  The  medulla  is  often  deeply  pigmented 
and  is  particularly  rich  in  bloodvessels,  non-medullated  nerve-fibers, 
chromaffin  cells,  and  ganglia.  In  the  centre  is  a  large  vein,  surrounded 
by  a  comparatively  large  amount  of  unstriped  muscle. 

The  physiological  importance  of  the  suprarenal  glands  is  somewhat 
obscure.  It  is  generally  accepted,  however,  that  they  belong  to  the  cate- 
gory of  ductless  glands  and  elaborate  an  internal  secretion  of  great  im- 
portance to  the  economy.  The  great  vascularity  of  the  organs  and  the 
close  relationship  of  the  capillaries  to  the  groups  of  cells  support  this 
view.  The  muscle  bundles  surrounding  the  central  vein  suggest  a 
mechanism  for  controlling  the  amount  of  blood  in  the  organ.  This  is 
possibly  regulated  by  the  sympathetic  system,  the  fibers  and  ganglia  of 
which  are  particularly  numerous  in  the  medulla.  Whatever  the  complete 
action  of  the  internal  secretion  may  be,  we  have  experimental  proof  that 
extracts  and  alkaloids  derived  from  the  medulla  have  the  power  of  con- 
tracting bloodvessels  and  increasing  blood  pressure,  while  in  cases  where 
the  suprarenals  are  extensively  disorganized,  as  from  tuberculous  disease 
or  tumors,  cardiovascular  phenomena  and  asthenia  are  marked  features. 


ADDISON'S  DISEASE  701 

This  is  well  seen  in  Addison's  disease.  In  this  affection,  in  the  majority 
of  cases,  caseous  tuberculosis  of  the  glands  is  found,  although  other 
gross  lesions,  such  as  tumors,  may  be  present.  Addison's  disease  does 
not,  however,  usually  result  unless  the  medullary  portion  is  largely 
destroyed.  Exceptional  cases  are  on  record  where  the  suprarenals  were 
found  to  be  normal,  the  only  lesions  being  in  the  semilunar  ganglia  and 
the  solar  plexus  (see  also  p.  681). 

Clinically,  the  disease  is  characterized  by  great  bodily  weakness, 
cardiovascular  asthenia,  vomiting,  and  a  peculiar  pigmentation  of  the 
skin  and  mucous  membranes.  The  pigmentation,  which  is  brownish, 
occurs  first  as  an  obscure  mottling,  but  soon  becomes  uniform.  It 
affects  chiefly  the  skin  of  the  face,  neck,  hands,  flexures  of  the  joints,  and 
any  parts  subjected  to  irritation. 

What  connection  there  is  between  the  suprarenal  and  pigmentation 
of  the  skin  is  still  largely  unknown.  That  there  is  some  relation  is  indi- 
cated by  the  fact  that  in  negroes  the  glands  are  deeply  pigmented  (see 
also  p.  681). 

CONGENITAL  ANOMALIES. 

Complete  defect  and  hypoplasia  are  rare.  There  seems  to  be  some 
relationship  between  the  development  of  the  suprarenals  and  that  of 
the  brain,  for  in  hydrocephalus  and  anencephaly,  hypoplasia,  partial 
aplasia,  and  fibrosis  of  the  former  organs  have  been  found.1 

Accessory  suprarenals  form  the  most  common  anomaly.  They  may 
be  found  in  widely  distant  parts,  in  the  capsule  of  the  suprarenal,  at 
the  hilus  of  the  kidney,  on  the  renal  and  spermatic  veins,  in  the  liver, 
on  the  ovary,  and  broad  ligament.  The  accessories  may  be  single  or 
multiple,  and  are  more  common  in  children.  Of  special  interest  are 
those  found  in  the  capsule  of  the  kidney,  between  the  capsule  and  the 
cortex,  or  within  the  kidney  substance,  since,  according  to  Grawitz  and 
others,  these  misplaced  "rests"  may  give  rise  to  tumors  (see  p.  768). 


CIRCULATORY  DISTURBANCES. 

Hyperemia. — Passive  congestion  is  common,  and  is  met  with  under 
the  same  conditions  as  elsewhere. 

Hemorrhages. — Hemorrhages  into  the  substance  of  the  gland,  either 
minute  or  larger  (hematoma)  are  met  with  from  traumatism  at  birth, 
in  passive  congestion,  fatty  degeneration  of  the  vessels,  inflammation, 
leukemia,  and  the  hemorrhagic  diathesis.  Chiari  has2  described  a  case 
in  which  the  hematoma  was  as  large  as  a  man's  head  and  weighed 
2  kilos.  In  some  cases  calcification  results  and  phleboliths  have  been 
observed. 

1  Weigert,  Virch.  Archiv,  100:  1885:  176:  u.  103;  1886:  204. 

2  Wien.  med.  Presse,  21 : 1880. 


702  THE  SUPRARENALS 

Woolley1  has  recorded  a  case  of  hemorrhagic  infarction  of  the  right 
suprarenal  in  the  newborn,  due  to  thrombosis  of  the  central  vein. 


INFLAMMATIONS. 

Hemorrhagic  Inflammation. — Under  the  term  hemorrhagic  inflam- 
mation, Virchow  has  described  a  condition  in  which  the  suprarenal  is 
swollen,  thickened,  and  infiltrated  with  blood.  Microscopically,  fatty 
degeneration  is  marked. 

Small  collections  of  inflammatory  round  cells  are  sometimes  met  with 
in  the  interstitial  substance  in  cases  of  infection,  pyemia  and  the  like. 
They  are  probably  due  to  embolic  infection. 

FIG.  184 


Early  caseous  tuberculosis  of  the  suprarenal  gland.     Leitz  obj.  No.  7,  without  ocular. 
{From  the  collection  of  Dr.  A.  G.  Nicholls.) 

Suppurative  Inflammation. — Suppurative  inflammation,  either  local- 
ized or  diffuse,  is  met  with  in  general  septicemia.  Abscesses  of  con- 
siderable size  may  result,  and  sometimes  burst  into  the  colon  or 
duodenum.  The  suprarenal  may  be  secondarily  involved  in  inflamma- 
tory processes  originating  in  the  kidney,  and  (in  the  case  of  the  right 
suprarenal)  in  the  liver. 

Tuberculosis. — Tuberculosis  of  the  suprarenals  is  not  uncommon 
in  cases  of  advanced  disease  of  the  lungs  and  other  organs.  Small 
miliary  granulomata  may  often  be  seen  in  the  interstitial  substance. 

1  Jour,  of  Med.  Research,  New  Series,  2:  No.  2:  1902:  231. 


TUMORS  703 

Larger  caseous  nodules  may  be  found  occupying  the  centre  of  the 
gland,  and,  through  liquefaction  and  necrosis,  may  convert  it  into  a 
kind  of  cyst.  Tuberculosis  of  these  organs  is  the  usual  lesion  found  in 
cases  of  Addison's  disease. 

Syphilis. — Syphilis  is  rare.  It  usually  takes  the  form  of  gumma. 
Coagulation  necroses  resembling  anemic  infarcts  have  been  met  with 
in  congenital  lues.1 


RETROGRADE  METAMORPHOSES. 

Atrophy. — Simple  atrophy  is  met  with  in  old  age  and  general  maras- 
mus, and,  occasionally,  in  Addison's  disease.  The  chief  feature  is  the 
great  diminution  in  the  amount  of  the  fat  which  is  normally  present  in 
all  adult  suprarenals. 

The  gland  is  often  found  to  be  softened  in  the  centre  and  cavitated. 
This  is  probably  a  postmortem  change,  although,  possibly,  in  some 
cases  this  may  be  assisted  by  careless  removal. 

Cloudy  Swelling. — Cloudy  swelling  is  met  with  here  under  the 
same  conditions  as  elsewhere. 

Fatty  Degeneration. — A  true  fatty  degeneration,  associated  with 
atrophy  and  disappearance  of  the  nuclei,  has  been  described  in  connection 
with  marasmus,  anemia,  and  affections  of  the  heart,  lungs,  and  vessels. 

Amyloid  Disease. — Amyloid  disease  is  fairly  common  in  all  cases 
of  widespread  amyloid  transformation.  It  affects,  chiefly,  the  walls  of 
the  capillaries  between  the  columns  of  the  zona  fasciculata. 

Coagulation  Necrosis. — Coagulation  necrosis,  either  focal  or 
diffuse,  is  a  rather  common  condition,  met  with  in  puerperal  eclampsia, 
chronic  tuberculosis,  and  a  great  variety  of  infections  and  intoxications. 
The  cells  of  the  zona  fasciculata  are  the  ones  usually  picked  out.  They 
are  opaque,  turbid,  have  lost  their  clear  vesicular  appearance,  and  the 
nuclei  stain  badly  or  not  at  all. 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — Virchow2  has  described,  under  the  term  struma  lipomatosa 
suprarenalis,  a  diffuse,  or,  occasionally,  a  nodular  overgrowth  of  the 
cortical  substance.  The  nodules  are  single  or  multiple,  sometimes 
bilateral,  and  may  reach  the  size  of  a  walnut.  They  are  yellowish  in 
color  and  are  situated  in  the  cortex,  less  frequently  in  the  medulla.  Micro- 
scopically, the  growths  are  composed  of  long,  sometimes  branched, 
masses  of  cells,  similar  to  those  of  the  zona  fasciculata,  containing  fat, 
and  often  pigment.  In  many  cases  the  connective  tissue  is  increased. 
Cystic  degeneration  and  calcification  sometimes  occur. 

1  Kokubo,  Centralbl.  f.  allg.  Path,  und  path.  Anat.,  14:  Nos.  16  and  17:  1903. 

2  Die  krankhafte  Geschwiilste,  2. 


704  THE  SUPRARENALS 

A  second  form  of  new-growth  has  also  been  described  by  Virchow,  as 
a  glioma,  which  takes  the  form  of  a  nodular  mass  in  the  medulla.  The 
cells  composing  it  are  pale,  irregular  or  stellate,  faintly  granular,  with 
relatively  large  nuclei,  and  have  been  regarded  as  homologous  with 
those  of  the  normal  medulla.  The  tumor  may  be  as  large  as  a  rasp- 
berry. A  ganglionic  neuroma  and  a  gangliofibromyoma  have  also  been 
described.  Lipoma  is  said  to  occur.  Angioma  and  cavernous  lymph- 
angioma1  are  rare. 

Among  the  malignant  tumors  that  have  been  described  are  sarcomas 
of  various  types,  round-celled  sarcoma,  myxosarcoma,  angiosarcoma, 
melanosarcoma,  lymphosarcoma,  and  carcinoma,  so-called. 

Malignant  tumors  may  arise  from  the  cortex  or  the  medulla. 

Those  originating  in  the  cortex  are  composed  of  cells  varying  more  or 
less  widely  from  those  of  the  normal  cortex,  but,  as  a  rule,  it  is  possible 
to  make  out  the  transition.  The  cells  are  moderately  large,  polyhedral 
or  flattened,  containing  a  relatively  large,  oval,  or  irregular  nucleus  with 
a  deeply  staining  chromatin-net.  The  interstitial  stroma  is  not  intercellu- 
lar, but  surrounds  masses  or  columns  of  the  cells.  Multinucleated  cells 
are  numerous.  Such  tumors  from  their  histological  appearance  might, 
therefore,  be  called  carcinomas.  In  view  of  the  recent  careful  studies 
of  Minot,2  Aichel,3  and  others,  however,  we  have  to  believe  that  the 
suprarenals  are  derived  in  their  entirety  from  the  Wolffian  body  and 
are,  consequently,  mesoblastic,  or,  more  precisely,  to  use  Minot's  term, 
mesothelial.  Malignant  tumors  of  such  derivation  cannot  properly, 
then,  be  termed  carcinomas,  if  we  attach  to  this  name  its  usual  significa- 
tion. As  a  matter  of  fact,  these  tumors,  microscopically,  present  in  some 
cases  a  carcinomatous  appearance,  and  in  others  are  more  like  the  sar- 
comas. As  illustrating  this  point,  Woolley4  has  described  a  tumor  of 
the  suprarenal,  resembling  a  carcinoma,  the  metastases  of  which  in  the 
lung,  brain,  and  lymph-glands  were  indistinguishable  from  sarcoma. 
The  secondary  growths  in  the  lung  were  of  transitional  appearance, 
varying  from  a  tumor  composed  of  polyhedral  cells  in  the  younger 
portions  to  a  spindle-celled  form  in  the  older.  In  the  brain,  the  meta- 
stases were  chiefly  composed  of  round  cells.  Possibly  it  would  be 
less  confusing  if,  with  Woolley,  we  speak  of  tumors  arising  from  the 
parenchyma  of  the  adrenals  as  mesotheliomas,  irrespective  of  their  his- 
tological appearance. 

Tumors  composed  of  polygonal  cells,  with  oval  nuclei,  and  containing 
granular  pigment,  have  been  described  by  Morris,  among  others.  They 
are  strictly  comparable  to  the  normal  medullary  structure.  Malignant 
new-growths  of  the  medulla,  however,  often  resemble  ordinary  round- 
celled  sarcoma. 


1  Oberndorfer,  Beit.  z.  allg.  Path.  u.  z.  path.  Anat.,  29:  1901:  516. 

2  The  Embryological  Basis  of  Pathology,  Science,  New  Series,  13:  1901:  481. 

3  Vergleichende  Entwickl.  u.  s.  w.  der  Neben.  Arch.  f.  mikros.  Anat.,  65: 1900: 1. 

4  A  Primary  Carcinomatoid  Tumor  (Mesothelioma)  of  the  Adrenals,  with  Sarcom- 
atous  Metastases,  Trans.  Assoc.  Amer.  Phys.,  17: 1902:  627. 


THE  HYPOPHYSIS  OR  PITUITARY  BODY  705 

Secondary  carcinoma,  sarcoma,  and  endothelioma  are  met  with  in  the 
suprarenals. 

The  malignant  tumors  originating  in  the  suprarenal  are  particularly 
liable  to  invade  the  veins,  and  metastases  are  usually  rapidly  produced. 
It  has  been  noted  that  they  are  liable  to  be  associated  at  the  same  time 
with  similar  tumors  of  the  thyroid  or  genitalia. 

It  should  be  remarked  in  concluding  the  discussion  of  tumors  of  the 
suprarenal  that  the  nomenclature  is  at  present  confusing.  This  is 
perhaps  due  to  the  fact  that  the  exact  nature  of  many  of  these  new- 
growths  is  open  to  debate.  It  is  the  fashion  nowadays,  however,  to 
speak  simply  of  all  tumors  arising  from  the  parenchyma  of  the  suprarenal 
or  of  suprarenal  "rests"  as  hypernephromata  (Birch-Hirschfeld).  Many 
of  the  growths  called  by  the  earlier  writers  "malignant  tumor,"  "alveolar 
sarcoma,"  "carcinoma,"  would  properly  be  classified  under  this  head 
(see  p.  768). 

THE  HYPOPHYSIS  OR  PITUITARY  BODY. 

The  pituitary  body  is  a  peculiar  gland-like  structure  about  the  size  of 
a  pea,  situated  at  the  base  of  the  brain,  to  which  it  is  attached  by  a  slender 
stalk.  It  weighs  usually  from  3  to  6  decigrams  and  fits  snugly  into  an 
excavation  in  the  floor  of  the  skull,  known  as  the  sella  turcica.  It  is 
generally  described  as  consisting  of  an  anterior  and  a  posterior  portion, 
together  with  a  stem  or  infundibulum.  The  anterior,  or  glandular 
part,  is  composed  of  a  connective-tissue  stroma,  rich  in  capillaries,  in 
which  are  embedded  rounded  or  elongated  masses  of  epithelial  cells  or 
acini,  rather  closely  packed  together.  The  cells  composing  these  acini 
are  polyhedral,  round  or  oval,  and  are  of  two  main  varieties,  chromo- 
philic  and  chromophobic.  In  the  chromophilic  cells,  the  cytoplasm  is 
abundant  and  contains  granules  that  stain  reddish  or  yellowish  with  eosin, 
or  else  assume  a  purplish  tinge  with  hematoxylin.  Klotz,  working  at  the 
Royal  Victoria  Hospital,  has  noted  the  fairly  constant  presence  of  clear 
mononuclear  cells  that  have  an  affinity  for  acid  dyes.  The  chromophobic 
cells,  or  "chief"  cells,  are  more  numerous,  smaller,  and  have  a  clear 
cytoplasm.  According  to  Berkeley,1  the  nerve  elements  are  derived 
from  the  carotid  sympathetic  plexus.  At  the  periphery  of  the  lobe, 
and  especially  in  the  boundary  zone  between  the  two  lobes,  the  acini  are 
often  dilated,  lined  with  cubical  cells,  and  filled  with  what  appears  to  be 
a  colloid  substance,  which  is  also  to  be  seen  in  the  neighboring  lymph 
spaces  and  vessels. 

The  smaller  posterior,  or  infundibular,  portion  of  the  hypophysis  is 
composed  of  vascular  connective  tissue  in  which  are  numerous  spindle- 
shaped  or  stellate  cells,  often  pigmented.  The  structure  is  practically 
that  of  neuroglia.  Berkeley  has  reported  the  presence  of  ganglia  and 
varicose  nerve-fibrils,  but  Kolliker  denies  the  existence  of  nerve  elements 
in  this  portion. 

1  Johns  Hopkins  Hosp.  Rep.,  4:  1895:  2S5. 
45 


706 


THE  PITUITARY  BODY 


In  the  boundary  zone  between  the  two  lobes  the  vessels  are  particularly 
numerous,  and,  in  man,  there  is  a  bilobed  cavity  lined  with  ciliated 
cylindrical  epithelium. 

The  embryological  development  of  the  pituitary  is  curious  and  sug- 
gestive. In  the  amphioxus  and  ammocetes  there  is  a  duct  which  passes 
from  the  buccal  to  the  neural  cavity.  This  is  lined  with  ciliated  epithe- 
lium, and  in  its  wall  are  glandular  structures.  Nerve-cells  are  grouped 
about  the  end  which  communicates  with  the  ventricular  cavity.  And- 
riezen1  regards  this  duct  as  a  nutrient  channel,  through  which  water, 
pigment  granules,  and  other  substances  are  conveyed  to  the  central 
neural  canal.  With  Gaskell,2  we  must  consider  it  to  be  the  anterior  ex- 
tremity of  the  primitive  gut  or  neurenteron. 


FIG.  185 


Epiphysis. 


Cer.  evagination 

(hypophysis). 

Buccal  evagination. 


— Chorda  dorsahs. 


Buccal  cavity.       Pharynx. 

Origin  of  epiphysis  (pineal  gland)  and  hypophysis  (pituitary  gland).  The  blue  part  corre- 
sponds to  the  intermediate  brain  or  thalamencephal.  The  red  part  is  a  portion  of  the  buccal 
ectoderm.  The  hypophysis  is  formed  by  the  coalescence  of  two  evaginations  (after  Mihalcovics, 
as  modified  by  Charpy  in  Poirier's  Traite*  d' anatomic  Humaine.) 

In  man,  the  pituitary  has  a  twofold  origin.  The  anterior,  glandular 
portion  originates  in  a  diverticulum  from  the  primitive  oral  cavity.  In 
time,  the  proximal  portion  of  the  resulting  tube  becomes  constricted  and 
impermeable;  traces  of  it  are  frequently  to  be  detected.  Lanzert  states 
that  he  has  recognized  the  canalis  craniopharyngeus  in  10  per  cent,  of 
children  examined.  Suchannek  also  found  in  a  child  a  prolongation  of 
the  dura  mater,  surrounding  a  column  of  epithelial  cells,  which  extended 
from  the  pituitary  through  the  sphenoid  bone  to  a  cul-de-sac  in  the 
posterior  portion  of  the  pharyngeal  vault.  The  Luschka's  tonsil  marks 
the  site  of  the  termination  of  the  duct.  Occasionally  cysts  are  found  at 

1  Brit.  Med.  Jour.,  1:  1894. 

2  The  Origin  of  Vertebrates,  Longmans,  Green  &  Co.,  1908:  321. 


THE  FUNCTION  OF  THE  PITUITARY  BODY  707 

this  point,  due  to  closure  of  the  so-called  pouch  of  Rathke,  from  which 
the  duct  springs. 

The  posterior  lobe  is  formed  by  an  evagination  from  the  floor  of  the 
primitive  midbrain,  the  cavity  of  which  is  in  communication  with  the 
central  neural  canal.  The  proximal  portion  here  also  becomes  con- 
stricted and  forms  the  infundibulum,  the  lumen  being  in  time  generally, 
though  not  invariably,  obliterated.  In  the  higher  vertebrates  the  two 
diverticula  just  described  come  in  contact  and  unite,  but  do  not  com- 
municate. The  mode  of  development  is  well  seen  in  the  accompanying 
diagram  (Fig.  185). 

The  persistence  of  the  pituitary  in  some  form  or  other  throughout  a 
wide  range  of  animal  life  and  its  rather  complicated  structure  in  the 
higher  forms  suggest  that  it  plays  an  important  part  in  the  economy. 

FIG.  186 


Diagram  to  illustrate  the  mutual  relationship  of  certain  of  the  ductless  glands,  and 
their  connection  with  bodily  development. 

The  nature  of  its  function,  while  still  obscure,  is  gradually  becoming 
clearer.  Evidence  is  accumulating  which  goes  to  prove  that,  like  the 
thyroid  and  the  suprarenals,  it  belongs  to  the  group  of  ductless  glands, 
and  elaborates  an  internal  secretion  of  importance  in  metabolism. 
There  is,  for  instance,  a  somewhat  close  resemblance  to  the  thyroid  gland, 
which  is  generally  admitted  to  elaborate  an  internal  secretion,  in  its 
vascularity  and  the  presence  of  acini  and  lymphatic  vessels  containing 
colloid.  Schnitzler  and  Ewald1  have  also  demonstrated  the  existence  of 
iodine  in  minute  amounts  in  the  pituitary.  There  is,  further,  a  compen- 
satory hypertrophy  of  the  organ  after  thyroidectomy  in  the  lower  animals 
(Rogo witch,2  Hofmeister3),  and  in  cases  of  atrophy  of  the  thyroid  (Boyce 
and  Beadles4).  Then,  again,  those  curious  anomalies  of  growth  and 
development,  known  as  acromegaly  and  gigantism,  in  a  large  majority 
of  the  cases  are  associated  with  lesions  in  the  hypophysis,  such  as  cysts, 

1  Wien.  klin.  Woch.,  1896.  2  Ziegler's  Beitrage,  4:  1889:  453. 

3  Fortschritte  der  Medizin,   1892.  4  Jour,  of  Path.,  1:  1893:  223,  359. 


708  THE  PITUITARY  BODY 

hypertrophy,  or  tumors.  In  acromegaly,  too,  the  thyroid  has  occasion- 
ally been  found  enlarged,  cystic  or  atrophic,  and  the  disease  may  be 
complicated  with  myxoedema  or  exophthalmic  goitre.  There  appears 
also  to  be  some  relationship  between  the  pituitary  and  the  genital  system. 
One  of  us  (Nicholls)  some  years  ago  performed  an  autopsy  on  a  woman, 
about  thirty  years  of  age,  with  a  tumor  of  the  pituitary,  in  whom  there 
was  a  pronounced  infantile  condition  of  the  sexual  apparatus,  and  a 
similar  state  of  things  has  been  noted  in  the  male  (Pechkranz1).  Genital 
inadequacy,  genital  hypoplasia,  and  malformation  have  also  been  fre- 
quently observed  in  acromegaly  and  gigantism  (Gamier  et  Santenoise,2 
Thoma,3  Babinski4). 

Thus,  as  the  earlier  anatomists  were  feign  to  make  a  fanciful  com- 
parison between  the  three  vital  centres — heart,  lungs,  and  brain — and  a 
tripod,  which  they  called  the  "  tripod  of  life,"  so  we  may  see  a  threefold 
relationship  between  the  ductless  glands — pituitary,  thyroid,  and  sexual 
glands — and  somatic  development.  Although,  if  we  have  to  admit  the 
right  of  the  suprarenals  to  be  classed  with  the  other  organs  controlling 
this  particular  form  of  metabolism,  as  seems  possible,  our  tripod  is  in 
danger  of  becoming  a  four-legged  stool. 

Other  phases  of  this  subject  have  been  discussed  in  the  introduction 
to  this  section  (p.  675). 

CONGENITAL  ANOMALIES. 

Partial  or  complete  persistence  of  the  ductus  craniopharyngeus,  and 
cysts  in  its  course  have  already  been  referred  to.  Absence  of  the  pituitary 
body  is  very  rare. 

CIRCULATORY  DISTURBANCES. 

Hyperemia. — Hyperemia  is  found  in  general  cerebral  congestion  and 
in  inflammatory  and  circulatory  disorders  at  the  base  of  the  brain. 

Hemorrhage. — Hemorrhage  into  the  posterior  lobe  has  been  observed 
(Eppinger).  It  is  often  agonal. 


INFLAMMATIONS. 

Inflammation  of  the  hypophysis  is  generally  secondary  to  disease  of  the 
meninges  or  of  the  bones  at  the  base  of  the  skull.  Stengel5  mentions  a 
case  in  which  the  anterior  lobe  was  inflamed,  apparently  from  infection 
which  reached  it  from  the  parotid  through  the  retropharyngeal  lymphatics. 

1  Zur  Casuistik  der  Hypophysis-Tumoren,  Neurol.  Centralbl,  No.  5:  1899:  203. 

2  Arch,  de  neurol.,  March,  1898. 

3  Text-book  of  Gen.  Path.,  1:  1896:  198,  English  edition. 

4  Neurol.  Soc.  of  Paris.,  7:  June,  1900. 

5  Text-book  of  Pathology,  1900:  796,  W.  B.  Saunders  &  Co.,  Philadelphia. 


TUMORS  709 

Tuberculosis. — Caseous  tubercles  have  been  described  as  occurring 
in  the  pituitary  by  Boyce  and  Beadles.1 

Syphilis. — According  to  Lancereaux,  the  pituitary  is  enlarged  and 
indurated  in  congenital  syphilis.  Gummas  have  also  been  met  with.2 

Parasites. — A  few  cases  of  Echinococcus  cysts  have  been  observed 
(Sommering,  Lancereaux). 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy  may  occur.  In  cretinism  and  myxcedema,  the  sella  turcica  has 
been  found  to  be  enlarged,  quite  out  of  proportion  to  the  gland  resting 
in  it.  This  has  been  explained  as  due  to  an  involution  of  the  hypophysis 
subsequent  to  a  previous  enlargement. 

Large  colloid  deposits  are  sometimes  found  within  dilated  follicles  and 
in  the  lymphatics.  Whether  this  is  to  be  interpreted  as  due  to  over- 
activity  of  the  gland  or  as  a  degenerative  manifestation  is  doubtful. 

Fatty  degeneration  of  the  cells  and  hyaline  changes  in  the  vessels  have 
been  described  in  old  people.  Amyloid  disease  of  the  bloodvessels  has 
been  observed  in  advanced  general  amyloidosis.  Calcareous  deposits 
occasionally  may  be  found. 


PROGRESSIVE  METAMORPHOSES. 

Vicarious  hypertrophy,  often  of  considerable  amount,  has  been  noted 
in  experimental  thyroidectomy,  fibroid  goitre,  cretinism,  and  myxcedema. 

Tumors .— Struma  or  goitre  of  the  pituitary  body  is  a  condition  not 
always  readfly  distinguishable  from  adenoma.  In  it  the  organ  is  con- 
siderably enlarged,  owing  to  the  cystic  dilatation  of  the  acini  with  an 
excess  of  colloid  material,  together  with  proliferative  and  vascular  changes 
in  the  stroma.  The  enlarged  pituitary  may  reach  the  size  even  of  a  hen's 
egg  and  produce  marked  pressure  disturbances  in  the  brain  and  floor 
of  the  skull. 

Cysts,  lined  with  ciliated  epithelium  and  containing  homogeneous  or 
granular  material,  have  been  described  by  Weichselbaum.3 

Considering  the  complicated  development  of  the  hypophysis,  we 
should  expect  to  find  that  teratoid  new-formations  were  rather  common, 
but  this  is  not  the  case.  Dermoid  cysts  have  been  described  by  Beck4 
and  Weigert.5  Hale  White  has  reported  a  neuromyoma  composed  of 
striated  muscle  and  medullated  nerve-fibers.  Cystic  tumors,  presum- 
ably derived  from  the  infundibular  canal,  have  been  noted  by  Rayer, 
Rokitansky,  and  Langer. 

1  Jour,  of  Path,  and  Bact.,  1:  1893:  223  and  359. 

2  Hektoen,  Trans.  Chicago  Path.  Soc.,  2:  1897:  129. 

3  Xeubildungen  der  Hypophysis,  Virch.  Archiv,  75:  1879:  444. 

4  Teratom.    Zeit,  f .  Heilk.,  4 :  1883. 
5Teratom.  Virch.  Archiv,  75:  1875. 


710  THE  PITUITARY  BODY 

Of  the  simpler  benign  tumors  may  be  mentioned  lipoma  (Weichsel- 
baum),  angioma  and  chondroma  (Lancereaux). 

The  most  important  growths,  however,  are  the  carcinoma  and  sar- 
coma. These  form  diffuse  or  nodular  masses,  destroying  the  structure  of 
the  gland,  and  often  leading  to  infiltration  of  the  brain,  optic  tracts,  and 
base  of  the  skull.  They  may  extend  to  the  nasopharynx  and  orbit. 
The  pituitary  being  mainly  epiblastic,  the  new-growths  originating 
in  the  parenchyma  are  properly  to  be  termed  carcinomas.  Sarcomas 
probably  arise  from  the  sheath  of  the  vessels,  the  endothelial  linings  of 
the  blood  and  lymph-channels,  and  from  the  pia.  We  have  met  with 
one  case  of  endothelioma1  and  another  of  perithelial  angiosarcoma. 


FIG.  187 


Perithelial  angiosarcoma  of  the  pituitary  body.     Winckel  obj.  No.  5,  without  ocular. 
(From  the  collection  of  Dr.  A.  G.  Nicholls.) 

Secondary  tumors  may  involve  the  hypophysis.  Colloid  carcinoma 
and  melanosarcoma  have  been  met  with. 

In  addition  to  the  general  symptoms  of  brain-tumor,  growths  in  the 
pituitary  rather  early  involve  the  optic  tracts  and  other  cranial  nerves 
in  the  neighborhood,  so  that  blindness  and  various  local  paralyses  are 
met  with.  Pronounced  systemic  anemia  is  a  curious  symptom  occasion- 
ally found.  The  explanation  of  this  is  not  easy.  It  should  be  noted 
that  pituitary  tumors  may  exist  without  the  symptoms  of  acromegaly 
becoming  manifest.  In  such  cases  we  must  assume  either  that  the 
growth  has  progressed  so  rapidly  that  death  has  occurred  before  this 

1  See  James  Stewart,  The  Symptomatology  of  Tumors  Involving  the  Hypophysis. 
Cerebri,  Phila.  Med.  Jour.,  3:  1899:  1169. 


THE  CAROTID  GLAND  711 

disease  could  appear,  that  compensation  has  taken  place,  or  that  the 
intact  portion  of  the  gland  or  perhaps  the  tumor  itself  has  been  adequate 
to  supply  the  necessary  secretion. 


THE  PINEAL  GLAND. 

The  pineal  gland,  or  epiphysis,  is  formed  by  a  diverticulum  from  the 
roof  of  the  posterior  portion  of  the  anterior  cerebral  vesicle.  It  is  gener- 
ally believed  to  have  no  function,  but  is  the  atrophied  remnant  of  a  central 
eye,  which  is  somewhat  better  marked  in  certain  of  the  lower  animals. 

Histologically,  it  consists  of  a  connective-tissue  stroma,  in  which  are 
numerous  alveoli,  intersected  by  fine  trabeculse  and  filled  with  rounded 
cells,  often  possessing  delicate  processes.  The  follicles  also  contain 
considerable  "brain  sand."  The  organ  is  highly  vascular  and  contains 
a  plexus  of  sympathetic  nerve-fibers. 

The  epiphysis  is  congested  in  inflammatory  conditions. 

Hemorrhage  into  its  substance  may  occur  with  the  formation  of  a 
hematoma. 

Hyaline  degeneration  of  the  vessels  is  described. 

The  calcareous  matter  may  be  notably  increased  (psammoma)  and 
cysts  may  be  present. 

Hyperplasia  and  tumors  occur. 

Sarcoma1  is  the  most  important  form.    Dermoid  cysts  also  are  described. 


THE  CAROTID  GLAND. 

This  is  a  small  body,  about  1  to  3  mm.  in  diameter,  lying  in  the 
bifurcation  of  the  common  carotid  artery,  slightly  to  the  posterior  side 
of  the  internal  carotid  artery  just  as  it  leaves  the  main  trunk.  At  first, 
it  was  thought  to  be  of  epithelial  origin,  derived,  like  the  thyroid  and 
thymus  glands,  from  the  branchial  clefts.  This  is  now  known  to  be 
incorrect.  It  is  formed  from  the  primitive  vascular  anlage,  from  cells 
of  epithelioid  type,  which  become  heaped  up  at  this  point  and  at  first 
are  continuous  with  those  of  the  vessel-wall.  The  resulting  nodule  is 
enclosed  in  adventitia.  Soon,  capillary  vessels,  derived  from  the 
common  carotid,  enter  the  mass,  and  assume  a  form  not  unlike  a  glomer- 
ulus.  AYhen  complete,  the  gland  is  enclosed  in  a  fibrous  capsule  which 
sends  in  trabeculse,  dividing  it  into  lobules.  The  unit  of  structure  is  the 
"cell-ball,"  composed  of  a  tuft  of  capillaries  that  unite  to  form  a  vein. 
Several  of  these  cell-balls  are  included  in  a  lobule.  The  glomerule- 
like  tufts  are  surrounded  by  epithelioid  cells,  arranged  in  cords  or 
trabecuLne,  which  have  an  oval  nucleus  and  nucleolus  and  a  granular 
protoplasm.  Besides  this,  the  structure  contains  ganglion  cells  and 
sympathetic  fibers  derived  from  the  cervical  ganglion.  There  are  also/ 

1  Turner,  Spindle-celled  Sarcoma,  Trans.  Path.  Soc.  of  London,  1885. 


712 


THE  CAROTID  GLAND 


FIG.  188 


fc 


Tumor  of  the  carotid  gland;  Ge,  vessels;  Bl,  hemorrhage  into  a  column  of  cells;  at  d  the  cells  of 
the  growth  are  taking  on  a  more  connective-tissue  type;  at  c,  hyaline  degeneration. 

FIG.  189 


Portion  of  the  same  tumor  more  highly  magnified  to  show  peritheliomatous  arrangement 
of  the  tumor  cells  in  relationship  to  the  vascular  endothelium.     (Paltauf.) 


THE  COCCYGEAL  GLAND  713 

to  be  seen  certain  cells  possessing  the  same  affinity  for  chromic  acid 
(chromaffin  cells)  that  similar  cells  do  which  are  found  in  the  adrenals 
and  coccygeal  gland.  The  function  of  the  carotid  body  is  quite  un- 
known, but  it  is  undoubtedly  connected  with  the  sympathetic  nervous 
system. 

Very  little  is  written  about  the  disorders  of  this  curious  little  structure. 
Probably  some  of  them  escape  observation  on  account  of  its  small  size 
and  from  the  fact  that  it  is  but  rarely  examined.  Some  eight  cases  of 
tumors  have  been  described,  all  of  them  removed  by  operative  procedure. 

Histologically,  they  are  peritheliomas  and  are  highly  vascular.  They 
are  composed  of  capillaries,  about  which  are  layers  of  epithelioid  cells. 
The  lobular  arrangement  of  the  gland  is  retained.  So  far,  no  case 
has  been  recorded  in  which  there  was  recurrence  or  the  formation  of 
metastases.1 

THE  COCCYGEAL  GLAND. 

The  coccygeal  gland,  discovered  by  Luschka2  in  1860,  is  a  small 
body  about  the  size  of  a  pea,  situated  near  the  tip  of  the  coccyx  just 
above  the  coccygeal  attachment  of  the  sphincter  ani,  in  the  small  tendin- 

FIG.  190 


Section  of  a  perithelioma  of  Luschka's  or  the  coccygeal  gland.     (Von  Hleb-Koszanka.) 

ous  interval  formed  by  the  union  of  the  levator  muscles  of  the  anus. 
In  structure,  it  somewhat  resembles  the  carotid  body,  and  is  composed 
of  numerous  loops  of  bloodvessels,  anastomosing  freely  with  each  other, 

1  For  literature  see  H.  Gideon  Wells,  Ref.  Handbk.  Med.  Sci.,  8: 1904: 413. 

2  Die  Steisdruse  d.  Menschen.,  Virch.  Archiv,  18:  1860. 


714  THE  COCCYGEAL  GLAND 

derived  from  the  middle  sacral  artery.  These  are  enclosed  in  one  or 
more  layers  of  granular  polyhedral  cells,  sharply  differentiating  it  from 
the  dense,  fibrillar  connective  tissue  round  about.  The  whole  structure 
is  surrounded  by  a  connective-tissue  capsule,  which  sends  in  trabeculse 
dividing  the  interior  into  a  number  of  lobules.  Sympathetic  nerve 
and  muscle  fibers  have  also  been  demonstrated. 

Its  function  is  quite  obscure.  But  little,  also,  is  known  about  its 
pathology.  Luschka  thought  that  in  it  he  had  found  the  starting  point 
of  the  various  tumors  of  the  sacral  region.  Subsequent  study  has  shown, 
however,  that  the  majority  of  these  are  to  be  classed  as  spinae  bifidse, 
and  new-growths  derived  from  misplaced  "rests,"  or  teratomas,  and  have 
no  connection  whatever  with  the  coccygeal  gland.  From  its  structure, 
one  would  infer  a  priori  that  tumors  originating  in  this  organ  would 
assume  the  type  of  an  angiosarcoma  rather  than  that  of  the  forms  just 
mentioned.  Klebs  has  described  a  cystosarcoma,  Fausto-Buzzi1  an  angio- 
sarcoma, and  Schmidt2  a  teratoma,  apparently  arising  in  this  little  body. 


1  Virch.  Archiv,  109:  1887,  9.  2  Ibid.,  112:  1888:  372. 


SECTION   VI. 
THE  URINARY  SYSTEM. 


CHAPTER    XXXIV. 

THE  RENAL  FUNCTIONS  AND  THEIR  DISTURBANCES. 

As  a  basis  for  understanding  the  pathology  of  any  organ,  it  is  essential 
that  we  have  a  grasp  of  its  physiology.  Unfortunately  we  are  far  from 
being  assured  about  some  of  the  most  basal  matters  regarding  the  normal 
functions  of  the  kidneys,  and,  as  a  result,  there  are  many  moot  points 
in  connection  with  the  morbid  disturbances  in  those  organs  and  their 
significance.  Thus,  we  know,  from  a  study  of  the  liver,  that  some  glands 
possessing  excretory  ducts  may  perform  a  double  function — may  discharge 
an  internal  as  well  as  an  external  secretion,  both  being  essential  parts 
of  their  activity;  they  may  so  metabolize  the  material  brought  to  them 
by  the  blood  that  some  of  the  products  are  eliminated,  others  elaborated 
for  the  use  of  the  economy.  How  far  is  this  the  case  with  the  kidneys  ? 
It  is  difficult  to  say. 

Upon  general  grounds  it  may  be  laid  down  that  external  excretion  is 
the  all-important  function  of  these  organs.  Embryologically,  the  kidney 
of  the  higher  animal  is  the  homologue  of  the  segmental  ducts  of  the 
worms  and  other  lowlier  forms.  And  these  segmental  organs  are  tubes 
of  direct  communication  between  the  body  cavity  and  the  exterior. 
There  is  no  question  regarding  their  function;  they  are  primarily 
excretory,  to  discharge  the  excess  body-fluid,  nor  are  they  of  sufficient 
length  to  exercise  extensive  absorption  from  the  fluid  passing  through 
them.  Later,  we  find  a  stage  in  which  the  kidney  possesses  tubes 
having  both  a  funnel-shaped  orifice  into  the  body  cavity  and  glom- 
eruli.  Higher  up  again  in  the  scale  the  funnels  wholly  disappear, 
the  glomeruli  taking  their  place.  In  other  words,  with  the  development 
of  the  blood-vascular  system,  the  discharge  of  fluid  is  from  the  blood, 
and  not  from  the  body  cavity.  But  simultaneously  with  the  development 
of  glomeruli,  the  relative  length  of  the  urinary  tubules  undergoes  an 
extraordinary  increase.  What  is  the  meaning  of  this  ?  The  cells  lining 
these  tubules  assume  further  the  character  we  have  learned  to  associate 
with  secretory  cells;  they  do  not  form,  at  least  so  far  as  regards  their 
cortical  portions,  a  mere  inert  lining-epithelium.  How  do  they  act? 
Do  they  discharge  material  taken  from  the  abundant  surrounding  capil- 


716        THE  RENAL  FUNCTIONS  AND  THEIR  DISTURBANCES 

laries  into  the  lumen;  or,  on  the  contrary,  do  they  resorb  material 
useful  for  the  economy  which  has  been  flushed  out  of  the  blood  through 
the  glomeruli;  or,  thirdly,  do  they  accomplish  both  of  those  objects? 
Here,  on  general  principles,  we  are  forced  to  conclude  that  their  main 
function  is  excretory,  with  discharge  into  the  lumen  of  the  tubules — and 
that  because,  when  irritated  or  inflamed,  we  observe  that  it  is  the  portion 
of  the  cell  toward  the  lumen  that  undergoes  well-marked  breaking-down 
processes,  with  dissociation  and  discharge  of  its  substance  into  the 
lumen,  the  discharge  often  taking  the  form  of  delicate  fluid  vesicles. 
So  also  it  may,  we  think,  be  laid  down  as  a  broad  principle  that  where 
a  glandular  surface  has  an  absorptive  function,  there  as  large  and  not 
as  small  an  area  as  possible  is  provided.  The  stomach,  we  know, 
has  little  absorptive,  but  active  discharging  powers,  and  in  it  we  have 
abundant  long  tubules,  but  no  villi;  absorption  of  foodstuffs  is  at  its 
height  in  the  small  intestine,  and  there  we  find  abundant  villous  processes, 
affording  the  largest  possible  amount  of  surface.  The  structure  of  the 
renal  tubules  is  the  very  reverse,  the  largest  surface  is  exposed  to  the 
surrounding  lymph,  the  smallest  to  the  contained  urine.  The  arrange- 
ment indicates  that  absorption  from  that  lymph  and  discharge  into  the 
lumen  is  the  main  function. 

It  was  considerations  of  this  sort  that  led  Bowman,  in  1842,  to  pro- 
pound his  theory  that  the  glomerulus  "  furnishes  water  to  aid  in  the 
separation  of  the  urinous  products  from  the  epithelium  of  the  tube." 
Nor,  must  it  be  confessed,  have  we,  in  the  last  sixty  years,  advanced 
surely  very  much  farther.  Only  two  years  later  Ludwig  enunciated  the 
"mechanical  theory,"  to  the  effect  that  under  blood  pressure  a  dilute 
fluid  is  filtered  in  abundance  through  the  glomeruli,  and  that  the 
function  of  the  tubular  epithelium  is  to  concentrate  this  until  it  acquires 
the  normal  character  of  the  urine.  But,  as  pointed  out  by  Heidenhain 
and  his  pupils,  there  are  many  objections  to  this  theory.  We  shall 
not  enter  into  the  long-continued  contest,  or  describe  how,  up  to  the 
present  day,  the  battle  has  surged  to  and  fro.  For  ourselves  we 
cannot  accept  the  Ludwig  hypothesis,  if  only  because,  as  Heidenhain 
pointed  out,  so  small  is  the  amount  of  urea  in  the  blood  that  if  this  were 
removed  by  mere  filtration,  then  no  less  than  about  70  kilos  of  fluid 
would  have  daily  to  be  filtered  and  resorbed  by  the  tubules  to  explain 
the  amount  of  urea  present  in  the  day's  urine.  This  is  asking  altogether 
too  much.  Further,  we  have  the  fact,  curiously  neglected  by  the  ad- 
herents of  the  filtration  hypothesis,  of  the  existence  of  hydronephrosis, 
and  of  its  physiological  cause,  the  fact  that  the  pressure  under  which 
the  urine  is  discharged  into  the  pelvis  is  capable  of  being  greater  than  the 
blood  pressure;  there  is  no  escape  from  the  conclusion  that  primarily 
this  indicates  an  active  excretory  process.  All  the  same,  we  have  to 
admit  that  it  is  difficult  to  bring  forward  absolute  evidence  in  favor  of 
the  secretory  hypothesis.  Heidenhain  and  others  have  attempted  to 
prove  the  matter  by  demonstrating  the  passage  into  and  through  the  cells 
of  the  convoluted  tubules  of  indigo-carmine  and  other  substances 
which  are  recognizable  under  the  microscope,  and  in  this  and  other 


OBSERVATIONS  OF  HEIDENHAIN  AND  OTHERS  717 

ways,  there  has  been  accumulated  a  fair  amount  of  evidence  that  the 
different  regions  of  the  renal  tubules  subserve  different  functions.  But 
admittedly,  it  is  difficult  to  obtain  preparations  by  these  means  that  are 
absolutely  convincing  and  not  capable  of  two  interpretations;  nay,  with 
ordinary  carmine,  the  bulk  of  evidence  lies  in  the  other  direction. 

Dreser's  observations  and  the  conclusions  he  has  drawrn  from  them 
present  the  same  ambiguity.  We  know  that  the  blood  and  lymph  are 
alkaline  in  reaction,  the  normal  urine  in  general  acid.  Dreser  em- 
ployed acid  fuchsin  as  an  indicator.  This  in  acid  solutions  is  of  brilliant 
red  color;  almost  colorless  in  weak  alkaline  solutions.  Injecting  the  dye 
into  the  dorsal  lymph-sac  of  a  frog,  the  urine  excreted  in  the  course  of 
an  hour  or  two  is  of  a  brilliant  red  color.  And  now  the  glomerular 
regions  of  the  kidneys  on  section  were  found  to  be  almost  colorless,  i.  e., 
the  discharge  through  the  glomeruli  was  alkaline,  the  tubules  below  were 
filled  with  a  bright  red  fluid.  Here  it  will  be  seen  there  are  two  possi- 
bilities— either  that  the  cells  of  the  tubules  excrete  acid  bodies,  or  that 
they  absorb  those  bodies  which  give  to  the  lymph  its  alkaline  reaction. 
Were  the  latter  the  case,  the  cells  of  the  tubules  should  be  colorless.  This 
they  were,  as  a  matter  of  fact,  in  the  early  stages  of  such  experiments, 
but  on  repeating  the  injection,  they  assumed  the  red  stain.  On  the  whole, 
this  staining  must  be  regarded  as  favoring  the  secretion  hypothesis. 
There  is,  however,  indubitable  evidence  from  GurwitchV  experiments, 
that  in  the  frog  the  convoluted  tubules  excrete  pigment  matter.  In  that 
animal  there  is  a  separate  blood  supply  for  the  glomeruli  and  the  tubules 
respectively.  If  the  tubular  blood  supply  (through  the  renal  portal 
vein)  be  cut  off,  no  pigment  appears  in  the  urine,  although  the  pigment 
has  free  access  to  the  glomeruli.  The  glomeruli  and  the  cells  of  the 
convoluted  tubules  in  the  frog  have  the  same  general  architecture  as  those 
in  man,  and  this  to  a  striking  degree,  and  it  would  seem  that  if  excretion 
occurs  through  the  epithelium  in  the  one  animal,  it  must  be  the  same  in 
the  other.  Easier,  by  forcing  pigment  into  the  tubules  from  the  ureter 
found  no  absorption,  although  under  similar  conditions  sugar  and  ferro- 
cyanide  solutions  were  absorbed  and  appeared  in  the  urine  of  the  other 
kidney.  What  is,  however,  most  in  favor  of  the  secretion  hypothesis  is 
a  consideration  of  the  metabolic  and  constructive  processes  undergone 
in  the  kidney.  One  of  these  has  been  known  for  long.  Hippuric  acid 
is  one  of  the  normal  constituents  of  the  urine;  it  can  be  synthesized  and 
excreted  by  the  passage  of  glycine  and  benzoic  acid  through  the  vessels 
of  the  kidney.  The  conversion  is  not  found  to  take  place  in  other  organs ; 
it  is  a  function  of  the  kidney  tissue;  it  is  inconceivable  that  it  occurs  in 
the  glomeruli ;  we  must  conclude  that  it  is  brought  about  by  the  epithelium 
of  the  tubules.  Uric  acid,  again,  is  a  most  insoluble  substance;  the  abun- 
dance of  the  same  in  the  urine  of  birds  is  such  that  we  cannot  believe 
that  it  passes  out  of  the  glomeruli  in  that  form.  In  fact,  we  have  indica- 
tions that  it  is  not  present  in  the  blood  as  the  acid,  but  as  a  more  soluble 
sodium  salt;  while  the  researches  of  the  late  Sir  William  Roberts  indicate 

1  Pfliiger's  Arch.  f.  d.  Ges,  Physiol.,  91 : 1902  :  71. 


718       THE  RENAL  FUNCTIONS  AND  THEIR  DISTURBANCES 

that  even  in  the  renal  tubules  of  the  bird — and  of  man — it  is  first  dis- 
charged as  a  compound  salt,  the  "quadriurate,"  which  undergoes  disso- 
ciation with  liberation  and  deposition  of  the  uric  acid.  It  is  further  note- 
worthy that  uric  acid  introduced  into  the  human  economy  appears  largely 
in  the  urine  as  urea,  while,  contrariwise,  urea  fed  to  birds  reappears  as 
uric  acid.  When,  further,  as  pointed  out  by  Gowland  Hopkins,  we 
observe  that  the  renal  excretives  are,  as  a  class,  more  complex  or  less 
stable  than  their  immediate  precursors  in  the  body,  it  is  difficult  not  to 
conclude  that  the  terminal  steps  of  nitrogenous  metabolism,  whereby 
urea,  uric  acid,  hippuric  acid,  creatinin  and  other  bodies  appear  in  the 
urine,  are  very  largely  controlled  by  the  renal  epithelium. 

The  Vascular  Supply  of  the  Kidney.— The  distribution  of  the 
blood  in  the  kidney  is  not  a  little  remarkable,  and  possibly  throws 
some  light  upon  the  mechanics  of  urinary  excretion.  In  the  first  place 
the  arterial  supply  of  the  cortex  passes  almost  entirely  to  the  glomeruli. 
A  study  of  injected  specimens  shows  that,  with  rare  and  inconsiderable 
exceptions,  the  interlobular  branches  of  the  cortical  arteries  pass  to  the 
glomeruli,  constituting  the  afferent  arterioles.  The  efferent  vessels  from 
the  glomeruli  break  up  into  an  intricate  meshwork  of  capillaries  around 
the  convoluted  tubules.  On  the  other  hand,  in  the  medulla  the  blood 
supply  of  the  collecting  tubules  is  by  a  capillary  network  proceeding 
directly  from  the  arterise  recta?.  All  are  agreed  that  the  main  bulk 
of  the  water  in  the  kidney  passes  out  of  the  delicate  walls  of  the  glomeru- 
lar  tuft.  Cut  off  the  glomerular  blood  supply,  as  in  Nussbaum's  well- 
known  experiment  upon  the  frog  (which  has  a  double  blood  supply 
through  the  renal  arteries  supplying  the  glomeruli,  and  the  renal  portal 
vein  supplying  the  tubules),  and  the  secretion  of  urine  immediately  falls 
to  almost  nil.  It  follows,  therefore,  that  the  blood  circulating  in  the 
cortical  capillaries,  in  having  already  passed  through  one  capillary  system 
(that  of  the  glomeruli),  is  both  more  concentrated  and  under  a  lower 
pressure  than  that  supplying  the  collecting  tubules  in  the  medulla. 
With  a  dilute  urine,  that  is,  in  the  convoluted  tubules,  and  a  low  external 
blood  pressure,  we  can  imagine  a  resorption  or  reverse  filtration  of  the 
urinary  water  from  the  tubules.  These  considerations  would  seem  to 
favor  Ludwig's  theory,  but  the  same  line  of  thought  would  suggest  that 
if  this  theory  be  accepted,  when  the  urine  reaches  the  collecting  tubules 
its  greater  concentration,  and  the 'higher  capillary  blood  pressure  should 
favor  additional  discharge  of  water  from  the  blood  into  the  tubules. 

That  the  discharge  of  urine  is  largely  determined  by  circulatory  condi- 
tions must  be  clearly  accepted;  reduce  the  general  blood  pressure  by  any 
means  to  40  mm.  Hg.  or  below,  and  the  flow  of  urine  ceases;  increase 
the  amount  of  blood  circulating  through  the  organ,  as  by  ligaturing  the 
arteries  passing  to  important  areas  of  the  body,  and  the  flow  is  greatly 
increased.  Ludwig  laid  down  that  it  is  the  blood  pressure  that  mechan- 
ically determines  the  flow.  Heidenhain,  on  the  other  hand,  pointed 
out  that  if  the  renal  vein  be  ligated,  the  kidney  becomes  enormously  con- 
gested, the  blood  pressure  within  it  is  greatly  increased,  and  nevertheless 
there  is  a  complete  stoppage  of  urinary  flow.  It  is,  he  postulated,  the 


THE  NERVE  SUPPLY  719 

rate  of  blood  flow  through  rather  than  the  blood  pressure  in  the  glomeruli 
that  determines  the  discharge.  He  concluded  that  with  arrest  of  blood 
flow,  the  glomerular  epithelium  becomes  asphyxiated,  swollen  and 
unable  to  function.  Ludwig,  on  the  other  hand,  explained  the  results 
as  due  to  the  intense  capillary  congestion  causing  compression  and  ob- 
struction of  the  urinary  tubules.  The  rapid  resumption  of  urinary 
discharge  in  the  inflamed  and  congested  kidney  which  follows  Edebohls' 
operation  of  decortication  of  the  kidney,  or  excision  of  its  capsule, 
favors  Ludwig's  explanation;  but,  on  the  other  hand,  it  deserves  note 
that  the  trend  of  modern  work,  as  represented  by  the  studies  of  F.  Miiller 
and  Marchand  and  his  school,  is  to  ascribe  the  oliguria  of  acute  nephritis 
more  and  more  to  glomerular  lesions  rather  than  to  mechanical  disturb- 
ances of  the  circulation. 

From  the  above  data  it  will  be  seen  how  difficult  it  is  at  the  present 
time  to  reach  any  precise  conclusion  regarding  the  nature  of  the  urinary 
discharge.  On  the  whole,  we  conclude  that  while  the  water  of  the  urine 
is  in  the  main  discharged  through  the  glomeruli,  along  with  sundry 
simple  soluble  salts  and  other  substances  (peptone,  grape-sugar),  that  dis- 
charge is  not  a  simple,  but  a  selective  filtration,  and  that  while  in  the 
passage  down  the  tubules  there  may  be  a  resorption  of  certain  constitu- 
ents of  the  discharge,  this  resorption  is  of  secondary  importance  com- 
pared with  the  active  excretion  of  such  substances  as  urea,  uric  acid, 
and  other  "extractives,"  bodies  of  the  nature  of  toxins,  coupled,  it 
would  seem,  with  active  anabolic  processes  to  form  bodies  like  hippuric 
acid. 

The  Nerve  Supply. — The  nerves  passing  to  the  kidney  form  a 
fine  plexus  surrounding  the  renal  artery.  Various  experiments  have 
shown  that  these  nerves  are  very  largely  vasomotor  in  function,  although 
Berkeley,  in  his  admirable  studies  of  the  terminal  distribution  of  the  nerve 
filaments  within  the  organ,  by  means  of  Golgi's  method,  has  demonstrated 
the  existence  of  a  wide  network  of  filaments  throughout  the  cortex  and 
medulla  with  end  knobs  upon  the  Bowman's  capsules  and  other  termina- 
tions penetrating  the  membrana  propria  of  the  convoluted  tubules,  an 
arrangement  which  suggests  strongly  that  these  nerve-filaments  are 
secretory  in  function.  It  is  the  vasomotor  effects  that  have  been  most 
studied. 

Section  of  the  spinal  cord  in  the  cervical  region,  by  removing  the  in- 
fluence of  the  main  medullary  vasomotor  centres,  leads  to  a  diffuse 
dilatation  of  the  arteries  of  the  trunk,  and  lowering  of  the  blood  pressure 
to  40  mm.  Hg.  or  under,  with  which  the  urinary  discharge  is  completely 
arrested.  The  renal  vessels,  along  with  the  others,  are  relaxed,  but  at 
the  same  time  the  blood  flow  is  so  much  diminished  that  the  organ  lessens 
in  size.  If,  after  such  section  the  renal  nerves  be  divided,  and  now  the 
distal  cut  end  of  the  cord  be  stimulated,  there  is  pronounced  general 
rise  of  blood  pressure  and  resumption  of  the  urinary  flow.  Such  section 
of  the  renal  nerves  leads  to  vasomotor  paralysis  in  the  organ,  and  if 
practised  alone,  is  also  followed  by  increased  flow  of  urine,  in  consequence 
of  the  augmented  blood  flow  through  the  organ.  Stimulation  of  the 


720       THE  RENAL  FUNCTIONS  AND  THEIR  DISTURBANCES 

renal  nerves,  on  the  contrary,  causes  contraction  of  the  organ  and  its 
vessels,  and  diminished  urinary  flow.  The  observations  here  are  similar 
to  what  is  observed  with  organs  and  vessels  in  general,  namely,  that 
disturbance  of  the  tonic  vasoconstrictor  nerves  may  be  demonstrated 
with  fair  ease.  That  vasodilators  also  exist  has  been  shown  by  Brad- 
ford; appropriate  stimulation  of  the  anterior  roots  of  the  eleventh, 
twelfth  and  thirteenth  dorsal  nerves  leads  to  definite  expansion  and  con- 
gestion of  the  kidney  without  alteration  in  the  general  blood  pressure. 
As  Starling1  points  out,  it  is  extremely  probable  that  such  vasodilator 
stimulation  is  the  cause  of  the  extreme  hydruria  encountered  in  hysteria 
and  other  nervous  affections — and  we  may  add,  of  the  polyuria  of  emo- 
tional states.  Similarly  the  anuria  following  catheterization  is  best 
ascribed  to  reflex  extreme  vasoconstrictor  effects. 

Recognizing,  thus,  the  profound  influence  that  the  nervous  system 
has  upon  the  amount  of  the  urinary  discharge,  it  may  be  asked  what  is 
the  mechanism  whereby  that  discharge  is  controlled.  The  only  satis- 
factory answer  at  the  present  time  is  that  the  amount  of  urine  excreted 
is  primarily  dependent  upon  the  glomerular  blood  supply,  and  this, 
in  its  turn,  depends  upon  the  tonus  of  the  interlobular  arteries.  It  is 
the  relative  contraction  or  dilatation  of  these  arteries  that  determines 
the  amount  of  blood  entering  the  glomerular  system.  Histologically, 
a  striking  feature  of  the  arteries  in  question  is  the  good  development  of 
their  muscular  coat.  Berkeley  was  unable  to  find  any  nerve-filaments 
passing  into  the  glomerular  loops;  or  otherwise,  we  have  no  evidence 
of  independent  contraction  and  expansion  of  the  glomeruli;  these  are 
passive. 

It  is,  to  repeat,  the  afferent  vessels  that  primarily  determine  the  blood 
supply,  and  so  the  extent  of  excretion  of  the  urinary  fluid.  Only 
secondarily  the  constituents  of  the  blood  as  they  act  upon  the  capillary 
walls  in  the  glomeruli  may  influence  their  filtration  capacity.  As  with 
the  arterioles  in  general  (pp.  25  and  26),  we  have  to  recognize  that  con- 
traction and  expansion  of  these  arterioles  may  be  brought  about  either 
by  central  nervous  influence,  or  directly  by  the  action  of  substances 
diffusing  out  from  the  circulating  blood. 

The  Relationship  between  Circulatory  Disturbances  and  Chronic 
Interstitial  Nephritis. — Where  there  has  been  a  history  of  long- 
continued  rise  of  general  blood  pressure,  there  we  are  apt  to  find 
the  muscular  coat  of  the  cortical  arteries  hypertrophied — an  indication 
of  continued  increased  functional  activity,  and,  whether  from  extreme 
contraction  or  from  the  later  condition  of  "  endarteritis  obliterans/' 
certain  glomeruli  undergo  hyaline  degeneration  and  become  completely 
impervious.  These  now  are  represented  as  solid,  shrunken,  transparent 
bodies,  and  are  a  characteristic  feature  in  the  contracted  areas  of  the 
granular  contracted  kidney  of  chronic  interstitial  nephritis.  The  sim- 
plest explanation  of  this  hyaline  degeneration  is  that  it  is  a  necrobiotic 
change  induced  by  the  progressive  diminution  of  the  blood  supply, 

1  Schafer's  Physiology,  1 : 1898 :  646. 


CONCLUSIONS  721 

through  arteriolar  contraction  and  obliteration,  preceded  by  a  swollen 
state  of  the  glomerular  epithelium.  It  is  to  be  noted  that  where  certain 
glomeruli  show  this  degeneration,  others  in  their  neighborhood  exhibit 
the  condition  (of  compensatory)  hypertrophy,  and  may  be  of  twice  the 
normal  diameter,  with  strikingly  large  capillary  loops;  and  these  it  would 
seem  are  more  pervious  than  normal,  for  in  this  condition  of  chronic 
interstitial  nephritis,  instead  of  there  being  a  diminished  excretion  of 
urine,  there  is  apt  to  be,  on  the  contrary,  an  increased  passing  out  of 
thin,  watery  urine  of  low  specific  gravity,  containing  a  small  amount 
of  albumin,  or,  otherwise,  these  distended  glomeruli  permit  an  abun- 
dant discharge  of  fluid  through  their  thinned  walls,  and  with  this,  some 
escape  of  albumin  from  the  blood  plasma.  Friedrich  Muller  is  of 
opinion  that  this  increased  discharge  is,  in  part,  due  to  a  modification 
in  function  of  the  epithelium  of  certain  tubules.  That  epithelium 
becomes  flattened  and  endothelial  in  type.  This  indicates,  he  holds, 
that  now  it  permits  the  freer  passage  of  fluid.1 

Vascular  Disturbances  and  their  Relationship  to  Chronic  Inter- 
stitial Nephritis. — We  shall,  in  the  ensuing  chapter,  describe  the 
different  forms  of  the  contracted  kidney — the  postinflammatory,  follow- 
ing upon  an  acute  nephritis,  the  atrophic  or  senile,  and  the  arterio- 
sclerotic.  It  is  this  last,  and  the  form  allied  to  it,  that  we  here  refer  to, 
pointing  out  that  a  similar  sequence  of  changes  occur  in  the  cortex 
when  there  is  intimal  overgrowth  with  obliteration  of  the  vascular  lumen, 
and  when,  without  such  overgrowth,  hypertrophy  of  the  media  and  con- 
traction of  the  arterioles  lead  to  degeneration  of  certain  of  the  glomeruli. 
In  either  case,  if  the  glomerular  supply  be  cut  off,  the  capillary  network 
connected  with  the  efferent  vein  of  the  affected  glomeruli  (1)  receive 
no  urine,  and  (2)  have  an  impoverished  surrounding  blood  supply.  As 
a  result,  they  undergo  atrophy,  and  shrink  until  they  are  represented  by 
columns  of  small  cells  with  a  scarcely  visible  lumen,  and  as  they  shrink 
there  is  some  compensatory  overgrowth  of  the  surrounding  interstitial 
connective  tissue.  Thus,  as  not  all  the  interlobular  arteries  and  their 
branches  are  similarly  affected,  we  find  areas  of  hyaline  glomeruli  and 
shrunken  tubules  with  interstitial  fibrosis  alternating  with  other  areas 
of  distended  glomeruli  and  large  tubules  with  large  lumina.  This  is 
the  commonest  type  of  so-called  chronic  interstitial  nephritis,  a  con- 
tracted granular  kidney,  and,  we  would  emphasize,  in  this  the  primary 
lesion  would  seem  to  be  arterial. 

Conclusions. — To  sum  up,  it  would  seem  that  the  quantity  of  urine 
discharged  depends  directly  upon  the  quantity  of  blood  flowing  through 
the  glomeruli.  This  blood  flow  depends  in  the  first  place  upon  the 
difference  between  the  pressure  in  the  renal  artery,  and  that  in  the  renal 
vein.  If  the  arterial  pressure  be  increased  without  increase  in  the 
venous,  then  the  flow  is  greater,  and  the  urinary  excretion  increases; 
if,  on  the  contrary,  through  local  obstruction  or  cardiac  incompetence, 
the  pressure  in  the  renal  vein  be  raised  without  corresponding  rise  in  the 

Verhandl.  d.  Deutsch.  pathol.  Gesellsch.,  Meran,  9: 1905:  73. 
46 


722        THE  RENAL  FUNCTIONS  AND   THEIR  DISTURBANCES 

arterial  pressure,  the  urinary  excretion  is  diminished.  At  the  same  time 
the  size  of  the  arterial  channels  in  the  kidney  has  to  be  taken  into  account; 
not  only  is  there  increased  excretion  when  the  general  arterial  pressure  is 
raised,  but  without  rise  of  this  general  pressure,  if  the  interlobular  and 
afferent  arteries  become  dilated,  there  is  increased  blood  flow  and 
increased  excretion,  while,  contrariwise,  if  without  alteration  of  the 
general  blood  pressure  the  afferent  arterioles  undergo  contraction,  the 
glomerular  circulation  is  diminished,  and  with  this  the  urinary  excretion 
falls. 

Polyuiia. — According  to  these  general  principles,  polyuria  and  in- 
creased excretion  of  urine  may  be  brought  about  (1)  by  increase  in  the 
general  arterial  pressure  without  renal  change,  or  (2)  dilatation  of  the 
cortical  arterioles  without,  of  necessity,  any  rise  in  the  general  blood 
pressure.  The  condition  of  diabetes  insipidus  would  seem  to  come  under 
the  second  category.  Here  we  encounter  an  excessive  discharge  of  a 
thin,  watery  urine  free  from  sugar.  There  is  a  tendency  toward  increased 
excretion  of  urea  (in  the  twenty-four  hours),  although  this  is  attributed 
to  the  increased  consumption  of  food  which,  as  in  diabetes  mellitus,  is 
often  present.  A  marked  feature  of  this  disturbance  is  the  frequent 
presence  of  inosite  in  the  urine.  Inosite,  C6H12O6,  is  a  benzene  derivative 
found  in  muscle,  liver,  and  other  organs,  and,  it  may  be  added,  seen 
also  in  the  urine  of  other  pathological  conditions.1  The  condition  has 
often  been  noted  as  affecting  several  members  of  the  same  family.  In 
other  cases  brain  lesions  have  been  present,  affecting  the  pons,  cerebellum, 
or  medulla.  This  suggests  that  disturbance  of  the  vasomotor  centres  in 
the  medulla  may  play  a  role.  Experimentally,  as  shown  by  Claude 
Bernard,  a  similar  polyuria  may  be  brought  about  by  injury  to  the 
medulla,  and,  as  already  noted,  transient  polyuria,  evidently  of  the  same 
order,  is  seen  in  hysterical  and  emotional  states. 

With  this  increased  discharge  there  is,  broadly  speaking,  a  correspond- 
ing decrease  in  the  total  solids  of  the  urine;  the  solids,  that  is  to  say, 
are  reduced,  although  the  reduction  of  the  different  constituents  is  not 
parallel,  and  individual  cases  show  variation  in  its  extent.  The  total 
amount  of  sodium  chloride,  for  example,  is  independent  of  the  amount 
of  urine,  suggesting  that  the  escape  of  the  salt  is  governed,  and  is  not 
a  mere  act  of  filtration. 

These  considerations  lead  to  a  reference  to  the  action  of  diuretics, 
and  the  recognition  of  a  very  possible  third  factor  in  the  production  of 
polyuria.  Of  such  diuretics  there  are  two  groups:  those  inducing 
heightened  blood  pressure  plus  improved  circulation  through  the  kid- 
ney, of  which  digitalis  is  an  example;  and  those  having  little  or  no  effect 
upon  the  general  blood  pressure,  among  which  are  to  be  included  the 
soluble  crystalloid  substances — dextrose,  urea,  sodium  chloride,  and  other 
saline  diuretics.  Regarding  the  former  there  is  this  to  be  noted,  that 
drugs  which  cause  heightened  blood  pressure  accomplish  this  by  con- 
traction of  the  arterioles,  and  thus  we  must  conclude  either  that  the 

\  See  Meillere,  Inosurie,  Paris,  1906. 


OLIGURIA   AND  ANURIA  723 

diuretic  members  of  this  group  have  a  specific  lack  of  action  upon  the 
renal  vessels,  or  that  the  rise  in  blood  pressure  more  than  compensates 
the  contraction  of  the  renal  vessels.  As  a  matter  of  fact,  the  drug  that 
causes  the  most  extreme  arteriolar  contraction — adrenalin — materially 
reduces  the  flow  of  urine,  and  digitalis  is  often  without  diuretic  effect  upon 
individuals  in  sound  health.  As  regards  the  latter,  it  is  still  a  matter  of 
debate  as  to  whether  they  act  by  local  specific  dilatation  of  the  renal 
vessels  (for  the  kidneys  exhibit  distinct  enlargement),  or  directly  stimulate 
the  renal  cells,  their  discharge  through  these  being  accompanied  by  an 
amount  of  water  necessary  to  retain  them  in  a  state  of  solution.  It  has 
been  urged  that  all  these  salts  abstract  water  from  the  tissues,  render  the 
blood  more  hydremic,  and  so  favor  increased  filtration;  the  fact  that 
these  salts  initiate  excretion  when  added  to  the  blood  perfused  through 
the  extirpated  kidney  supports  the  other  evidence  we  possess  that  these 
salts  are  largely  discharged  through  the  tubular  epithelium,  that  they 
exercise  a  direct  secretory  influence. 

Oliguria  and  Anuria. — Along  similar  lines  it  is  to  be  laid  down  that 
reduction  or  suppression  of  urinary  flow  can  be  brought  about  (1)  by 
direct  contraction  of  the  afferent  arterioles  (as  through  the  action  of 
adrenalin),  (2)  by  a  lowering  of  the  arterial  pressure,  or  (3)  a  rise  in 
the  venous  pressure.  These  last  two  may  be  combined  and  expressed 
by  saying  that  a  reduction  in  the  difference  between  arterial  and  venous 
pressure  leads  to  oliguria.  Examples  of  lowered  arterial  pressure  have 
already  been  given ;  of  raised  venous  pressure,  the  oliguria  accompanying 
obstructive  heart  disease  is  the  commonest  example.  A  fourth  cause 
needs  to  be  taken  into  consideration,  namely,  obstruction  to  the  out- 
flow, provided  that  this  affects  both  kidneys.  Such  obstruction  may 
occur  within  the  renal  tubules  through  blockage  of  the  same  with  in- 
spissated excretion,  as  in  renal  hematuria  and  hemoglobinuria,  or 
along  the  course  of  the  ureters  and  passages  of  discharge. 

As  we  have  stated,  there  is  still  debate  as  to  how  far  the  oliguria  of  acute 
nephritis  is  due  to  swelling  of  the  tubular  epithelium  and  obliteration 
of  the  lumen  of  the  tubules  as  a  result  of  congestion ;  how  far  it  is  due  to 
glomerular  disturbance.  At  the  Meran  meeting  of  the  German  Patho- 
logical Society,  Friedrich  Miiller1  laid  down  very  precisely  that  the 
oliguria  of  acute  nephritis  is  correlated  to  the  extent  of  glomerulonephritis 
found  present.  The  acute  swelling  of  the  glomerular  epithelium  fol- 
lowed by  proliferation  of  the  same,  arrests  the  function  of  these  organs. 
Lohlein,2  from  Marchand's  laboratory,  has  made  an  extended  study  of 
the  glomerular  changes  in  different  forms  of  nephritis,  and  concludes 
that  two  types  of  acute  nephritis  are  to  be  determined:  (1)  in  which  the 
tubular  epithelium  alone  is  involved  (kidneys  of  cholera  and  diphtheria, 
of  sublimate  poisoning,  and  many  kidneys  of  pregnancy;  such  kidneys, 
owing  to  the  great  regenerative  power  of  the  tubular  epithelium,  may 

1  Verhandl.  d.  Deutsch.  Pathol.  Gesellsch.,  9:  1905:  64. 

2  Ueber    die    entziindlichen    Veranderungen    der    Glomeruli    der    menschlichen 
Nieren,   Leipzig,   Hirgel,   1907. 


724       THE  RENAL  FUNCTIONS  AND  THEIR  DISTURBANCES 

undergo  rapid  and  complete  healing);  and  (2)  what  he  would  term 
acute  nephritis  proper,  in  which  the  glomeruli  are  characteristically 
involved.  Of  this  the  classical  example  is  the  scarlatinal  kidney.  Here 
the  extent  of  the  tubular  change  appears  to  depend  upon  the  severity 
of  the  glomerular  change.  Of  chronic  forms  of  the  disease,  he  lays 
down  that  chronic  parenchymatous  nephritis  (chronic  nephritis  with 
dropsy)  is  a  later  stage  of  unhealed  glomerulonephritis,  or  a  septic 
glomerulonephritis  of  insidious  origin.  So  also*  the  "  secondary  con- 
tracted kidney" — the  contracted  kidney  following  upon  acute  nephritis — 
shows  regularly  changes  in  the  glomeruli,  exhibiting  transition  stages 
to  those  seen  in  the  acute  disease. 

Hydronephrosis. — Obstruction  to  the  outflow,  leading  to  the  accu- 
mulation of  urine  in  the  pelvis  or  pelvis  and  ureters,  if  complete,  leads  to 
atrophy  of  the  affected  kidney;  if  incomplete  and  intermittent,  induces 
the  condition  of  hydronephrosis.  It  is  now  generally  accepted  that  a 
pressure  of  60  mm.  Hg.  within  the  renal  pelvis  causes  complete  arrest  of 
urinary  excretion,  and  if  continued,  so  compresses  the  renal  capillaries 
that  now  the  epithelium  undergoes  atrophy.  If  continued,  the  atrophy 
is  rapid.  If  some  escape  of  urine  be  possible,  with  intermittent  increased 
pressure,  there  is  dilatation  of  the  pelvis  and  more  gradual  compression 
and  atrophy  of  the  kidney  substance,  which  may  continue  until  the 
kidney  is  represented  by  a  multisacculated  cyst  of  great  size,  the  walls  of 
which,  but  a  millimeter  or  two  in  thickness,  still  contain  indications  of 
atrophied  tubules  and  glomeruli. 

Qualitative  Changes  in  the  Urine. — There  may  be  pronounced 
variations  in  the  relative  amounts  of  the  normal  constituents  of  the  urine 
— the  urea,  uric  acid,  phosphates,  chlorides,  and  other  salts,  and  the 
urinary  extractives,  kreatinin,  etc.,  and  the  pigments  or  urochrome;  as, 
again,  bodies  not  present  in  the  normal  urine,  or  present  in  almost  infini- 
tesimal quantities,  may  be  discharged  in  notable  amounts.  Among  these 
are  various  proteins,  more  particularly  serum  albumin,  serum  globulin, 
hemoglobin,  albumoses  and  nucleoproteins,  dextrose,  lactose  and  levu- 
lose,  pentose  acetone,  diacetic  and  oxybutyric  acids,  homogentisic  and 
uroleucic  acids  (alkapton),  cystin,  indican,  phenol  and  other  aromatic 
compounds,  bile  pigments,  foodstuffs  and  drugs  absorbed  from  the 
alimentary  canal  and  discharged  without  modification  (egg-albumin 
when  taken  in  excess,  methylene  blue,  etc.),  erythrocytes,  leukocytes, 
etc.  In  our  first  volume  we  have  discussed  the  greater  number  of  these 
and  their  significance.  Here  we  must  only  note  those  whose  presence 
in  the  urine  is  associated  with  gross  disturbance  in  the  renal  mechanism. 
!  Albuminuria. — Various  proteins  may  appear  in  the  urine — un- 
changed from  absorbed  food,  as  egg-albumin  after  an  excessive  diet  of 
raw  eggs,  mucins  in  inflammatory  or  degenerative  states  of  the  kidneys, 
nucleo-albumin  and  nucleinic  acids  from  disintegration  of  the  renal 
parenchyma  (although  these  bodies  may  also  be  derived  from  the  urinary 
passages  and  bladder).  But  these  are  inconsiderable  in  amount  and 
frequency  of  appearance,  as  compared  with  serum  albumin  and  the 
serum  globulins.  It  is  with  these  latter  bodies  that  we  are  specially 


PLATE  VII 


jr   ™ „ 

:  »-« v    ***>® 

^L    ^®  s" "A    -^« 

f*J5»       ft^-^^* 


Acute   Glomerulonephritis,  Scarlatinal,  of  about  the 
Twenty-first  Day. 

Portion  of  an  affected  glomerulus.    The  glomerular  loops  are  plump,  with 
an  increased,  number  of  nuclei. 


BL,  blood  within  the  capillaries,  which  exhibit  also  endothelial  proliferation  and  contain  some 
leukocytes.  G.  E.,  swollen  and  proliferated  glomerular  epithelium,  showing  some  desquamation.  P.,  capsular 
proliferation  with  adhesion  in  part  of  glomerular  epithelium  to  the  capsule  in  regions  devoid  of  capsular 
epithelium.  (After  Lohlein.) 


QUALITATIVE  CHANGES  IN  THE  URINE  725 

concerned  when  we  speak  of  albuminuria.  They  are  the  dominant 
proteins  of  the  blood  plasma,  hence  their  presence  in  the  urine  in  the 
main  indicates  an  abnormal  escape  of  these  colloidal  constituents  of 
the  fluid  of  the  blood. 

This,  in  the  first  place,  may  be  laid  down  with  precision;  unlike  the 
ordinary  capillaries,  the  glomerular  loops  do  not  normally  permit  the 
escape  of  the  proteins  of  the  plasma.  Take,  on  the  one  hand,  a  per- 
fectly healthy  kidney  immediately  after  death;  cut  off  small  pieces  of  the 
cortex,  and  plunge  into  boiling  water  or  a  solution  of  corrosive  sublimate, 
and  make  sections;  the  capsule  chambers  of  the  Malpighian  bodies  are 
found  perfectly  free  from  any  coagulum.  Take,  on  the  other  hand, 
the  kidney  of  an  animal  in  which  by  one  or  other  means  albuminuria 
has  been  induced;  repeat  the  process,  and  now  in  many  of  the  capsule 
chambers  larger  or  smaller  menisci  of  coagulated  albumin  are  to  be 
seen.  It  is  clear  from  this,  in  the  first  place,  that  albumin  does  not 
normally  filter  through  the  glomeruli  to  be  subsequently  resorbed  in 
its  passage  down  the  tubes;  and  in  the  second,  that,  when  albuminuria 
develops,  the  main  discharge  is  through  the  delicate  walls  of  the  glomer- 
ular tuft.  In  the  frog,  and  even  in  much  higher  animals,  the  presence 
of  long  cilia  in  the  neck  or  first  part  of  the  renal  tubule  hinders  regurgita- 
tion  of  urine  from  the  tubule  into  the  capsule  chamber;  this  albumin, 
therefore,  cannot  have  been  excreted  in  the  first  place  into  the  convoluted 
tubules. 

Is  this  the  only  source  of  the  albumin?  Probably  not.  It  is  inher- 
ently probable,  that  is,  that  in  acute  nephritis,  with  the  active  disintegra- 
tion of  the  tubular  epithelium  there  occurring,  the  products  of  the  broken- 
down  cells  swell  the  amount  of  albuminous  bodies  present  in  the  urine. 
So,  also,  when  the  tubular  epithelium  becomes  so  disorganized  that  it  is 
cast  off  and  the  naked  basement  membrane  alone  left,  albumin-containing 
lymph  may  exude  into  the  damaged  tubules;  and  thus  in  acute  nephritis 
with  diminished  urine,  and  that  so  full  of  albumin  that  in  heating  it 
clots  into  a  solid  mass,  it  may  well  be  that  the  tubules  as  distinct  from 
the  glomeruli,  have  contributed  a  considerable  proportion  of  the  proteins. 
But  in  the  milder  and  more  chronic  cases,  we  are  safe  to  conclude  that 
the  bulk  of  the  albumins  have  been  discharged  through  the  glomeruli. 
Here  it  is  of  interest  to  recall  that  cell-disintegration  is  not  necessarily 
associated  with  the  presence  of  albumin  in  the  urine;  casts,  the  coagulated 
products  of  cell-disintegration,  may  be  present  in  urine  that  gives  no 
reaction  for  albumin.  Most  often  the  albumin  consists  of  serum 
albumin  alone,  but  the  proportion  may  vary  within  wide  limits,  and 
Maguire1  and  others  have  recorded  cases  in  which  the  globulins  alone 
were  present,  or  with  traces  of  serum  albumin  so  small  that  they  could 
not  be  estimated.  And  this  notwithstanding  that,  according  to  Salvioli, 
the  proportion  of  albumin  to  globulin  in  the  blood  plasma  (roughly  3  :  2) 
is  remarkably  constant,  and  that  from  their  constitution,  the  globulins 
should  be  the  more  diffusible.  Here,  we  have  some  of  the  strongest 

lancet,  London,   1:  1886:  1062  and  1100. 


726       THE  RENAL  FUNCTIONS  AND  THEIR  DISTURBANCES 

evidence  that  the  glomeruli  are  not  simple  filters,  but  exert  a  selective 
or  controlling  influence  upon  the  fluid  passing  through  their  walls. 
What  then  are  the  conditions  favoring  albuminuria?  This  question  is 
perhaps  best  answered  by  detailing  the  conditions  under  which  albu- 
minuria is  encountered. 

1.  Physiological  Albuminuria. — Albumin  is  apt  to  appear  in  the  urine 
after  cold  baths,  and  violent  exertion,  the  latter  more  particularly  if 
partaken  of  in  the  morning,  soon  after  change  from  the  supine  to  the 
erect  position.     In  these  conditions  it  is  supposed  that  glomerular  con- 
gestion, with  dilatation  of  the  capillary  loops,  favors  the  exudation. 

2.  Cyclical  Albuminuria. — This  is  noted  more  especially  in  boyhood 
and  adolescence,  although  cases  are  on  record  in  which  it  has  continued 
into  adult  life.     The  subjects  may  appear  to  be  in  excellent  health,  but 
usually  they  complain  of  tiring  easily.     The  characteristic  feature  is 
that  albumin  is  absent  from  the  urine  passed  on  rising,  but  after  this 
makes  its  appearance,  rising  rapidly  in  amount  until  the  forenoon,  dis- 
appearing in  the  afternoon.     In  some  of  these  cases  paraglobulin  alone 
is  present.     Here,  again,  the  change  in  the  circulation  accompanying 
the  change  from  the  resting  to  the  erect,  active  state  would  appear  to  be 
a  primary  factor,  although  with  it  there  must  be  assumed  a  peculiar 
sensitiveness  or  idiosyncrasy  of  the  glomerular  epithelium.     The  condi- 
tion may  follow  scarlet  fever,  in  which  we  know  that  the  glomeruli  are 
peculiarly  liable  to  be  affected,  and  has  been  noted  after  other  fevers. 
There  is  no  clear  evidence  that  this  form  passes  on  to  chronic  nephritis, 
although  Dukes,  one  of  the  first  to  call  attention  to  its  frequency  among 
schoolboys,  has  placed  on  record  cases  in  which,  after  disappearance, 
it  has  shown  itself  again  years  later  under  various  stresses. 

3.  Albuminuria  from  Circulatory  Disturbances. — Anything  which  materi- 
ally slows  the  rate  of  blood  flow  through  the  kidneys  favors  the  super- 
vention of  albuminuria,  and  this  presumably  by  partial  asphyxia,  and, 
therefore,  imperfect  function  of  the  glomeruli  with  or  without  dilatation 
of  the  capillary  loops.     Thus,  on  the  one  hand,  contraction  of  the  renal 
arteries,  as  in  lead  colic,  or,  more  commonly,  on  the  other,  various  ob- 
structions (as  from  heart  disease  or  local  obstruction  of  the  renal  vein) 
is  accompanied  by  a  definite  grade  of  albuminuria.     In  the  former  case 
there  is  lowered  pressure  in  the  glomeruli  and  renal  capillaries,  in  the 
latter,  heightened  pressure.    In  both  forms  the  amount  of  urine  excreted 
is  reduced.     In  both  cases  also  it  is  possible  that  the  malnutrition  of 
the  tubules  leads  to  some  disintegration  of  the  parenchyma,  and  to  a 
contributory  albuminuria  from  this  cause. 

4.  Toxic   Albuminuria. — There   are   various   drugs   which   appear   to 
act  more  particularly  on  the  tubules — to  be  excreted  through  those  tubules 
and,  indeed,  to  influence  specifically  particular  regions  of  the  same. 
Perhaps  the  most  marked  examples  have  been  afforded  from  Ehrlich's 
laboratory,  Levaditi1  and   Rehns2  having   shown  that  vinylamin  and 

1  Arch,  internat.  de  Pharmacodynamie  et  Therap.,  8:  1901:  45. 

2  Ibid.,  p.  199. 


CHYLURIA  727 

tetrahydroquinole'ine  cause  a  necrosis  strictly  localized  to  the  papillae 
and  collecting  tubules.  More  often,  as  by  sublimate  and  cantharidin, 
the  convoluted  tubules  are  involved.  Some  of  these  in  their  action 
induce  a  well-marked  albuminuria.  Here,  we  include  chrome  and  various 
other  metallic  salts,  cantharidin,  various  balsams,  several  bacterial 
toxins,  and  the  so  far  unknown  toxic  substance  of  the  eclamptic  state. 
As  already  noted,  Lohlein  and  others  doubt  whether  the  degenerative 
changes  set  up  by  these  agents  should  be  classed  as  true  nephritis. 
With  some  of  these  there  is  evidence  of  accompanying  glomerular  dis- 
turbance, but  in  general  the  parenchymal  disturbance  is  so  severe  that 
we  may  attribute  the  albuminuria  mainly  to  this  cause. 

5.  Infectious  Albuminuria.— As  laid  down  on  page  724,  the  typical  acute 
nephritis  of  infectious  disease,  notably  of  scarlatina  and  streptococcal 
conditions,  is  characterized  by  pronounced  irritation  and  disturbance  of 
the  glomeruli,  and  here  in  general  we  find  the  most  abundant  discharge 
of  albumin.  The  disturbance  in  some  cases  is  so  acute  that  there  is 
an  escape  of  blood  corpuscles  through  the  glomeruli  (acute  hemorrhagic 
nephritis).1  With  these  changes  there  develop  also  notable  disturbances 
of  the  parenchyma,  with  cloudy  swelling,  fatty  degeneration,  necrosis 
and  desquamation.  More  rarely,  as  in  scarlatina,  there  may  be  associ- 
ated an  acute  interstitial  nephritis,  with  accumulations  of  plasma  cells 
between  the  tubules. 

Hemoglobinuria. — Besides  the  hematuria  above  mentioned,  there 
may  be  escape  of  dissolved  hemoglobin  into  the  urine.  Experimentally, 
this  may  be  brought  about  in  a  variety  of  ways,  by  introducing  substances 
into  the  circulation  which  cause  hemolysis  (large  quantities  of  water, 
glycerin,  pyrogallic  acid,  toluylenediamin,  etc.).  In  disease,  hemoglo- 
binuria also  follows  acute  hemolysis  (after  snake  bite,  potassium  chlorate 
poisoning,  and  in  paroxysmal  hemoglobinuria).  We  have  discussed 
these  conditions  in  our  first  volume  (p.  880),  and  only  refer  to  them 
here  to  note  that,  as  shown  by  one  of  us,2  it  is  possible  to  demon- 
strate the  discharge  of  hemoglobin  through  the  glomeruli  when  the 
blood  pressure  is  so  low  that  anuria  has  set  in,  as  also  to  obtain  a  urine 
containing  three  times  the  amount  of  hemoglobin  present  in  the  blood 
plasma.  In  other  words,  the  presence  of  hemoglobin  in  the  urine  is  due 
to  active  excretion,  and  not  mere  filtration. 

Chyluria. — Rarely,  w~e  encounter  cases  in  which  the  urine  is  diluted 
with  lymph.  Such  chyluria  may  persist  with  exacerbations  over  long 
years.  When  due  to  renal  disturbance,  the  cause  is  now  generally  re- 
garded as  a  lymphangiectatic  condition  affecting  the  papillae,  with  rup- 
ture of  one  or  more  of  the  dilated  vessels. 


1  As  Cornil  well  pointed  out,  the  capsule  chamber  of  the  glomeruli  may  be  com- 
pared with  a  serous  sac  like  the  pericardium  or  pleural  cavity.  It  may  be  affected 
by  the  same  series  of  acute  inflammatory  changes,  and  with  continued  subacute 
inflammation  may  show  proliferative  changes  in  its  epithelium  and  the  development 
of  synechia  and  localized  adhesions. 

'-'  Adami,  Jour,  of  Physiol.,  6:  1S86:  382. 


CHAPTEE    XXXV. 

THE  KIDNEYS  AND  URETERS. 

THE  KIDNEYS. 
CONGENITAL  AND  ACQUIRED  ANOMALIES. 

THESE  consist  largely  in  defective  development  and  abnormalities 
in  size,  shape,  position,  and  anatomical  structure. 

Aplasia. — One  or  other  of  the  organs  may  be  absent  (aplasia)  in 
people  otherwise  perfectly  formed.  This  occurred  three  times  in  one 
thousand  autopsies  of  our  series.  According  to  Ballowitz,  in  57  per  cent, 
of  cases  it  is  the  left  kidney  which  is  defective.  The  condition  is  usually 
of  no  great  consequence,  as  the  remaining  kidney  undergoes  compensatory 
hyperplasia.  The  occurrence  of  the  condition  should  be  remembered  in 
connection  with  surgical  operations,  however,  for  cases  have  been  known 
where  the  only  kidney  was  removed  for  disease,  with,  of  course,  disastrous 
results.  As  a  rule,  the  corresponding  ureter  is  absent,  although  occasion- 
ally a  rudiment  of  it  may  be  found  connected  with  the  bladder.  Where 
only  one  kidney  is  present  it  is  often  long  and  narrow,  suggesting  the 
superposition  of  one  kidney  upon  the  other,  and  may,  moreover,  be 
provided  with  a  double  pelvis  and  two  ureters. 

Both  kidneys  may  be  absent  in  certain  monsters,  a  condition  which  is, 
of  course,  inconsistent  with  life. 

Hypoplasia. — Congenital  hypoplasia  of  one  kidney  is  rather  common. 
The  organ  is  small,  surrounded  by  much  perirenal  fat,  the  secreting 
structure  is  scanty,  and  there  may  be  considerable  fibrosis.  The  vessels 
supplying  it  are  also  small.  More  rarely,  both  kidneys  are  thus  affected. 

Horseshoe  Kidney. — An  interesting  anomaly,  which  is  rather  in- 
frequent, occurring  according  to  our  statistics  in  0.4  per  cent,  of  all 
autopsies,  is  the  so-called  "horseshoe"  kidney.  In  this  condition  the 
kidneys  are  imperfectly  separated  one  from  the  other.  They  lie  close  to 
the  vertebral  column,  and  are  united  most  commonly  at  their  lower 
end  by  kidney  substance,  or,  more  rarely,  by  a  fibrous  band.  Rarely, 
the  union  takes  place  at  the  upper  or  middle  portion.  The  horseshoe 
kidney  is  usually  situated  somewhat  lower  than  normal,  and  may  be 
found  upon  the  promontory  of  the  sacrum.  In  the  latter  situation  it 
may  prove  an  impediment  to  labor.  One  of  the  component  organs  lies 
somewhat  higher  than  the  other.  The  ureters  generally  pass  out  to 
the  front  and  the  vessels  are  abnormal  in  their  origin.  The  condition 
is  of  no  great  moment  and  is  usually  discovered  accidentally,  but  occa- 
sionally serious  symptoms  have  resulted,  such  as  thrombosis  of  the  under- 


DOUBLE  PELVIS  AND  URETER  729 

lying  veins  and  pressure  upon  the  ureters  giving  rise  to  hydronephrosis  or 
pyelonephritis.  Such  kidneys  would  seem  to  be  more  liable  to  disease 
than  normal  ones.  We  have  met  with  one  case  in  which  one-half  of  the 
organ  was  tuberculous,  and  another  in  which  it  contained  a  large  coral 
calculus. 

FIG.  191  FIG.  192 


Horseshoe  kidney.     (From  the  Pathological  Museum  of         Complete    double  ureter.     (From 
McGill  University.)  the  Pathological  Museum  of  McGill 

University.) 

Kidneys  are  not  uncommonly  found  which  are  elongated,  rounded, 
spleen-like,  or  hogbacked  in  shape.  The  hogbacked  kidney  is  probably 
not  due  to  alcoholism,  as  used  to  be  thought,  but,  rather,  is  congenital, 
for  it  is  found  often  in  children  and  in  others  who  have  never  touched 
alcohol.  In  the  spleen-  or  cake-like  kidney  the  pelvis  is  frequently 
situated  posteriorly. 

Double  Pelvis  and  Ureter. — Another  fairly  common  anomaly  is 
for  one  or  both  kidneys  to  be  provided  with  a  double  pelvis  and  ureter. 
The  ureters  usually  unite  somewhere  about  their  lower  third  and  continue 


730 


THE  KIDNEYS 


as  a  single  channel,  or  may  fuse  at  the  point  of  junction  with  the  bladder, 
or,  again,  more  rarely,  may  empty  by  separate  orifices  into  the  bladder. 
When  more  or  less  separate,  one  ureter  invariably  crosses  the  other. 
Such  kidneys  are  often  abnormally  long,  as  if  showing  a  tendency  to 
reduplication  in  series. 

The  ureter  of  a  normal  kidney  may,  instead  of  emptying  into  the  blad- 
der, discharge  into  the  colliculus  seminalis,  a  seminal  vesicle,  the  urethra, 
vagina,  or  uterus. 

Foetal  Lobulation. — A  very  common  anomaly  is  fcetal  lobulation. 
Here,  on  the  surface  of  the  organ  are  numerous  shallow  furrows  repre- 
senting the  original  divisions  of  the  various  renculi.  Rarely,  the  kidney 
is  composed  of  a  number  of  small  separate  organs. 

FIG.  193 


Congenital  cystic  kidney.     Zeiss  obj.  DD,  without  ocular.     (From  the  Royal  Victoria 
Hospital  collection.) 

Dislocation  of  the  Kidney. — Dislocation  of  a  kidney  is  fairly  fre- 
quent also.  It  may  be  congenital  or  acquired.  In  the  congenital  form 
the  vessels  present  an  abnormal  origin,  while  the  ureter  is  usually 
shortened.  In  the  acquired  form,  the  vessels  are  normal  in  origin  and 
distribution,  though  both  the  vessels  and  ureter  are  lengthened  and 
tortuous.  The  suprarenals  usually  occupy  their  normal  position.  The 
abnormally  situated  kidney  may  lie  on  the  vertebral  column,  either  on 
its  own  or  the  opposite  side,  on  the  sacral  promontory,  in  the  pelvis,  or 
beneath  the  anterior  abdominal  wall.  The  organ  may  be  fixed  in  its 
abnormal  situation  by  fibrous  adhesions. 

Congenital  dislocation,  according  to  Kupfer,  is  due  to  a  deficiency  in 
the  movement  of  the  embryonic  rudiments  of  the  kidneys,  which,  up  to 


PASSIVE  HYPEREMIA  731 

a  certain  period,  are  formed  just  in  front  of  the  point  of  bifurcation 
of  the  aorta. 

The  acquired  form  seems  to  be  due  to  deficiency  in  the  amount  of  the 
perirenal  fat,  such  as  occurs  in  prolonged  wasting  disease;  pressure,  as 
from  tight-lacing;  traumatism;  heavy  lifting;  or  to  a  relaxed  abdominal 
wall,  resulting  in  diminished  intra-abdominal  pressure.  In  most  cases 
it  is  part  of  a  general  gastro-enteroptosis  or  splanchnoptosis.  Occasion- 
ally, the  dislocation  is  brought  about  by  the  weight  of  a  renal  tumor. 

Cysts. —  Cysts  of  the  kidney  are  not  uncommon.  The  organs  are 
greatly  enlarged,  warty,  and  on  section  present  great  numbers  of  sacs 
containing  thin  fluid.  The  condition  is  supposed  to  be  due  to  retention 
owing  to  imperfect  fusion  of  the  collecting  tubules  with  the  secretory 
portion.1 


CIRCULATORY  DISTURBANCES. 

Oligemia. — Generalized  oligemia  of  the  kidney  is  found  in  cases  of 
general  systemic  oligemia,  either  essential  or  secondary.  In  the  early 
stages,  the  organ  on  section  is  uniformly  pale,  of  a  grayish-yellow  color 
and  fairly  translucent.  In  the  advanced  condition,  as  is  well  seen  in 
pernicious  anemia,  the  kidney  is  pale,  yellow,  and  turbid-looking, 
owing  to  the  resulting  fatty  degeneration. 

Local  anemia  is  met  with  in  the  white  infarct. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  is  met  with 
in  acute  inflammations,  a  forcibly  acting  left  heart,  various  intoxications, 
and  in  death  from  cerebral  tumors,  meningitis,  and  the  like. 

Passive  Hyperemia. — Passive  hyperemia  is  usually  due  to  some  ob- 
struction in  the  general  circulation,  such  as  valvular  heart  disease,  or 
some  pulmonary  disturbance.  A  unilateral  lesion  is  rarer,  and  results 
from  some  obstruction  in  the  inferior  vena  cava  or  renal  vein,  as  from 
thrombosis  or  the  pressure  of  enlarged  glands  or  tumor-masses  upon 
the  vessel. 

The  kidney  is  enlarged,  its  consistency  firmer  than  normal,  and  the 
capsule  peels  off  with  great  ease.  The  stellate  veins  are  injected  and 
the  surface  has  a  dark  purple  red,  or  cyanotic  appearance.  On  section, 
the  organ  is  very  firm,  drips  blood,  and  has  a  uniformly  dark  red  appear- 
ance. In  the  milder  grades,  the  straight  vessels  can  be  made  out  as 
red,  converging  lines  and  the  glomeruli  as  minute  reddish  points  in  the 
cortical  portion. 

Microscopically,  the  vessels  are  all  enlarged  and  congested,  the  Bow- 
man's capsules  contain  albumin,  with  possibly  a  few  red  blood-cells, 
and  the  tubules  contain  a  few  hyaline  casts.  Certain  of  the  epithelial 
.cells,  notably  those  of  the  descending  loops,  contain  pigment  granules 
derived  from  altered  blood.  In  long-standing  cases  the  capillary  and 

1  An  excellent  account  of  the  various  anomalies  of  the  kidneys  will  be  found  in 
Huntingdon's  Harvey  Lectures,  1906-7:  222,  Lippincott. 


THE  KIDNEYS 
FIG.  194 


Anemic  infarct  of  cortex  of  kidney  to  show  coagulation  necrosis,  with  surrounding  zone 
of  congestion,     a,  artery.     (Orth.) 


White  infarct  of  the  kidney  undergoing  organization.     Leitz  obj.  No.  3,  without  ocular. 
The  necrotic  area  is  to  the  right.     (From  the  collection  of  Dr.  A.  G.  Nicholls.) 


THE  ARTERIOSCLEROTIC  CONTRACTED  KIDNEY  733 

venous  walls  appear  thickened,  there  is  increase  in  the  interstitial  con- 
nective tissue,  and  occasionally  a  round-celled  interstitial  infiltration — a 
condition  known  as  "cyanotic  induration."  In  some  instances  the 
secreting  epithelium  is  found  to  be  fatty. 

Hemorrhage. — (See  pp.  743  and  746.) 

Infarction. — Infarction  of  the  kidney  is  due  to  a  sudden  stoppage 
of  the  circulation  in  a  portion  of  the  organ,  through  embolism  of  one  of 
the  branches  of  the  renal  artery.  It  is  usually  anemic  in  nature.  In  the 
course  of  a  few  hours  the  part  which  is  deprived  of  its  blood-supply, 
becomes  pale,  grayish-white,  and  more  or  less  opaque  and  granular. 
The  area  is  usually  roughly  wedge-shaped,  with  the  apex  toward  the 
boundary  zone.  The  margin  is  well-defined  by  a  zone  of  hyperemia  or 
hemorrhage. 

Microscopically,  the  affected  area  presents  all  the  appearances  of 
coagulation  necrosis.  The  cells  are  swollen,  granular,  opaque,  and  take 
a  diffuse  muddy  stain.  The  nuclei  are  pale  or  invisible.  Round  the 
margin  the  vessels  are  congested,  there  may  be  hemorrhage,  and  an 
accumulation  of  leukocytes  due  to  a  reactive  inflammation.  The 
infarcted  areas  vary  in  size  from  that  of  a  bean  to  a  third  or  even 
the  whole  of  the  organ.  The  necrosed  portion  may  reach  quite  to  the 
capsule  or  there  may  be  a  small  zone  of  healthy  or  relatively  healthy 
kidney  tissue  between,  depending  on  the  amount  of  anastomosis  with 
the  vessels  of  the  capsule.  In  course  of  time  the  affected  cells  undergo 
fatty  and  hydropic  degeneration  and  are  eventually  absorbed  and  de- 
stroyed, their  places  being  taken  by  proliferating  connective  tissue  from 
margin.  Ultimately,  only  a  scar  is  left.  The  scar-tissue  is  grayish- 
white  or  reddish  in  color,  sometimes  pigmented.  Where  there  have 
been  numerous  infarcts,  the  kidney  is  contracted,  scarred,  with  a  mark- 
edly irregular  surface  (einbolic  granular  kidney). 

The  Arteriosclerotic  Contracted  Kidney. — The  arteriosclerotic 
contracted  kidney  is  a  form  of  granular  kidney  originating  in  a  narrowing 
and  eventual  obstruction  of  the  afferent  vessels  of  the  organ.  The  condi- 
tion may  be  restricted  to  the  renal  artery  and  its  branches,  or  may  be 
part  of  a  general  arteriosclerotic  process.  The  changes  are  of  the 
nature  of  a  chronic  proliferation  of  the  intima  or  media.  This  leads  to 
collapse  of  the  glomerular  capillaries  and  atrophy  of  the  tufts.  The 
glomeruli,  through  hyaline  changes  in  the  capillaries,  become  converted 
into  rounded  masses,  at  first  relatively  poor  in  nuclei  but  ultimately 
structureless  or  fibroid.  The  Bowman's  capsules  may  be  thickened, 
though  usually  not  to  any  great  extent.  As  a  result  of  the  degeneration 
of  the  glomeruli  the  efferent  tubules  become  collapsed  and  atrophied. 
The  process,  as  a  whole,  tends  to  affect  certain  vascular  districts  corre- 
sponding to  the  interlobular  arteries.  As  the  various  structures  atrophy, 
their  place  is  taken  by  connective  tissue,  which  gradually  shrinks,  leading 
to  the  formation  of  a  scar.  Thus,  the  surface  of  the  kidney  becomes 
more  or  less  warty  or  granular,  and  the  capsule  is  somewhat  adherent. 
The  atrophic  process  is  more  marked  in  some  regions  than  in  others,  with 
the  result  that  localized  rather  sharply-defined  depressed  areas  are  to 


734  THE  KIDNEYS 

be  seen  on  the  surface.     The  interlobular  vessels  become  also  tortuous 
or  spiral  through  sinking  in  of  the  cortex. 

The  arteriosclerotic  kidney  is  usually  bright  red  or  grayish-red  in 
color,  or,  owing  to  fatty  degeneration,  may  be  streaked  with  yellow. 
It  is  diminished  in  size,  the  capsule  is  adherent,  and  the  cortex  is  narrowed 
and  distorted.  The  organ  cuts  more  firmly  than  usual  and  the  glomeruli 
stand  out  as  whitish  dots.  The  smaller  arterioles,  through  thickening 

FIG.  196 


An  arteriosclerotic  kidney.     The  preparation  shows  well  the  irregular  coarse  granulations  on 
the  exterior  of  the  organ.     (From  the  Pathological  Museum  of  McGill  University.) 

of  their  walls,  are  easily  recognized.  The  renal  artery  itself  often  shows 
marked  sclerotic  changes.  A  striking  feature  is  the  marked  narrowing 
of  cortex  and  medulla.  The  pelvis  is  relatively  large  and  filled  with 
fatty  tissue. 

The  condition,  though  etiologically  different,  is  not  easily  differentiated 
from  the  inflammatory  contracted  kidney.  In  the  latter  type,  however, 
the  process  is  more  apt  to  be  diffused  and  even  in  its  character,  and 
the  signs  of  arterial  sclerosis  throughout  the  body  are  not  so  marked. 

Senile  Atrophy. — Allied  to  the  arteriosclerotic  kidney  is  senile  atrophy. 
This  is  also  a  form  of  granular  kidney,  but  the  diminution  in  size  of  the 
organ  is  usually  not  extreme.  The  capsule  is  thickened  and  somewhat 
adherent,  the  granulation  fine.  The  condition  is  often  confined  to  but 
few  of  the  glomeruli,  so  that  the  scarring  is  slight  and  the  macroscopic 
changes  are  not  obtrusive.  The  condition  is,  in  large  measure,  at  all 
events,  due  to  impoverished  blood  supply,  since  arteriosclerosis  is  so 


NEPHRITIS  735 

common  in  elderly  people,  but  various  retrogressive  processes  no  doubt 
play  a  part. 

INFLAMMATIONS. 

Nephritis. — This  section  brings  us  to  the  consideration  of  one  of  the 
most  complicated  and  obscure  problems  in  the  whole  realm  of  pathology. 
The  truth  of  this  statement  will  become  evident  as  we  proceed.  The  chief 
difficulty  consists  in  this,  that  it  is  sometimes  impossible  to  determine  the 
etiological  factors  at  work  in  certain  cases  and  to  trace  the  course  of 
the  morbid  processes  thereby  set  up,  while  it  is  often  extremely  difficult 
to  bring  the  clinical  manifestations  into  close  correspondence  with 
the  anatomical  conditions. 

The  kidneys  are  the  chief  eliminative  organs  of  the  body.  Their 
structure  is  glandular  and  their  function  is  to  excrete  the  various  waste 
products  of  metabolism  that  reach  them  through  the  blood  stream. 
Abnormal  substances  introduced  into  the  body  from  without,  or  resulting 
from  disturbed  or  perverted  metabolism  within  the  system,  may,  and  often 
do,  lead  to  irritation  of  these  important  organs  and  even  to  structural 
change.  The  result  in  any  given  case  will  depend  on  the  amount  of  the 
deleterious  substances  present  as  well  as  their  quality.  In  fact,  sub- 
stances normally  produced,  if  present  in  too  great  quantity,  may  act 
similarly  to  those  that  are  essentially  toxic  in  their  nature.  Conversely, 
functional  inadequacy  of  the  kidneys  is  not  without  its  effect  upon  the 
composition  of  the  blood.  The  quantity  of  water  eliminated  may  deviate 
considerably  from  the  normal  and  waste  products  may  be  retained 
instead  of  excreted.  The  quality  of  the  blood  is  thus  depreciated,  and, 
being  laden  with  toxic  substances,  it  in  turn  exerts  an  irritating  and 
deteriorating  effect  upon  the  kidneys.  In  this  way  a  vicious  circle  is 
set  up,  as  a  result  of  which  the  condition  of  the  patient  goes  rapidly 
from  bad  to  worse. 

We  can,  perhaps,  better  appreciate  the  pathogeny  of  kidney  affections 
if  we  consider  for  a  moment  the  relationship  which  the  organs  in  question 
bear  to  other  parts  of  the  body.  In  addition  to  the  kidneys,  the  organs 
concerned  in  the  elimination  of  toxic  substances  are  the  intestines,  liver, 
lungs,  and  skin.  Any  disturbance  of  these  organs  which  inhibits  their 
function  of  necessity  throws  more  work  upon  the  kidneys,  and  vice  versa. 
The  functions  of  these  various  structures  are,  then,  to  a  certain  extent, 
complemental.  As  an  example  of  this  we  may  cite  the  well-known  fact 
of  the  excretion  of  urea  by  the  skin  when  the  function  of  the  kidneys 
is  defective.  Nephritis  also  has  been  known  to  follow  upon  gastro- 
enteritis (Ebstein,1  Dupeu2).  Again,  sudden  chilling  of  the  surface  of 
the  body  may  be  followed  by  nephritis. 

These  results  may  be  brought  about  in  two  ways:  namely,  through 
the  circulatory  and  through  the  nervous  systems.  Thus,  toxins  elab- 
orated by  abnormal  metabolism  in  distant  parts  of  the  body  may  be 

1  Deutsch.  med.  Woch.,  58 :  1897 :  1.  2  Jour,  de  Med.,  July  10,  1897. 


736  THE  KIDNEYS 

carried  to  the  kidneys  by  the  bloodvessels,  and  untoward  nervous  im- 
pressions may  result  in  other  profound  disturbance.  The  latter  effect  is, 
perhaps,  also,  at  bottom  circulatory  in  that  the  caliber  of  the  renal 
vessels  may  be  modified  reflexly,  though  it  is  not  impossible  that  the 
changes  in  point  are  neurotrophic  in  character.  At  any  rate,  we  can, 
with  safety,  conclude  that  the  bloodvessels  play  the  controlling  part  in 
the  production  of  most  forms  of  nephritis  by  determining  the  quality 
and  the  quantity  of  the  blood  brought  to  the  kidneys.  Sudden  or 
repeated  congestion  of  the  kidneys  will  impair  their  vitality  and  pre- 
dispose them  to  infection  and  other  forms  of  irritation.  Any  condition, 
whether  of  general  or  local  character,  that  lessens  the  amount  of  blood 
going  to  the  organs  will  lead  to  their  deterioration.  Toxic  substances 
circulating  in  the  blood  pass  through  the  capillary  walls  into  the 
tubules,  bringing  about  in  their  transit  degenerative  changes  in  the 
lining  endothelium  and  in  the  secreting  cells.  When  the  condition  is 
sufficiently  prolonged,  reactive  and  reparative  phenomena  make  their 
appearance,  and  we  then  get  evidences  of  inflammation.  Degeneration 
and  inflammation  are,  therefore,  the  keynotes  to  the  proper  understand- 
ing of  the  many  differing  conditions  grouped  under  the  generic  term 
nephritis.  Degeneration  is  manifested  in  the  retrogressive  changes, 
cloudy  swelling,  vacuolation,  and  necrosis  met  with  in  the  endothelial 
and  secreting  cells;  inflammation  is  indicated  by  the  serous  and  cellular 
infiltration,  the  congestion,  and  the  hyperplasia  of  tissue.  The  distinc- 
tions between  the  degenerative  and  the  inflammatory  manifestations 
are  often  neither  clinically  nor  anatomically  to  be  sharply  drawn.  The 
same  causes  which  bring  about  a  pure  degeneration  are  often  com- 
petent to  excite  inflammation.  Conversely,  certain  peculiar  changes  in 
the  urine,  to  be  referred  to  more  at  length  shortly,  may  result  equally 
from  degenerative  or  inflammatory  processes.  As  a  matter  of  fact, 
degeneration  and  inflammation  are  nearly  always  combined,  either 
in  the  relationship  of  cause  and  effect,  or  as  a  fortuitous  association  of 
conditions. 

The  Urine.— Valuable  information  as  to  the  condition  of  the  kidneys 
may  be  gathered  from  an  examination  of  the  urine.  The  most  striking 
changes  consist  in  a  more  or  less  marked  deviation  from  the  normal  in 
point  of  quantity,  specific  gravity,  and  reaction;  the  appearance  of 
albumin;  and  the  presence  of  foreign  material,  such  as  casts,  red  blood 
cells,  leukocytes,  epithelium,  cell-detritus,  blood-pigment,  fat-droplets, 
shreds  of  tissue,  urinary  salts,  and  bacteria.  This  phase  of  the  subject 
is  more  suitably  relegated  to  works  on  clinical  diagnosis,  and  we  cannot 
do  more  here  than  draw  attention  to  the  main  outlines. 

Acute  Parenchymatous  Nephritis. — In  acute  parenchymatous  nephritis 
the  amount  of  urine  excreted  in  the  twenty-four  hours  is  greatly  reduced, 
namely,  to  300  c.cm.  or  even,  in  the  earlier  stages  of  the  attack,  to  100  c.cm. 
Complete  suppression  is  occasionally  met  with.  The  urine  is  acid,  of 
high  specific  gravity  (1024  to  1030),  turbid,  and  of  high  color.  It  may 
be  smoky,  or  even  bright  red  in  appearance  from  the  admixture  of  blood. 
The  percentage  of  urea  may  be  increased  but  the  total  output  of  urea, 


THE  URINE  737 

as  of  other  nitrogenous  substances,  is  diminished.  The  uric  acid  may 
be  normal  in  amount,  and  the  purin  bases  are  generally  in  excess.  The 
urine  contains  albumin,  varying  in  amount  from  0.3  to  1  per  cent,  (by 
weight,  5  to  10  grams  daily).  The  sediment,  which  is  usually  abundant, 
consists  of  red  blood-cells,  leukocytes,  renal  and  bladder  epithelium, 
crystals  of  uric  acid  and  oxaiates,  hyaline,  granular,  epithelial,  leukocytic, 
and  blood  casts.  Hemoglobin  may  be  present  in  the  urine  in  cases  in 
which  severe  blood  destruction  has  taken  place  (hemoglobinemia).  Blood 
and  blood  pigment  may  be  present  in  considerable  amounts  in  the  forms 
known  as  acute  hemorrhagic  nephritis.  It  has  been  shown  recently 
that  the  freezing  point  of  the  urine  in  cases  of  nephritis  differs  from  that 
of  normal  urine.  The  process  for  the  determination  of  this  fact  is  called 
cryoscopy.  The  freezing  point  of  normal  urine  has  been  demonstrated 
to  be  from  1.3°  to  2.3°  C.  below  that  of  distilled  water,  while  that  of  the 
urine  in  nephritis  is  only  1°  C.  or  less  below  that  of  distilled  water.  This 
is  due  to  the  molecular  concentration  of  the  urine,  which  is  less  in  neph- 
ritis than  in  health. 

The  urine  in  acute  embolic  suppurative  nephritis  is  practically  that 
of  acute  hemorrhagic  non-suppurative  parenchymatous  nephritis. 

Chronic  Diffuse  Nephritis. — In  the  chronic  diffuse  nephritis  (without 
induration),  or  large  white  kidney,  the  urine  is  also  diminished  in  amount, 
varying  between  300  and  700  c.cm.  The  urine  is  acid,  turbid,  and  of 
increased  specific  gravity  (1018  to  1025).  It  is  often  highly  colored,  and 
may  contain  a  notable  amount  of  blood.  It  always  contains  consider- 
able albumin,  from  15  to  30  grams  in  the  twenty-four  hours.  The 
urea  and  other  solids  are  below  the  normal  quantity.  The  sediment  may 
consist  in  any  of  the  elements  mentioned  under  the  acute  form. 

Chronic  Interstitial  Nephritis. — In  the  chronic  interstitial  form  (chronic 
diffuse  nephritis  with  induration),  the  urine  is  increased  in  amount,  from 
1800  to  4000  c.cm.  daily.  It  is  acid,  pale  in  color,  and  of  low  specific 
gravity  (1002  to  1015).  Albumin  is  trifling  in  amount  and  may  even 
be  absent  for  prolonged  periods.  Casts  are  few  and  usually  hyaline  in 
character.  Occasionally  a  few  erythrocytes  may  be  found.  The  solids 
of  the  urine  are  generally  diminished. 

Pyelonephritis. — In  the  cases  of  pyelitis  and  pyelonephritis  resulting 
from  stone,  granular  and  cellular  debris,  urinary  salts,  epithelium,  and 
pus  cells  are  often  present  in  considerable  amounts.  Massive  hemor- 
rhage may  also  occur  into  the  urine.  Where  ulceration  takes  place, 
shreds  of  tissue  may  be  passed  into  the  urine. 

The  systemic  poisoning  resulting  from  the  absorption  of  the  urinary 
salts  and  other  products  of  metabolism  is  known  as  uremia. 

In  many  cases  of  nephritis,  especially  those  complicating  the  various 
infectious  fevers,  bacteria,  usually  those  specific  for  the  primary  disease, 
can  by  suitable  methods  be  found  in  the  urine.  The  bacteriology  of 
chronic  nephritis  has  not  as  yet  been  worked  out.  In  tuberculosis 
of  the  kidney,  the  specific  bacilli,  often  in  considerable  numbers,  can  be 
detected. 

The  subject  of  the  classification  of  certain  affections  of  the  kidneys 
47 


738  THE  KIDNEYS 

is  one  fraught  with  much  difficulty.  This  is,  in  large  part,  due  to 
the  difficulty  in  determining  the  correlation  between  the  etiology,  the 
morbid  anatomy,  and  the  clinical  manifestations  in  many  cases  of 
nephritis.  On  the  one  hand,  the  same  etiological  factor  may  bring  about 
a  diversity  of  anatomical  changes,  and,  on  the  other,  one  and  the  same 
clinical  picture  may  result  from  a  variety  of  morbid  causes.  Again, 
the  severity  of  the  outward  manifestations  of  the  disease  does  not  always 
bear  a  direct  relationship  to  the  apparent  extent  of  the  lesions.  Time 
and  again,  we  find  at  autopsy  advanced  renal  disease  in  cases  where  from 
the  clinical  features  we  would  not  have  expected  it,  and,  conversely,  we 
may  have  well-marked  clinical  evidence  of  disease  with  kidneys  that  are 
practically  normal  to  gross  examination.  As  a  consequence,  we  do  not 
find  perfect  unanimity  among  writers  as  to  what  constitutes  nephritis 
and  what  does  not. 

There  are  three  ways  of  classifying  kidney  affections:  (1)  according  to 
etiology;  (2)  according  to  the  location  of  the  lesions;  and  (3)  according 
to  the  nature  of  the  inflammatory  process. 

The  etiological  method  would  be  eminently  scientific,  but  presents 
the  practical  difficulties  that  have  just  been  mentioned.  On  this  basis 
we  might  differentiate  congestive,  toxic,  and  infective  disturbances. 

The  second,  or  topographical  method,  is  theoretically  possible  and  is 
not  devoid  of  merit.  We  may,  recognizing  that  the  epithelium,  the 
glomeruli,  the  interstitial  stroma,  and  the  bloodvessels  may  be  affected, 
divide  nephritis  into  parenchymatous,  glomerular,  interstitial,  and 
arteriosclerotic  forms.  The  first  three  of  these  forms  may  again  be 
divided  into  acute  and  chronic;  the  last  is,  of  course,  always  chronic. 
The  chief  objection  to  this  classification  lies  in  the  fact  that  it  is  impossible 
to  draw  a  hard  and  fast  line  between  parenchymatous  and  interstitial 
inflammations.  To  indicate  all  the  possible  permutations  and  com- 
binations would  necessitate  a  cumbrous  terminology. 

According  to  the  third  method,  we  may  recognize  with  Delafield, 
in  the  first  instance,  three  types  of  kidney  affections — congestion,  degen- 
eration, and  inflammation.  Inflammation  may,  again,  be  divided  into 
acute  exudative  nephritis;  acute  productive  nephritis;  chronic  nephritis 
with  exudation;  chronic  nephritis  without  exudation;  and  suppurative 
nephritis.  The  considerations  just  detailed  are  sufficient  to  indicate  the 
difficult  nature  of  the  problem  before  us. 

That  dropsy  and  albuminuria  are,  in  certain  cases,  related  to  affections 
of  the  kidneys  has  been  recognized  for  centuries.  Aetius  (367  A.D.), 
Avicenna  (980  to  1036),  and  van  Helmont  (1577  to  1644)  all  held  that 
certain  cases  of  dropsy  were  due  to  disease  of  the  kidneys.  Cotunnius, 
in  1770,  discovered  that  the  urine  of  dropsical  patients  could  be  coagu- 
lated by  boiling. 

We  owe  our  modern  conceptions  of  kidney  inflammations,  however, 
to  Richard  Bright,  of  Guy's  Hospital,  who  published,  in  1827,  the  first 
thorough  and  scientific  studies  of  this  type  of  disease.  Bright  demon- 
strated the  dependence  of  albuminuria  and  dropsy  on  disease  of  the 
kidneys.  He  accurately  described  the  morbid  changes  in  the  kidneys, 


BRIGHT'S  DISEASE  739 

and  showed  the  relationship  of  the  clinical  symptoms  to  the  anatomical 
lesions.  He,  further,  described  many  of  the  associated  conditions  and 
sequela?,  such  as  uremia  and  the  cardiovascular  phenomena,  blindness, 
apoplexy,  and  inflammation  of  the  serous  membranes.  So  accurate  and 
thorough  was  his  work  that  most  of  it  has  stood  the  test  of  subsequent 
inquiry.  As  a  result,  the  term  Bright's  disease  has  been  adopted  the 
world  over  as  the  designation  among  clinicians  for  the  non-suppurative 
inflammations  of  the  kidney  usually  associated  with  albuminuria  and 
dropsy. 

Further  advances  were  made  by  Rokitansky,  who  described  in  1842 
the  amyloid  kidney.  Johnson  (1852)  drew  attention  to  the  vascular 
changes  in  the  kidney,  work  which  was  taken  up  and  amplified  in  1872 
by  Gull  and  Sutton  in  their  study  of  what  they  called  "  arteriocapillary 
fibrosis,"  in  which  they  emphasized  the  relationship  of  certain  changes 
in  the  bloodvessels  to  cirrhosis  and  atrophy  of  the  kidney. 

A  matter  of  some  importance  is  to  decide  how  much  we  should 
include  under  the  term  Bright's  disease.  Some  authorities,  such  as 
Leyden,  regard  Bright's  disease  as  embracing  all  forms  of  kidney  dis- 
ease associated  with  albuminuria  and  hydrops,  and  would,  therefore, 
include  in  this  category  cases  of  degeneration  of  the  kidney  epithelium, 
pyelonephritis,  and  the  amyloid  kidney.  Others,  again,  with  Klebs, 
would  separate  the  non-inflammatory  degenerative  manifestations  from 
true  Bright's  disease.  At  the  present  time,  it  seems  to  be  fairly  generally 
agreed  to  differentiate  the  circulatory  and  degenerative  disorders  of 
the  kidneys  from  the  primarily  inflammatory  affections,  or  true  Bright's 
disease.  With  regard  to  the  forms  of  nephritis  proper,  Sir  Thomas 
Grainger  Stewart,  in  1871,  recognized  three  types — the  inflammatory, 
the  amyloid,  and  the  contracting  forms.  Under  the  first  he  described 
three  stages,  that  of  inflammatory  exudation,  that  of  fatty  degeneration, 
and  that  of  induration,  a  classification  practically  coinciding  with  that 
of  Bartels,  who  classified  these  affections  into  acute  parenchymatous, 
chronic  parenchymatous,  and  interstitial  nephritis. 

In  the  classification  that  we  have  suggested  here  we  have  sided  with 
those  who  would  differentiate  between  congestive,  degenerative,  and 
inflammatory  affections  of  the  kidney.  The  further  division  of  nephritis 
proper  into  non-suppurative,  suppurative,  and  specific  forms  of  inflam- 
mation is  so  convenient  and  accurate  that  it  is  hardly  likely  to.  arouse 
serious  antagonism.  More  difference  of  opinion  may,  perhaps,  arise 
over  the  various  subvarieties  of  non-suppurative  nephritis,  but  the  forms 
mentioned  here  are  all  types  well  known  to  the  pathological  histologist, 
and,  at  any  rate,  do  not  conflict  with  clinical  experience.  They  are, 
however,  to  be  regarded  merely  as  types,  without  it  being  understood 
that  they  are  separated  one  from  the  other  by  hard  and  fast  lines. 

Under  the  affections  of  the  kidney,  ordinarily  associated  with  either 
albuminuria  or  anasarca,  or  both,  we  recognize,  therefore,  the  following: 


740 


THE  KIDNEYS 


A.  Congestion 


B.  Degenerations 


C.  Inflammation 


Non-suppurative  - 


Acute 


Suppurative 


Specific 


Passive  hyperemia. 
fa.  Parenchymatous  degeneration. 
j  6.  Embolic    granular   kidney,    or 
infarcted  kidney. 

c.  Senile  and  atrophic. 

d.  Arteriosclerotic  kidney. 

Ie.  Cyanotic  induration. 
/.  Amyloid  fatty  kidney. 
[g.  Amyloid  contracted  kidney. 

a.  Acute  parenchymato us  nephri- 
tis. 

1.  Hemorrhagic  nephritis. 

2.  Glomerulitis. 

3.  Desquamative      papillary 

nephritis. 

6.  Acute  interstitial  nephritis, 
c.  The  kidney  of  pregnancy. 
i  d.  Acute  diffuse  nephritis. 


a.  Chronic  diffuse  nephritis. 

1.  Hemorrhagic  nephritis. 
Chronic   <j          2.  Glomerulitis. 

Ib.  Chronic  interstitial  nephritis. 
c.  Primary   (genuine)    contracted 
[  kidney. 


a.  Embolic  abscesses. 

b.  Pyelonephritis. 
-j  c.  Pyonephrosis. 

j  d.  Traumatic  nephritis. 

[  e.  Nephritis  per  extensionem. 

fa.  Tuberculosis. 
|  b.  Syphilis. 

c.  Glanders. 

d.  Actinomycosis. 

e.  Leprosy. 


With  regard  to  the  etiology  of  acute  nephritis  it  may  be  remarked 
here  that  the  most  important  single  causal  element  is  infection.  We 
may  recognize  four  main  classes  of  this  affection : 

1.  Those  due  to  various  intoxications,  such  as  from  alcohol,  lead, 
cantharides,  phosphorus,  chlorate  of  potash,  and  salicylic  acid. 

2.  Those  complicating  the  acute  infections,  such  as  scarlatina,  small- 
pox, pneumonia,  acute  endocarditis,  erysipelas,  diphtheria,  typhoid  fever, 
septicemia,  acute  rheumatism,  cholera,  acute  tonsillitis,  vaccinia,  septic 
wounds,  epidemic  cerebrospinal  meningitis,  and  certain  gastro-intestinal 
disorders. 

3.  Those  associated  with  chronic  diseases  and  cachexias,  as  diabetes, 
carcinoma,  pulmonary  tuberculosis,  and  syphilis. 

4.  The  so-called  "idiopathic"  cases. 

It  has  been  abundantly  demonstrated  by  experimental  studies  that 
a  great  variety  of  toxins,  both  mineral  and  bacterial,  are  competent  to 
set  up  degenerative  changes,  of  the  nature  of  cloudy  swelling,  fatty 
degeneration,  and  even  necrosis,  in  the  secreting  epithelium  of  the  kidney. 
For  example,  as  Wandervelde1  has  shown,  toxins  like  those  of  cholera, 


Act.  d.  pois.  sur  les  cell,  epithet,  d.  canalicules  contournees,  Brux.,  1894. 


NEPHRITIS  741 

cholera  nostras,  tuberculosis,  diphtheria,  pneumonia,  influenza,  and 
certain  chemical  substances,  such  as  chromic  acid,  lead,  phosphorus, 
mercuric  chloride,  when  injected  into  laboratory  animals,  exert  a  harmful 
influence  upon  the  glandular  structure  of  the  kidney,  bringing  about 
changes  identical  in  appearance  with  those  met  with  in  the  human 
subject.  How  far  these  are  inflammatory  is  open  to  debate.  Paren- 
chymatous  degeneration  is  doubtless  the  first  stage  of  inflammation  in 
many  cases  of  nephritis,  but,  from  the  pathologist's  point  of  view  at  least, 
can  occur  independently  of  inflammation,  and,  moreover,  need  not  neces- 
sarily give  rise  to  it.  When  inflammation  does  supervene  in  such  cases 
it  is  quite  fair  to  assume,  in  the  absence  of  positive  information,  that 
it  is  as  likely  due  to  secondary  infection  as  to  the  influence  of  the  circu- 
lating toxin.  Consequently,  for  the  sake  of  clearness,  while  it  is  well  to 
preserve  in  our  minds  the  distinction  between  "  degeneration"  and 
"  inflammation,"  practically,  as  we  have  before  remarked,  it  is  not 
always  possible  to  distinguish  between  them. 

We  are,  however,  on  more  certain  ground  when  we  come  to  discuss 
the  forms  of  nephritis  occurring  in  the  course  of  infective  processes. 
Here  a  microbic  origin  can  be  traced  in  nearly  every  case.  The  bacteria 
found  in  the  urine  of  such  cases  are  usually  those  specific  of  the  primary 
disease.  Thus,  streptococci  and  staphylococci  have  been  found  in 
the  urine  in  cases  of  acute  endocarditis  (Weichselbaum,  Mannaberg) ; 
the  Typhoid  bacillus,  in  cases  of  nephritis  arising  during  the  course  of 
typhoid  fever  (Blumer);  the  Pneumococcus,  in  cases  of  pneumonia 
(Massalong,  Klebs,  Michelle).  In  a  study  of  this  subject  made  by  one 
of  us  (A.  G.  N.1)  some  years  ago,  in  32  cases  of  acute  nephritis  of  various 
forms,  bacteria,  usually  the  specific  germs  of  the  primary  disease,  were 
present  in  28. 

It  is  difficult,  therefore,  to  avoid  the  conclusion  that  most  cases  of  acute 
nephritis  in  man  are  due  to  infection  with  microorganisms.  The  specific 
changes  are  produced,  it  may  be  presumed,  in  the  course  of  an  attempt 
on  the  part  of  the  kidneys  to  eliminate  the  offending  agents. 

The  acute  forms  of  nephritis  arising  in  the  course  of  chronic  diseases 
are  largely  of  the  nature  of  terminal  infections. 

The  relationship  of  infection  to  chronic  nephritis  is,  unfortunately, 
much  more  obscure.  No  doubt  certain  cases  of  acute  nephritis  pass 
imperceptibly  over  into  the  chronic  type,  and  it  is  possible,  and  indeed 
likely,  that  in  these  instances  the  infective  agents  are  still  at  work.  As 
a  proof  of  this,  it  may  be  pointed  out  that  cirrhosis  of  the  kidneys  has 
been  known  to  result  from  infective  diseases,  such  as  pneumonia  and 
influenza.  It  is  true  that  one  important  class  of  chronic  kidney  disease 
is  due  to  arteriosclerosis,  and  another  (primary  contracted  kidney)  to 
the  influence  of  alcohol,  lead,  and  gout.  (This  latter  form,  however, 
may  be  at  bottom  but  a  variety  of  the  arteriosclerotic  type.)  Yet, 
cirrhosis  of  the  kidneys  has  been  met  with  in  children,  in  whom  the  influ- 

1  Nicholls,  A  Contribution  to  the  Study  of  Bright's  Disease,  Montreal  Med.  Jour., 
28;  1899;  161. 


742  THE  KIDNEYS 

ence  of  arteriosclerosis  and  chronic  mineral  intoxication  could  with 
certainty  be  excluded.  The  possibility  of  infection  being  the  cause  of 
many  cases  of  chronic  nephritis  was  pointed  out  in  the  paper  just  referred 
to.  In  eight  cases  of  chronic  parenchymatous  nephritis  minute  diplo- 
cocci  were  found  in  four;  in  one  case  of  chronic  glomerulitis  the  same 
diplococcus  was  present;  in  the  chronic  diffuse  form  (11  in  number), 
bacteria  were  met  with  in  all;  in  10  cases  of  chronic  interstitial  nephritis, 
minute  diplococci  were  found  in  every  instance.  Altogether,  in  45  cases 
of  chronic  nephritis  of  all  forms,  minute  diplococci  were  seen  in  29 
and  bacilli  in  4  more.  The  germs  in  question  were  usually  found 
in  the  areas  of  interstitial  round-celled  infiltration,  suggesting  a  causal 
relationship.  The  source  of  these  bacteria  is  difficult  to  say,  but  it 
may  be  remarked  that  in  nearly  41  per  cent,  of  the  cases  there  was  a 
definite  history  of  preceding  gastro-enteric  disturbance. 

FIG.  197 


Acute  parenchymatous  nephritis.  The  section  shows  well  the  necrosis  of  the  secreting 
tubules.  Reichert^obj .  No.  7,  without  ocular.  (From  the  Pathological  Laboratory  of  McGill 
University.) 

Simple  or  Non-suppurative  Nephritis.  —  Acute  Parenchymatous 
Nephritis.  —  (Synonyms:  acute  degenerative  parenchymatous  nephritis, 
acute  tubular  nephritis,  desquamative  nephritis,  catarrhal  or  croupous 
nephritis,  acute  Bright's  disease.) 

This  form  of  nephritis  is  of  frequent  occurrence  and  is  characterized 
in  the  main  by  marked  degenerative  changes  in  the  secreting  tubules, 
such  as  cloudy  and  fatty  degeneration.  With  this  there  is,  however, 
congestion,  exudation  of  serum,  and  desquamation  of  the  secreting  cells. 
In  the  earlier  stages,  the  condition  is,  no  doubt,  identical  with  the  simple 


NEPHRITIS  743 

parenchymatous  degeneration  that  is  so  commonly  found  as  a  result  of 
intoxications  and  infections  of  various  kinds.  In  fact,  it  is  sometimes 
impossible  to  draw  the  line  between  cloudy  swelling  and  actual  nephritis. 
The  vascular  changes,  the  desquamation  of  the  cells,  and  certain  char- 
acteristic changes  in  the  urine,  are  usually  sufficient,  however,  to  make 
the  diagnosis. 

The  kidneys  are  usually  more  or  less  enlarged  and  oedema tous.  The 
capsule  peels  off  with  more  than  normal  ease,  owing  to  the  swelling  of 
the  kidney  substance,  which  tends  to  bulge  through  the  cut.  The  surface 
is  pale,  the  stellate  veins  injected,  and  the  lobules  well  indicated.  The 
cortex  is  swollen,  pale,  and  cloudy,  presenting  a  marked  contrast  to  the 
dark  red  or  bluish-red  medulla.  In  some  cases  the  cortex  is  somewhat 
congested.  Minute  petechial  hemorrhages  can  frequently  be  seen 
scattered  over  the  cortical  surface  and  throughout  its  substance. 

Microscopically,  the  structures  chiefly  affected  are  the  contorted 
tubules.  The  secreting  cells  are  swollen,  cloudy  or  granular,  often 
vacuolated,  while  the  nuclei  stain  badly  or  not  at  all.  Within  the  lumina 
of  the  tubules,  especially  in  the  collecting  portion,  are  to  be  seen  hyaline 
or  granular  casts,  and  droplets  of  albumin.  It  is  not  uncommon  to  find 
the  lining  cells  of  the  tubules  lying  free  within  the  basement  membrane 
in  all  stages  of  fatty  and  hyaline  degeneration.  In  some  cases  the  tubules 
contain  blood.  The  glomeruli  also  show  evidences  of  degeneration, 
the  epithelium  lining  the  Bowman's  capsules  being  swollen  and  often 
desquamated,  while  structureless  masses  of  albumin  can  be  seen  within 
the  spaces.  Hemorrhage  into  the  capsules  is  noted  in  some  cases. 
The  amount  of  effused  blood  may  be  so  great  that  free  blood  and  blood 
casts  appear  in  the  urine,  warranting  the  term  acute  hemorrhagic  nephritis. 
The  interstitial  substance  is  swollen  and  cedematous,  and  there  is 
sometimes,  although  not  invariably,  a  small  amount  of  round-celled  in- 
filtration and  a  deposit  of  fibrin  in  the  interstitial  stroma.  The  extent 
of  the  lesions  varies  in  different  cases.  As  a  rule,  the  contorted  tubules 
are  the  structures  first  and  chiefly  involved,  but  the  loops  of  Henle  and 
the  collecting  tubules  do  not  always  escape. 

In  certain  cases,  glomerular  changes  so  dominate  the  picture  that  we 
can  properly  speak  of  acute  glomerulitis  or  glomerulonephritis.  This 
form  is  most  commonly  met  with  in  scarlatina  and  diphtheria.  To  the 
naked  eye  the  kidney,  as  a  rule,  shows  very  little  change,  at  most  being 
somewhat  cloudy  and  hyperemic.  The  glomeruli  are  swollen  and  show 
up  as  reddish  or  pale  grayish  dots. 

Microscopically,  both  degenerative  and  productive  changes  in  the 
glomeruli  are  found,  so  that,  according  as  one  or  the  other  predomi- 
nates, we  may  differentiate  a  degenerative  and  a  productive  glomerulo- 
nephritis. In  the  former  variety,  the  capillaries  of  the  glomeruli  are 
congested  or  show  hyaline  degeneration,  while  the  epithelial  cells  lining 
the  Bowman's  capsules  are  degenerated  and  desquamating.  The  Bow- 
man's space  is  filled  with  degenerated  epithelial  cells,  red  and  white 
corpuscles,  albumin,  and  granular  detritus.  In  the  latter  form,  the 
lining  cells  and  the  various  endothelia  show  proliferative  changes. 


744  THE  KIDNEYS 

Desquamative  Papillary  Nephritis. — A  less  important  variety  is  the 
so-called  desquamative  papillary  nephritis  in  which  the  lesions  are  chiefly 
confined  to  the  tubules  in  the  papillary  portion,  the  cells  of  which  are 
swollen  and  desquamating. 

Acute  Interstitial  Nephritis. — (Synonyms:  acute  productive  nephritis, 
"  lymphomatous"  nephritis  of  Wagner.)  An  interesting  form  of  inflam- 
mation is  the  acute  interstitial  nephritis. 

This  affection  is  found  more  especially  in  scarlatina  and  diphtheria. 
It  also  occurs  in  measles,  pneumonia,  whooping-cough,  acute  endocar- 
ditis, and  epidemic  cerebrospinal  meningitis.  The  condition  is  probably 
due  to  the  action  of  bacteria.  The  pyogenic  cocci,  the  B.  coli,  the 
Klebs-Loeffler  bacillus,  and  the  Pneumococcus  are  the  microorganisms 
that  have  been  found  in  association  with  it.  Councilman,1  in  a  study 
of  such  cases,  would  lay  no  stress  upon  the  presence  of  these  germs,  as 
he  found  them  in  the  kidney  in  the  same  proportions  in  cases  other  than 
interstitial  nephritis.  It  may  be  remarked,  however,  that  the  production 
of  nephritis  probably  depends  on  other  factors  than  the  mere  presence  of 
bacteria,  namely,  on  the  number  and  virulence  of  the  germs  and  the 
vulnerability  of  the  tissues,  so  that  we  need  not  refer  any  form  of 
nephritis  to  the  selective  action  of  particular  microbes.  The  infective 
origin  is  supported  by  the  observations  of  Letzerich,2  who  has  described 
an  epidemic  of  acute  interstitial  nephritis  due  to  a  bacillus,  which 
morphologically  resembled  the  B.  tuberculosis  and  on  injection  into 
animals  produced  nephritis. 

In  this  form  the  kidney  is  somewhat  enlarged,  the  capsule  strips 
easily,  and  its  consistence  is  diminished.  The  cortical  surface  is  mottled 
and  of  a  gray,  grayish-white,  or  grayish-red  color.  The  stellate  veins  are 
injected.  On  section,  the  cortex  is  greatly  swollen,  paler  than  the 
medulla  and  of  a  streaky,  opaque,  grayish-white  appearance.  The 
striated  appearance  characteristic  of  the  normal  pyramids  is  lost.  The 
kidney  substance  is  soft,  moist,  and  friable.  The  changes  are  most 
marked  in  the  intermediate  zone. 

Microscopically,  the  degenerative  changes  in  the  secreting  cells  and 
the  Malpighian  tufts  are  reduced  to  a  minimum  and  the  characteristic 
feature  is  a  more  or  less  irregularly  distributed  accumulation  of  small 
round  cells  in  the  interstitial  substance.  This  infiltration  is  in  small 
patches  and  is  particularly  marked  at  the  bases  of  the  pyramids,  beneath 
the  capsule,  and  around  the  glomeruli.  The  cells  found  are  of  the 
type  of  lymphocytes,  with  some  plasma  cells. 

The  Kidney  of  Pregnancy. — What  may,  for  the  present,  be  conveniently 
called  the  "kidney  of  pregnancy"  is  a  peculiar  condition  met  with  in 
pregnant  women.  It  has  not  yet  been  settled  whether  the  condition  is 
purely  degenerative  or  whether  it  is  in  part  degenerative  and  in  part 
inflammatory,  but  the  lesions  on  the  whole  correspond  fairly  closely 

1  Journ.  Exper.  Med.,  3:  1898:  393. 

2  Untersuch.  u.  Beobacht.  ueber  Nephritis  bacillosa  interstitialis  primaria,  Zeit. 
f.  klin.  Med.,  13:  1887:  33. 


THE  KIDNEY  OF  PREGNANCY  745 

with  those  found  in  what  we  have  below  called  acute  diffuse  nephritis. 
It  is  important  to  note  that  the  pregnant  woman  may  be  the  subject 
of  an  acute  nephritis  exactly  in  the  same  way  as  other  individuals  may 
on  occasion  be  attacked,  and,  again,  that  pregnancy  may  occur  in  one 
already  suffering  from  Bright's  disease.  But,  apart  from  these  fortuitous 
associations,  the  kidney  may  become  affected  during  the  course  of  preg- 
nancy, and  apparently  in  some  way  as  the  result  of  it,  without  the 
ordinary  etiological  factors  being  discoverable.  This  is  the  condition  of 
things  which  is  so  often  associated  with  eclampsia.  The  affection  in 
question  usually  arises  during  the  latter  half  of  pregnancy,  and,  prefer- 
ably, in  young  primiparse  and  in  twin  pregnancies. 

The  kidney  varies  somewhat  in  different  cases,  but  is  usually  enlarged, 
the  capsule  peels  off  readily,  the  cortex  is  smooth,  pale,  and  of  a  yellowish 
color.  On  section,  the  cortex  is  swollen.  Microscopically,  the  lesions 
are  those  referable  to  an  acute  parenchymatous  degeneration,  while 
the  interstitial  substance  is  but  little  affected.  Cases  have  occurred  in 
which  complete  necrosis  of  the  parenchyma  occurred  (Klotz,1  Rose 
Bradford,  Jardine). 

The  condition  has  been  referred  to  auto-intoxication,  the  kidneys 
being  inadequate  to  eliminate  waste  products  for  both  mother  and 
child;  to  infection  and  toxemia;  to  increased  intra-abdominal  and  intra- 
pelvic  pressure  exerted  upon  the  renal  veins,  the  ureters,  or  the  cceliac 
ganglia ;  to  the  absorption  of  toxic  products  derived  from  the  placenta. 

While  for  purposes  of  description  it  is  convenient  to  refer  to  inflamma- 
tion of  the  kidney  as  " parenchymatous,"  "glomerular,"  or  "interstitial," 
according  as  the  lesions  are  chiefly  manifested  in  the  secreting  cells, 
the  capillary  tufts,  or  the  supporting  stroma,  it  would  be  far  from  correct 
to  think  that  the  pathological  process  in  any  case  is  confined  to  the 
structures  named  to  the  exclusion  of  the  rest.  Every  acute  and  subacute 
nephritis  is,  in  a  sense,  "diffuse,"  in  that  all  parts  of  the  kidney  are 
involved,  though  it  may  be  unequally.  We  name,  therefore,  the  various 
forms  according  to  the  predominating  features  of  the  morbid  changes. 
When  we  speak  of  acute  diffuse  nephritis  in  a  specific  sense  we  mean 
that  type  in  which  the  inflammatory  phenomena  are  more  or  less  uni- 
formly manifested  throughout  the  secreting  and  the  supporting  structures 
of  the  organ.  To  cloudy  swelling,  fatty  degeneration,  and  necrosis  of 
the  secreting  tubules,  are  added  oedema  and  leukocytic  infiltration  of  the 
interstitial  tissue.  As  might  be  expected,  however,  the  lesions  are  most 
marked  in  the  cortical  portion,  that  being  the  region  of  the  greatest 
functional  activity,  and  consequently,  the  most  vulnerable.  The  con- 
gestion, which  is  a  striking  feature  of  all  inflammations  in  their  earlier 
stages,  is  chiefly  manifested  in  the  pyramids,  the  vessels  of  the  cortex 
being  rendered  anemic  owing  to  the  swelling  of  the  cells  of  the  tubules 
and  the  pressure  of  the  effused  inflammatory  products.  The  diffuse  form 
is,  perhaps,  the  most  common  type  of  acute  and  subacute  nephritis. 

1  Journ.  of  Obstet.,  October,  1908. 


746  THE  KIDNEYS 

Chronic  Diffuse  Nephritis. — The  affection  known  as  chronic  diffuse 
nephritis  (chronic  parenchymatous  nephritis,  inflammatory  fatty  kidney; 
large  white  kidney;  variegated  kidney;  chronic  desquamative  nephritis; 
second  stage  of  Bright's  disease)  may  occur  as  the  sequel  of  the  acute 
diffuse  form,  degenerative  changes  in  the  epithelium  and  interstitial 
infiltration  becoming  still  more  marked  and  the  condition  passing 
imperceptibly  from  the  acute  to  the  subacute  and  finally  to  the  chronic 
state,  or,  more  often,  arising  insidiously.  The  anatomical  changes 
are  strictly  comparable  to  those  found  in  the  acute  and  subacute  stages, 
but  are  more  extreme.  Thus,  we  may  have  chronic  parenchymatous 
nephritis,  chronic  hemorrhagic  nephritis,  and  chronic  glomerulitis. 

To  gross  appearance  the  most  striking  features  are  the  inflammatory 
swelling  and  the  peculiar  color.  The  kidney  is  enlarged,  somewhat 
soft  and  doughy  in  consistence,  the  capsule  peels  off  readily,  and 
the  surface  is  smooth  and  of  a  grayish-yellow  or  grayish-white  color, 
occasionally  presenting  dilated  stellate  veins  or  minute  hemorrhagic 
spots.  The  pale  color  of  the  exterior  may  be  uniform  in  intensity,  but 
not  infrequently  is  irregular,  patches  of  congestion  alternating  with 
areas  of  pallor,  giving  the  organ  a  somewhat  variegated  appearance. 
On  section,  the  cortex  is  swollen,  of  a  uniform  or  patchy  pallor,  similar 
to  that  of  the  surface,  with  occasional  areas  of  congestion  or  hemorrhagic 
extravasation.  The  peculiarity  in  color  is  due,  in  part,  to  necrosis  and 
fatty  changes  of  the  secreting  cells  of  the  tubules  and  in  part  to  the 
anemia  produced  by  the  pressure  of  the  effused  inflammatory  material. 
Until  recently  it  was  held  that  fatty  degeneration  of  the  tubular  epithelium 
was  a  cardinal  feature  in  this  form.  We  know  now,  however,  that  in 
some  cases,  at  all  events,  the  fat  is  present,  not  as  free  fat,  but  com- 
bined in  the  form  of  soaps.  The  medullary  portion  is  more  or  less 
congested,  and  of  a  dull  red  color,  contrasting  markedly  with  the  pallid 
cortex.  In  the  most  extreme  forms  of  this  type  the  kidney  may  be  of 
normal  size,  the  surface  slightly  pitted,  and  the  capsule  somewhat 
adherent,  indicating  the  onset  of  atrophy  and  fibrosis.  When  the 
amount  of  hemorrhage  into  the  kidney  substance  is  considerable  it  may 
justify  the  term  chronic  hemorrhagic  nephritis. 

Now  and  then  cases  are  met  with  in  which  the  organ  is  not  specially 
enlarged  and  presents  no  marked  deviation  from  the  normal,  at  least 
so  far  as  macroscopic  appearance  is  concerned.  These  are  the  cases  in 
which  the  inflammatory  changes  are  chiefly  confined  to  the  glomeruli, 
while  the  rest  of  the  tissues  practically  escape  (chronic  glomerulitis). 

Microscopically,  the  morbid  changes  vary  considerably.  In  the  char- 
acteristic "  large  white  kidney "  the  most  notable  feature  is  the  presence 
of  fat  or  soaps  which  are  somewhat  widespread  in  the  epithelium  of 
the  glomeruli  and  the  secreting  tubules,  and  even  in  the  lining  cells  of 
the  bloodvessels.  With  this,  there  is  to  be  observed  a  more  or  less  ex- 
tensive inflammatory  infiltration,  oedema,  and  leukocytic  exudation  into 
the  interstices  of  the  interstitial  stroma.  In  the  more  advanced  cases 
there  may  be  indications  of  atrophy  of  the  glomeruli  and  secreting  cells, 
with,  possibly,  a  slight  amount  of  secondary  fibrosis. 


CHRONIC  INTERSTITIAL  NEPHRITIS  747 

The  glomeruli  in  many  cases  may  show  fatty  degeneration  of  the 
epithelium  of  the  tufts  and  the  Bowman's  capsules;  in  other  cases, 
swelling,  proliferation,  and  desquamation  of  the  epithelial  cells  are  more 
prominent,  though  not  infrequently  degenerative  and  reparative  processes 
may  be  combined.  In  the  Bowman's  spaces  there  is  an  effusion  of 
albuminous  fluid  which,  owing  to  the  method  of  hardening,  has  been 
coagulated  into  droplets,  granular  or  fibrinoid  masses.  Leukocytes 
and,  in  the  hemorrhagic  cases,  red  blood-cells  may  also  be  found.  The 
glomeruli  themselves  are  often  compressed  from  the  accumulation  of 
fluid  and  inflammatory  cells,  the  epithelium  is  fattily  degenerated,  the 
capillary  vessels  are  thickened,  and  may  contain  leukocytes  and  hyaline 
thrombi.  The  capillaries,  being  thus  filled  with  inflammatory  and 
degenerative  products,  become  blocked  and  may  in  time  be  converted  by 
organization  into  solid  strands.  Eventually,  a  certain  number  of  the 
glomeruli  shrink,  are  transformed  into  minute  hyaline  masses,  poor  in 
or  devoid  of  nuclei,  and  surrounded  by  a  contracted  and  thickened 
capsule. 

The  secreting  cells,  particularly  those  of  the  contorted  tubules,  show 
advanced  necrosis  and  a  deposit  of  fats  and  soaps  in  their  substance, 
with  consequent  exfoliation,  either  in  multiple  isolated  areas  or  uni- 
formly throughout  the  cortex,  though  the  collecting  tubules  do  not 
entirely  escape.  The  lumina  of  the  tubules  not  infrequently  are  filled 
with  degenerated  cells,  leukocytes,  pigment  and  fatty  particles,  together 
with  granular  detritus.  The  lining  cells  are  flattened,  causing  the 
lumina  to  appear  relatively  enlarged.  They  may,  however,  be  dilated 
from  obstruction.  In  hemorrhagic  cases  the  tubules  contain  numerous 
red  blood-cells.  Casts  of  various  kinds  maybe  also  detected.  These 
changes  in  the  tubules  are  usually  conspicuous  and  are  only  of  trifling 
extent  in  the  form  known  as  chronic  glomerulitis. 

The  interstitial  connective  tissue  is  cedematous  and  presents  here  and 
there  small  areas  of  cellular  infiltration.  In  the  hemorrhagic  forms  the 
stroma  also  contains  pigment  granules.  In  the  most  advanced  cases 
the  supporting  stroma  is  increased  in  amount  and  gives  definite  evidence 
of  proliferation, -while  the  Bowman's  capsules  are  also  thickened.  Such 
changes  are  found  in  those  kidneys  which  are  beginning  to  show  evidences 
of  atrophy  and  contraction  in  their  smaller  size  and  slightly  scarred 
cortex. 

Chronic  Interstitial  Nephritis. — This  form  of  chronic  nephritis  passes 
imperceptibly  into  the  next,  chronic  interstitial  nephritis  (chronic  pro- 
ductive nephritis;  chronic  indurative  nephritis;  granular  kidney;  chronic 
diffuse  nephritis  with  induration;  contracted  kidney;  secondary  con- 
tracted kidney;  fibroid  kidney;  cirrhotic  or  sclerotic  kidney;  third  stage 
of  Bright's  disease). 

It  is  generally  held  at  the  present  time  that  the  contracted  kidney 
is  of  four  types;  the  first  arising  as  a  sequel  to  the  large  white  kidney 
(secondary  contracted  kidney) ;  the  second,  originating  in  an  acute  neph- 
ritis and  apparently  not  passing  through  the  large  white  kidney  stage 
(primary  contracted  kidney)]  the  third,  due  to  sclerosis  of  the  renal 


748  THE  KIDNEYS 

vessels,  and,  therefore,  of  a  degenerative  rather  than  an  inflammatory 
nature  (arteriosclerotic  kidney;  see  p.  733);  and  the  fourth,  the  senile. 

From  the  nature  of  the  case  it  is  extremely  difficult,  and  in  some  in- 
stances impossible,  to  make  out  the  exact  sequence  of  events  in  these 
various  forms.  The  doubtful  points  are  hardly  likely  to  be  cleared  up 
until  we  have  the  results  of  more  extended  experimentation  at  our 
command.  Time,  therefore,  may  modify  our  present  conceptions  of 
renal  inflammation. 

In  a  well-marked  case  of  chronic  interstitial  nephritis  of  the  secondary 
contracted  type,  the  kidney  is  usually  diminished  in  size.  In  consistence 
it  is  firm  and  hard,  somewhat  elastic,  and  cuts  with  more  or  less  resistance, 
resembling  in  this  other  fibroid  structures.  The  capsule  is  somewhat 
thickened  and  is,  in  places,  adherent  to  the  cortex.  On  removing  it 
small  portions  of  the  cortex  are  torn  away.  The  surface  of  the  kidney  is 
irregular,  warty,  or  granular,  being  studded  with  prominences  of  some- 
what uneven  distribution,  and  of  greater  or  smaller  size.  Occasional 
cysts,  containing  a  clear  colorless  or  straw-colored  fluid,  are  usually 
to  be  seen.  The  color  of  such  a  kidney  varies,  and  it  may  be  mentioned 
in  passing  that  the  terms  "small  white  kidney"  and  "small  red  kidney," 
which  have  been  so  often  employed,  do  not  really  indicate  any  important 
difference  in  type,  the  color  of  the  organ  being  dependent  largely  on  the 
amount  of  blood  which  it  contains,  though  also  to  some  extent  upon 
the  degree  of  atrophy  and  fatty  changes  that  it  has  undergone.  Gener- 
ally, the  kidney  is  somewhat  translucent  and  of  a  dull  red  color,  but  it 
may  be  grayish  or  grayish-white,  mottled,  or  even  quite  white  and  opaque. 
The  superficial  furrows  are  usually  of  a  brighter  red  color  than  are  the 
granulations.  On  section,  the  medullary  portion  is  usually  somewhat 
brighter  colored  than  the  cortex,  but  may,  again,  resemble  it  in  hue. 
As  a  rule,  the  cortex  is  diminished  in  thickness  and  this  is  more  especially 
the  case  in  those  parts  immediately  in  association  with  the  depressions  of 
the  surface.  It  may,  however,  happen  that  in  other  regions  the  cortex 
is  of  normal  size,  or  even  increased,  owing  to  oedema.  The  larger  vessels 
usually  show  some  thickening. 

The  histological  features  may  be  epitomized  as  atrophy  and  degenera- 
tion of  the  secreting  structures  and  relatively  diffuse  new-formation  of 
connective  tissue.  The  relative  extent  of  these  factors  depends  on  the 
nature  of  the  case  and  how  closely  the  condition  approximates  to 
the  chronic  parenchymatous  form.  The  glomerular  epithelium  is,  in 
the  less  advanced  cases,  swollen  and  desquamating,  but  this  is  never  so 
marked  as  it  is  in  the  chronic  parenchymatous  form.  Later,  the  glomer- 
ular tufts  lose  their  epithelium  and  the  vessels  become  thickened  and 
impervious.  Eventually,  many  of  the  glomeruli  are  converted  into  a 
structureless,  hyaline  mass,  devoid  or  almost  devoid  of  nuclei,  and  more  or 
less  lobulated,  so  that  they  come  to  resemble  a  trefoil  or  rosette.  Not 
a  few,  however,  can  be  seen  which  have  evidently  retained  their  functions 
and,  in  fact,  have  undergone  a  compensatory  hypertrophy.  The 
capsule  at  the  same  time  shows  evidences  of  proliferation  and  thickening. 
The  changes  in  the  epithelium  of  the  convoluted  tubules  are  strictly 


CHRONIC  INTERSTITIAL  NEPHRITIS 
FIG.  198 


749 


Chronic  interstitial  nephritis,  showing  atrophy  of  tubules  and  certain  of  the  glomeruli; 
periglomerular  fibrosis;  dilatation  of  tubules.  Leitz  obj.  No.  3,  without  ocular.  (From 
the  collection  of  Dr.  A.  G.  Nicholls.) 

FIG.  199 


Chronic  interstitial  nephritis;   dilatation  of  the  tubules;   hyaline  casts.     Leitz  obj.  No.  7, 
without  ocular.     (From  the  collection  of  Dr.  A.  G.  Nicholls.) 


750  THE  KIDNEYS 

comparable  to  those  occurring  in  chronic  parenchymatous  nephritis, 
but  are  rarely  so  pronounced.  In  many  parts,  where  the  interstitial  tissue 
is  increased,  both  secreting  and  collecting  tubules  may  be  compressed 
or  collapsed,  and  are,  therefore,  smaller  than  normal,  being  lined  with 
cubical  cells.  Not  infrequently  it  will  be  found  that  certain  tubules 
have  entirely  disappeared,  or,  again,  their  places  are  indicated  by  a 
ring  or  double  row  of  simple  nuclei.  Such  epithelial  cells  as  remain  are 
small,  atrophic,  granular,  vacuolated,  or  fatty.  In  those  parts  of  the 
kidney  where  the  fibroid  induration  is  less  marked  a  different  picture  is 
to  be  seen.  Here,  the  tubules  are  often  dilated,  some,  indeed,  being 
cystic.  The  lumina  of  such  tubules  are  dilated  and  contain  granular 
debris  and,  perhaps,  an  occasional  cast,  while  the  lining  cells  are  flattened 
centrifugally  from  vertical  pressure  and  are  extended  laterally  to  cover 
the  increased  surface  of  the  basement  membrane.  The  epithelium  is 
also  fatty  and  shows  other  evidences  of  degeneration.  The  dilatation  of 
the  tubules  is  to  be  attributed  to  obstruction  of  the  lumina  in  some 
portion  of  their  course,  owing,  either  to  the  encroachment  of  the  newly 
formed  connective  tissue,  or,  again,  to  blocking  by  casts,  cellular  debris, 
and  precipitated  salts. 

In  all  cases,  there  is  an  increase  of  the  interstitial  connective  tissue, 
the  amount  varying  according  to  the  nature  of  the  case  and  the  chronicity 
of  the  disease.  The  depressions  of  the  surface  are  continuous  with 
bands  of  connective  tissue  which  extend  deeply  into  the  cortical  substance 
and  even  into  the  labyrinth.  These  bands  unite  freely  in  the  deeper 
portion,  thus  dividing  the  kidney  into  a  series  of  lobules.  In  such  areas 
of  hyperplasia  there  may  be  seen  accumulations  of  round  cells,  while 
here  and  there  can  be  made  out  developing  fibroblasts.  In  some 
few  cases,  the  fibrous  tissue  is  very  dense  and  fibrillar.  The  glomeruli 
and  tubules  in  these  districts  are  in  all  stages  of  atrophy  and  degeneration 
and  in  large  part  have  disappeared  altogether.  Between  the  fibrous 
patches  the  secreting  structures  that  remain  may  be  comparatively 
normal,  but  not  infrequently  show  a  certain  amount  of  fatty  change. 
Occasionally,  evidences  of  regenerative  hyperplasia  can  be  detected. 
It  is  by  no  means  uncommon,  moreover,  to  find  the  lesions  of  an  acute 
parenchymatous  nephritis  superimposed  on  those  of  the  chronic  inter- 
stitial form.  The  vessels  in  the  fibroid  areas  show  some  thickening  of 
the  adventitia  and  usually  of  the  intima  also,  leading  to  more  or  less 
obstruction  of  the  lumina.  Some  of  the  vessels  may  indeed  have  dis- 
appeared. 

An  important  form  of  chronic  Bright's  disease  is  that  known  as  primary 
chronic  interstitial  nephritis.  It  has  been  called  also  the  "  gouty  kidney," 
the  "genuine  contracted  kidney,"  and  the  "red  granular  kidney."  Ana- 
tomically, it  resembles  closely  the  other  types  of  chronic  interstitial 
kidney,  and,  indeed,  it  is  difficult  to  separate  it  from  them.  The  reasons 
for  recognizing  this  variety  as  a  separate  entity  are  largely  clinical.  The 
disease  begins  insidiously,  there  being  no  history  of  any  acute  renal 
inflammation.  When  symptoms  manifest  themselves,  the  kidney  is 
already  contracted.  The  affection  is,  therefore,  believed  not  to  pass 


ACUTE  HEMATOGENIC  SUPPURATIVE  NEPHRITIS  751 

through  the  "large  white  kidney"  stage.  The  process  is  primarily 
a  chronic  one.  The  etiology  is  often  obscure.  In  most  cases  there  is 
a  history  of  alcoholism,  gout,  or  lead  poisoning.  Sometimes  the  patient 
has  suffered  from  some  acute  infective  illness  years  before.  Syphilis, 
diabetes,  and  mental  strain,  are  also  mentioned  as  causative  factors. 
Heredity  seems  to  play  a  part  in  some  instances. 

In  this  form  the  kidney  is  usually  very  small,  smaller  than  that  of 
secondary  interstitial  nephritis,  hard,  and  granular.  The  granulations 
are  unusually  fine.  The  capsule  is  firmly  adherent  and  the  organ  is 
generally  of  a  dull  red  color.  Cysts  are  present  on  the  surface,  containing 
modified  urine  or  yellowish-green  colloid  material.  The  cortex  is  ex- 
tremely thin,  in  places  practically  absent.  The  pyramids  are  also 
reduced  in  size,  though  relatively  increased  in  proportion  to  the  cortex. 
In  the  gouty  cases  calcareous  and  uratic  deposits  may  be  found  in  the 
substance  of  the  organ.  The  branches  of  the  renal  artery  are  usually 
greatly  sclerosed.  The  pelvis  of  the  kidney  contains,  as  a  rule,  an  in- 
creased amount  of  fat,  and  the  atrophied  organ  is  also  embedded  in  a 
large  fatty  mass. 

Histologically,  the  pathological  changes  do  not  differ  materially  from 
those  described  in  the  secondary  form,  except  that  they  are  even  more 
intense,  and  widespread.  The  newly- formed  fibrous  tissue  is  particularly 
dense.  Compensatory  regeneration  is  also  met  with  here  in  the  cells  of 
the  tubules. 

Suppurative  Nephritis. — In  this  form  of  nephritis,  the  inflammation  is 
definitely  due  to  the  action  of  infective  agents,  usually  the  staphylococcus 
and  streptococcus,  which  are  brought  to  the  kidney  from  some  distant 
point.  The  pyogenic  microorganisms  in  question  reach  the  organ  in 
one  or  other  of  four  ways:  (1)  through  the  blood — hematogenic 
form;  (2)  through  the  urinary  tubules;  (3)  by  direct  introduction  from 
without — wound  infection;  (4)  and  by  extension  of  inflammatory  pro- 
cesses from  adjacent  parts — suppurative  nephritis  per  extensionem. 
The  most  common  methods  of  infection  are  through  the  blood  stream 
(descending  inflammation)  and  through  the  urinary  passages  (ascending 
inflammation). 

Acute  Hematogenic  Suppurative  Nephritis. — Acute  hematogenic  suppu- 
rative nephritis  is  usually  the  result  or  accompaniment  of  suppurative 
processes  elsewhere  in  the  body.  It  is,  therefore,  part  and  parcel 
of  a  generalized  septicemia.  The  most  important  predisposing  con- 
ditions to  which  this  form  of  nephritis  is  secondary,  are  ulcerative 
endocarditis,  osteomyelitis,  puerperal  sepsis,  decubitus,  pulmonary 
tuberculosis;  less  often,  typhoid  fever,  dysentery,  lobar  pneumonia, 
scarlatina,  variola,  and  actinomycosis.  The  offending  microorganisms 
reach  the  kidney  either  in  the  form  of  definite  emboli  (embolic  metastatic 
form  of  Orth),  or  singly  or  in  small  numbers,  when  they  become  en- 
tangled within  the  vessels  and  proliferate  there  (simple  metastatic  form 
of  Orth). 

In  typical  and  well-marked  cases  of  this  affection,  we  have  all  the  feat- 
ures of  an  acute  parenchymatous  inflammation  plus  the  characteristic 


752  THE  KIDNEYS 

manifestations  of  a  suppurative  process,  namely,  abscess-formation. 
Both  kidneys  are  usually  involved,  as  might  be  expected.  They  are 
swollen  and  cedematous,  and  the  capsule  peels  off  with  ease.  The  surface, 
which  is  paler  than  normal,  is  studded  with  a  variable  number  of  small 
abscesses  that  present  as  minute  elevations  of  an  opaque,  yellowish- 
white  color,  surrounded  by  a  congested  or  hemorrhagic  zone.  In  many 
instances  the  abscesses  are  not  larger  than  a  pin-head  in  size  or  even  less, 
and  are  equidistant  one  from  the  other  throughout  the  cortex.  This 
arrangement,  when  present,  should  always  suggest  the  embolic  nature  of 
the  case,  inasmuch  as  the  abscesses  resulting  from  ascending  infection 
from  the  lower  urinary  passages  are  massed  in  little  groups,  separated 
by  areas  of  comparatively  healthy  tissue,  corresponding  to  the  clusters 
of  papillary  tubules. 

On  section,  both  the  kidneys  are  found  to  be  riddled  with  abscesses 
chiefly  in  the  cortical  portion  but  also,  to  some  extent,  in  the  medulla. 
The  cortex  is  pale,  cloudy,  and  swollen  and  presents  in  the  neighborhood 
of  the  abscesses  all  the  features  of  an  acute  parenchymatous  inflammation. 
The  abscesses  in  the  cortex  are  rounded,  while  those  in  the  medulla  tend 
to  be  elongated,  following  the  course  of  the  tubules.  Exceptionally,  cer- 
tain of  the  abscesses  may  reach  the  size  of  a  hazel-nut.  In  the  simple 
metastatic  form  of  Orth,  the  abscesses  are  usually  found  in  the  pyramids 
(my colic  papillary  nephritis). 

The  histological  appearances  vary  in  different  parts  according  to  the 
age  and  intensity  of  the  infective  process.  Thus,  in  the  earliest  stage 
of  abscess-formation  we  can  detect  by  suitable  methods  of  staining 
clumps  of  bacteria  within  the  glomerular  and  peritubular  capillaries. 
In  the  immediate  vicinity  the  epithelial  cells  present  degenerative  and 
even  necrotic  changes.  When  the  lesion  is  more  matured,  numbers  of 
leukocytes  are  found  to  be  massed  within  the  capillaries,  within  the  Bow- 
man's space,  and  in  the  interstitial  stroma  in  the  neighborhood  of  the 
tufts.  When  the  abscess  is  fully  formed,  we  get  clumps  of  bacteria, 
surrounded  by  a  zone  of  necrotic  tissue,  and  bounded  externally  by  a 
mass  of  leukocytes.  In  the  immediate  vicinity  of  the  abscesses  the  kidney 
substance  is  markedly  congested  and  cedematous,  and  the  secreting 
cells  are  swollen,  cloudy,  and  degenerating.  In  the  mycotic  papillary 
form  the  appearances  are  similar,  but  the  bacteria  are  usually  found  in 
the  secreting  tubules  of  the  papillae  and  the  median  zone  of  the  pyramids, 
indicating  an  attempt  at  excretion  and  elimination  of  the  offending 
microorganisms. 

Suppurative  Pyelonephritis  and  Pyonephrosis.  —  The  type  of  the 
ascending  infection  of  the  kidney  is  the  disease  known  as  suppurative 
pyelonephritis  or  "surgical  kidney."  In  this  form  the  infective  agents 
reach  the  kidney  by  way  of  the  urine.  Inflammation  of  the  urethra 
(urethritis),  bladder  (cystitis),  ureters  (ureteritis),  or  the  pelvis  of  the 
kidney  (pyelitis)  may  all,  therefore,  be  the  immediate  precursors  of 
suppurative  nephritis.  The  liability  to  infection  is,  moreover,  greatly 
increased  by  fermentative  changes  in  the  urine,  obstruction  to  the  out- 
flow, the  presence  of  animal  parasites,  or  by  mechanical  irritation,  as 


SUPPURATIVE  PYELONEPHRITIS  AND  PYONEPHROSIS       753 

from  the  presence  of  calculi.  In  some  few  instances,  the  process  is  in 
the  first  instance  a  descending  one,  the  offending  microorganisms  being 
excreted  by  the  kidney  into  the  urine,  and  exerting  merely  a  passing 
influence  on  that  organ,  until  they  are  resorbed  from  the  lower  urinary 
passages.  Here  we  have  the  combined  effects  of  soluble  toxins  upon 
the  kidney  substance  and,  later,  of  the  actual  growth  of  the  germs 
within  the  kidney  substance. 

The  bacteria  chiefly  concerned  in  bringing  about  this  form  of  nephritis 
are  the  B.  coli,  the  various  pyogenic  cocci,  and  the  Gonococcus.  The 
descending  or  excretory  type  of  the  affection  is  met  with  in  such  diseases 
as  typhoid  fever,  scarlatina,  variola,  septicemia,  cholera.  The  -process 
may  attack  a  previously  intact  kidney  or,  again,  one  the  site  of  hydro- 
nephrosis.  The  cases  in  which  the  pelvis  of  the  kidney  is  filled  with  pus 
are  termed  suppurative  pyelit  s.  Sooner  or  later  the  kidney  substance 
becomes  involved,  a  condition  that  is  spoken  of  as  suppurative  pyelo- 
nephritis. 

In  a  case  that  is  not  too  far  advanced,  one,  and  usually  both  kidneys, 
are  swollen  and  cedematous,  soft,  and  congested.  On  section,  opaque, 
yellowish  streaks  can  often  be  made  out  in  the  pyramids  extending 
into  the  cortex,  their  long  axes  running  in  the  direction  of  the  collecting 
tubules,  bounded  by  a  hyperemic  zone.  In  the  cortex,  too,  are  similar 
areas  of  globular  shape,  aggregated  into  the  little  clusters,  and  separated 
from  each  other  by  comparatively  healthy  kidney  substance.  The  streaks 
in  question  are  due  to  the  inflammatory  products  which  have  accumu- 
lated in  the  tubules  and  the  neighboring  lymphatics.  The  opaque 
areas  in  the  cortex  are  minute  abscesses  in  the  connective-tissue  stroma 
of  those  parts  corresponding  to  the  various  lymphatic  districts. 

In  more  advanced  cases,  however,  the  abscesses  are  larger,  more 
numerous,  and  the  regional  distribution  is  not  nearly  so  evident.  When 
the  abscesses  become  confluent,  they  may  attain  a  considerable  size  and 
may  extend  through  the  greater  part  of  the  thickness  of  the  kidney. 
They  may  from  the  first  communicate  with  the  pelvis,  and  if  the 
destructive  process  be  extensive,  the  organ  may  be  converted  into  a 
fluctuating  sac  containing  pus  and  necrotic  tissue,  the  walls  of  which  are 
composed  of  the  kidney  capsule  and  shreds  of  disintegrating  kidney 
substance  (pyonephrosis).  When  the  patient  lives  long  enough  the 
smaller  abscesses  may  be  encapsulated  by  the  formation  of  fibrous 
tissue,  but  where  the  process  is  extensive  the  inflammation  may  extend 
to  the  capsule  of  the  kidney  (perinephritis)  or  even  to  the  fat  and  con- 
nective tissue  about  the  organ  (paarnephritis).  Thus,  the  whole  of  the 
secreting  substance  may  be  destroyed,  and  the  place  of  the  kidney  is 
indicated  by  a  mass  of  connective  tissue  containing  often  inspissated  pus 
and  calcareous  salts.  In  cases  due  to  calculus  we  may  find  merely  a 
fibrous  contracted  sac  filled  with  stones. 

Histologically,  it  can  be  made  out  that  the  inflammatory  process  begins 

through  infection  of  the  collecting  tubules,  beginning  in  the  pyramids 

and  extending  up  into  the  cortex.     The  kidney,  as  a  whole,  is  congested, 

and  especially  so  in  the  neighborhood  of  the  infected  areas.     The  lymph- 

48 


754  THE  KIDNEYS 

atics  are  often  distended  and  filled  with  leukocytes,  among  which,  by 
suitable  methods  of  staining,  bacteria  can  be  detected.  The  secreting 
cells  of  the  tubules  near  by  are  swollen,  cloudy,  and  often  degenerated. 
As  the  process  goes  on,  leukocytes  are  massed  at  the  part,  central  necro- 
sis of  the  infiltrated  area  takes  place  and  an  abscess  is  formed,  pushing 
aside  the  adjacent  tubules.  Later,  in  some  prolonged  cases,  a  certain 
amount  of  connective  tissue  hyperplasia  can  be  made  out  at  the  periphery 
of  the  necrotic  areas. 

Traumatic  Suppurative  Nephritis. — Traumatic  suppurative  nephritis 
may  result  from  stabbing  or  gunshot  wounds,  or,  again,  arise  in 
consequence  or  as  a  sequel  of  surgical  operations.  In  such  cases  the  in- 
fecting agents  are  introduced  directly  from  without  (wound  infection). 
The  condition  may  also  arise  from  contusion.  This,  by  lowering  the 
resisting  power  of  the  kidney,  renders  it  an  easy  prey  for  microorganisms 
which  may  reach  it  from  the  lower  urinary  passages  or  by  way  of  the 
blood  stream. 

Paranephritis. — Suppurative  nephritis  arising  per  extensionem  is  in- 
variably associated  with  paranephritis,  of  which  affection  it  forms  one 
phase.  The  infection  arises  often  from  some  quite  remote  part  and 
attacks  the  kidney  by  way  of  the  connective  and  fatty  tissue  surround- 
ing it.  The  chief  etiological  factors  are  traumatism,  lumbar  and  psoas 
abscesses,  suppuration  of  the  retroperitoneal  glands.  Among  the  rarer 
causes  may  be  mentioned  empyema,  abscess  of  the  liver,  abscess  of  the 
ligamentum  latum,  cholecystitis,  perforation  of  the  bowel,  and  para- 
typhlitis.  As  might  be  expected,  we  do  not,  in  this  affection,  find  the 
multiple  small  abscesses  so  characteristic  of  suppurative  nephritis,  but 
rather  a  single  large  abscess,  localized  to  one  part  of  the  organ.  The 
process  may  assume  the  type  of  a  gangrenous  as  well  as  a  suppurative 
inflammation. 

Besides  the  type  of  disease  just  described,  paranephritis  may  result 
from  infective  processes  originating  in  the  kidney  itself.  Thus,  sup- 
purative pyelonephritis,  with  or  without  nephrolithiasis,  is  a  most 
important  cause. 

Paranephritic  abscesses  are  often  large  and  usually  single.  Owing 
to  the  loose  nature  of  the  cellular  tissue  about  the  kidney,  they  extend 
rapidly.  In  some  few  cases,  generally  those  due  to  pyelonephritis, 
multiple  small  abscesses  develop,  which  may  undergo  absorption  or 
fuse  into  larger  ones.  Where  the  patient  survives  long  enough,  a  large 
amount  of  fibrous  scar  tissue  is  formed,  so  that  the  kidney  becomes 
embedded  in  a  dense  cicatricial  mass  of  almost  cartilaginous  hardness. 
Orth  has  recorded  a  case  of  this  kind  which  led  to  thrombosis  of  the 
renal  artery  and  necrosis  of  the  kidney.  Where  operative  interference 
is  not  resorted  to,  a  paranephritic  abscess  may  burrow  widely,  and  may 
rupture  externally  or  into  some  viscus.  It  usually  points  in  the  loin 
or  at  Poupart's  ligament.  More  rarely,  it  presents  below  the  gluteus 
maximus,  between  the  biceps  and  sartorius,  or  at  the  inguinal  ring. 
When  not  discharging  externally,  the  abscess  may  empty  into  the  colon, 
the  pleural  cavity,  or  into  the  lung,  into  the  peritoneal  cavity,  the  kidney, 


TUBERCULOSIS  OF  THE  KIDNEY  755 

pelvis,  urethra,  bladder,  and  vagina.     One  of  us  (A.  G.  N.1)  has  reported 
a  unique  case  in  which  rupture  took  place  into  the  stomach. 

Specific  Nephritis. — Tuberculosis. — Tuberculosis  of  the  kidney  is  by 
no  means  uncommon.  The  infection  is  usually  hematogenic,  but  may 
be  an  ascending  one,  arising  from  other  parts  of  the  genito-urinary 
system.  Occasionally,  it  is  due  to  the  extension  of  tuberculous  disease 
from  adjacent  structures,  as  from  the  suprarenals. 

The  affection  manifests  itself  in  two  forms,  an  acute  miliary  tuber- 
culosis and  a  chronic  local  tuberculosis.  The  first  variety  is  probably 
always  secondary,  and  is  usually  but  a  manifestation  of  a  generalized 
systemic  tuberculosis.  Occasionally,  the  primary  focus  is  quite  small,  and 
the  kidneys,  either  one  or  both,  are  the  only  organs  presenting  miliary 
involvement.  The  bacilli  reach  the  organ  through  the  renal  artery 
and  its  branches  and  are  often  entangled  in  the  .glomerular  capillaries. 
They  may,  however,  get  into  the  tubules,  evidently  as  the  result  of  an 
attempt  at  excretion  (elimination  tuberculosis  of  Cohnheim  and  Meyer). 
The  lesions  characteristic  of  this  affection  appear  as  minute  tubercles, 
or  milia,  of  a  grayish-white  color,  at  first  somewhat  pearly,  later,  dull 
and  opaque,  which  appear  usually  as  circumscribed  nodules,  but  some- 
times as  indefinite  streaks.  These  are  generally  bounded  by  a  hyperemic 
zone.  The  tubercles  may  be  few  in  number,  perhaps  restricted  to  a 
single  arterial  district,  or,  again,  are  abundant  and  scattered  throughout 
the  organ.  They  are  most  numerous  in  the  cortical  region.  Occasion- 
ally, two  or  more  may  coalesce  to  form  a  larger  focus. 

Microscopically,  the  lesion  is  that  typical  of  an  acute  tuberculosis. 
There  is  a  small  node  of  mononuclear  or  lymphoid  cells,  in  or  near 
a  glomerulus,  or  in  some  part  of  the  intertubular  connective  tissue. 
This  may  be  the  sole  manifestation.  The  older  and  larger  foci  present  in 
addition  a  small  amount  of  central  caseation.  The  vessels  in  the 
neighborhood  of  the  tubercle  are  congested,  while  those  within  the  node  are 
obstructed  by  inflammatory  products  or  by  thickening  of  the  intima. 
Inasmuch  as  the  process  is  usually  an  acute  and  terminal  one  we  do  not 
find  any  attempt  at  healing  in  the  form  of  fibrous  hyperplasia.  Giant 
cells  are  also  absent  or  scanty. 

Chronic  local  tuberculosis  of  the  kidney  is  either  hematogenic  or  ascend- 
ing in  its  origin.  One  or  both  organs  may  be  involved.  It  is  usual 
at  autopsies  to  find  both  kidneys  affected,  but  not  infrequently  the 
disease  is  much  more  advanced  in  one  than  the  other.  The  right  kidney 
is  affected  as  often  as  the  left.  A  question  that  has  given  rise  to  some 
debate  is  whether  genito-urinary  tuberculosis  is  ever  primary.  Inas- 
much as  a  tuberculous  septicemia,  without  a  local  lesion,  has  been 
demonstrated  as  a  possibility,  it  cannot  be  denied  that  primary  genito- 
urinary tuberculosis  can  occur.  It  is,  however,  never  safe  to  assume 
that  this  is  the  case,  unless  the  most  exhaustive  search  has  failed  to  reveal 
tuberculous  lesions  elsewhere.  The  further  point,  whether  in  genito- 
urinary tuberculosis  the  affection  begins  in  the  kidney,  subsequently 

1  Montreal  Med.  Jour.,  27: 1898:  119. 


756  THE  KIDNEYS 

extending  and  descending  to  the  kidney  pelvis,  ureter,  and  bladder,  or 
whether  it  originates  in  the  genitalia  or  bladder  and  travels  upward,  is 
very  difficult  to  decide.  The  preponderance  of  evidence  at  the  present 
time  favors  the  view  that  in  most  cases  the  infection  is  a  descending 
one,  the  bacilli  passing  through  the  kidney  and  setting  up  disease  else- 
where, as,  for  example,  in  the  ureters  and  bladder.  Later,  the  organisms 
are  carried  back  and  attack  the  kidneys. 

Three  main  anatomical  types  have  been  described.  The  first  and 
commonest  is  the  massive,  caseous,  or  ulcer  alive  form.  Here  the  process 
usually  begins  at  one  pole  of  the  organ,  generally  the  lower,  and  extends 
by  local  metastasis  until  the  whole  organ  becomes  involved.  At  first 
appearing  as  a  small,  grayish  nodule,  the  tubercle  enlarges,  becomes 
caseous,  and  finally  softens.  In  this  way  caseous  abscesses  are  formed 

FIG.  200 


Chronic  caseous  tuberculosis  of  the  kidney.      (From  the  Pathological  Museum  of 
McGill  University.) 

which  in  time  open  up  communication  with  the  pelvis.  The  kidney 
is  thus  converted  into  a  series  of  loculi  communicating  more  or  less  freely 
with  its  cavity  and  filled  with  a  soft,  pultaceous,  or  curdy  necrotic  material. 
These  loculi  are  separated  one  from  the  other  by  septa  formed  of  shreddy 
and  disintegrating  kidney  substance.  The  organ  is  usually  enlarged, 
although  its  shape  is  preserved.  To  the  touch  it  presents  in  places  a  soft, 
fluctuating  sensation.  Occasionally,  the  surface  will  show  a  series  of 
large  bosses  of  doughy  or  elastic  consistence,  over  which  the  capsule  is 
firmly  adherent.  On  section,  the  kidney  is  converted  into  a  number 
of  sacs,  many  of  them  communicating  with  the  pelvis,  and  extending 
up  into  the  cortex.  When  the  necrotic  material  filling  the  spaces  has  been 
washed  away,  the  walls  appear  either  smooth  or  covered  with  a  pyogenic 
membrane.  In  advanced  cases  the  kidney  may  be  totally  destroyed,  and 
is  represented  by  a  shrunken  mass,  consisting  of  a  thin  shell  of  kidney 


TUBERCULOSIS  OF  THE  KIDNEY  757 

substance,  or,  perhaps,  merely  the  capsule,  enclosing  inspissated  caseous 
material.  The  inflammatory  process  frequently  involves  the  kidney 
capsule  (tuberculous  perinephritis),  or,  again,  may  extend  to  the  sur- 
rounding connective  tissue  (tuberculous  paranephritis).  The  fibro-fatty 
tissue  surrounding  the  kidney  becomes  thereby  greatly  indurated  or 
the  seat  of  abscess-formation.  Another  sequel  of  tuberculous  nephritis 
is  that  the  inflammation  extends  to  the  mucous  membrane  lining  the 
pelvis  and  thence  to  the  ureter  and  bladder.  The  process  here  is  mani- 
fested by  the  appearance  of  scattered  elevated  tubercles  of  grayish  color 
on  the  mucous  surface.  These  are  not  uncommon  in  the  mucosa  of  the 
bladder  near  the  orifices  of  the  ureters.  In  advanced  cases  the  ureter 
may  become  obstructed  either  by  swelling  of  the  mucosa  and  thickening 
of  the  wall,  or  by  the  lodgement  therein  of  detritus.  If  there  be  not  com- 
plete destruction  of  the  secreting  portion  of  the  kidney  the  organ  becomes 
greatly  enlarged  and  distended,  owing  to  retention  of  the  urine  (tuber- 
culous pyonephrosis).  Finally,  the  destructive  inflammation  may  open 
up  the  renal  vein,  and  we  then  get  a  generalized  miliary  tuberculosis. 

In  the  second  type  of  renal  tuberculosis,  the  process  begins  in  the 
pyramids  and  leads  to  ulceration  of  the  apices  of  the  papillae.  Hematuria 
is  an  early  and  marked  symptom  in  these  cases. 

The  third  variety  is  characterized  by  the  fact  that  the  organ  is  uni- 
formly studded  writh  numerous  firm,  grayish-white  nodules,  varying  in  size 
from  that  of  a  pinhead  to  that  of  a  pea,  which  show  little  or  no  tendency 
to  necrosis.  The  capsule  is  adherent,  and  when  removed  reveals  small, 
elevated  nodules  upon  the  surface  of  the  kidney.  This  is  probably 
embolic  in  origin  and  merely  a  special  form  of  miliary  tuberculosis. 

An  affection  of  the  kidney  closely  simulating  tuberculosis,  and  due, 
as  we  know  now,  to  a  streptothrix,  was  described  by  Eppinger  in  1891, 
under  the  name  pseudotuberculosis  cladothricica. 

The  final  proof  of  the  existence  of  tuberculous  inflammation  of  the 
kidney  and  urinary  passages  is  afforded  by  the  detection  of  the  Bacillus 
tuberculosis  in  the  urine.  This  is  by  no  means  always  an  easy  task. 
The  germs  may  be  quite  few  in  number.  It  is  then  necessary  to  keep 
the  urine  for  twenty-four  hours,  allowing  it  to  deposit  in  a  suitable 
vessel,  centrifugalizing  if  necessary,  and  finally  examining  micro- 
scopically. 

A  further  difficulty  lies  in  the  fact  that  the  tubercle  bacillus  closely 
resembles  morphologically  certain  other  members  of  what  are  known  as 
the  "  acid-fast"  group  of  bacilli,  of  which  the  most  important  is  the 
smegma  bacillus,  found  in  smegma,  on  the  skin,  in  the  mouth,  and  in 
lung  cavities.  One  point  of  some  assistance  is  that  where  the  bacilli  of 
tuberculosis  are  present  in  numbers  they  are  apt  to  lie  in  fairly  large 
and  dense  clumps,  while  the  smegma  bacilli  are  more  scattered.  It  is 
possible,  however,  to  differentiate  the  two  organisms  by  means  of  certain 
staining  reactions.  It  should  be  pointed  out,  however,  in  this  connec- 
tion that  the  time-honored  Gabbett's  method  is  absolutely  unreliable, 
certainly  in  the  examination  of  urine,  and  it  would  undoubtedly  be 
safer  to  discontinue  its  use  in  the  examination  of  sputum.  A  better 


758  THE  KIDNEYS 

method  is  to  stain  in  the  ordinary  way  with  carbol-fuchsin,  decolorize 
with  30  per  cent,  mineral  acid  for  thirty  seconds,  and  then  with  abso- 
lute alcohol  for  three  minutes,  finally  counterstaining  with  methylene 
blue.  Or,  one  may  employ  1  per  cent,  rosolic  acid  in  absolute  alcohol 
for  five  minutes  as  a  decolorizer.  Even  these  methods  have  been  shown 
recently  to  be  open  to  objection.  G.  Basile  recommends  the  use  of 
a  2  per  cent,  solution  of  lactic  acid  in  absolute  alcohol.  The  tubercle 
bacillus  will  resist  decolorization  with  this  for  half  an  hour,  while 
the  smegma  and  other  acid-fast  organisms  lose  their  color  in  a  few 
minutes.1 

Another  point  of  practical  importance  is  that  the  urine  to  be  tested 
should  always  be  drawn  off  by  catheter,  after  preliminary  washing  of 
the  external  genitalia.  With  this  precaution,  the  entrance  of  the 
smegma  bacillus  is  rendered  much  less  likely. 

Syphilis. — The  manifestations  of  syphilis  in  the  kidney  are  very 
va.riable.  As  in  the  case  of  most  infectious  diseases,  we  may  get  nephritis 
of  an  acute  or  subacute  type,  which  may  result  eventually  in  chronic 
interstitial  change.  There  may  be  nothing,  however,  but  the  history  of 
the  case  to  identify  the  lesions  as  syphilitic.  Syphilitic  endarteritis  may 
lead  to  gradual  occlusion  of  the  branches  of  the  renal  artery  with  the 
formation  of  a  typical  arteriosclerotic  kidney.  According  to  Stroebe, 
intra-uterine  syphilis  may  result  in  hypoplasia  of  the  secreting  substance 
of  the  kidney  with  a  compensatory  increase  of  the  fibrous  stroma.  A 
striking  feature  is  that  the  kidney  is  imperfectly  developed.  Immature 
glomeruli  are  to  be  seen  in  the  cortex,  apparently,  in  many  cases,  without 
proper  communication  with  the  secretory  tubules. 

The  characteristic  lesion  of  syphilis,  the  gumma,  is  rare  in  the  kidney. 
Gummas  do  occur,  however,  and  may  be  fairly  numerous.  They  vary 
in  size  from  that  of  a  pin-head  to  that  of  a  hazel-nut,  and  are  surrounded 
by  a  grayish  or  hyperemic  zone.  Occasionally,  they  are  soft,  resembling 
abscesses.  As  they  heal  they  give  place  to  deep  fissures,  resulting  from 
the  contraction  of  the  fibrous  cicatricial  tissue  that  is  gradually  formed. 
The  kidney  may  thus  be  divided  into  a  series  of  lobules,  resembling 
closely  the  condition  of  things  met  with  in  the  syphilitic  liver  (so-called 
hepar  lobatum).  Bowlby2  has  described  a  diffuse  gummatous  infiltra- 
tion leading  to  a  notable  enlargement  of  the  kidney.  Miliary  gummas 
occur  but  are  extremely  rare. 

Actinomycosis. — This  affection  in  the  kidney  is  usually  secondary, 
the  primary  lesion  being  in  some  part  of  the  alimentary  tract,  mouth, 
pharynx,  or  intestine.  In  the  only  instance  we  have  met  with,  both  kidneys 
contained  small  cavities  filled  with  a  thick,  yellowish,  homogeneous- 
looking  material,  resembling  pus.  In  this  the  threads  of  the  fungus  were 
readily  demonstrated.  The  primary  lesion  was  in  the  liver,  and  the 
involvement  of  the  kidneys  was  clearly  embolic.  Israel3  has  reported 
what  he  considered  to  be  a  case  of  primary  renal  actinomycosis. 

1  Giorn.  Internaz.  d.  Scien.  Med.,  Naples,  30: 1908:  577. 

2  Trans.  Path.  Soc.  of  London,  48 : 1897 : 128. 

3  Chir.  Klin,  der  Nierenkrank.,  1901;  Handb.  der  prakt.  Chir. 


HYDHONEPHROSIS 


759 


Glanders. — Glanders  is  rare  in  the  human  kidney.  It  is  not  uncom- 
mon in  horses  affected  by  the  disease. 

Leprosy. — Chronic  parenchymatous  and  interstitial  nephritis  may  be 
found  in  cases  of  leprosy,  but  are  non-specific  so  far  as  their  anatomical 
peculiarities  are  concerned.  Amyloid  degeneration  has  also  been  de- 
scribed. In  one  instance  a  leprous  granuloma  was  found  in  the  kidney 
of  a  leper  (Hedenius;  Babes1). 


FIG.  201 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  kidneys  occurs  in  general  marasmus  and 
as  a  senile  change.  In  the  former  condition,  the  organs  are  small,  the 
perirenal  fat  is  scanty,  and  the 
secreting  cells  are  diminutive.  In 
the  senile  form,  the  kidneys  are 
small,  firm,  dark  colored,  and  the 
surface  is  finely  granular.  The 
granulation  is  due  to  atrophy  of 
the  secreting  structures  and  rela- 
tive increase  of  the  fibrous  stroma. 
In  many  cases  there  is  an  actual 
proliferation  of  connective  tissue, 
together  with  fibrosis  and  hyaline 
degeneration  of  the  glomeruli,  a 
condition  no  doubt  to  be  attrib- 
uted in  great  measure  to  the 
arteriosclerosis  so  often  present  in 
advanced  life.  Secondary  atrophy 
of  a  kidney  may  follow  obstruction 
of  the  ureter,  as  from  stone  or 
external  pressure,  nephrolithiasis, 
and  chronic  tuberculosis. 

Hydronephrosis.  -  -  Hydrone- 
phrosis  is  the  result  of  some  ob- 
struction to  the  free  evacuation  of 
urine.  This  usually  depends  upon 
the  presence  of  a  calculus  impacted 
in  the  ureter,  stricture  of  the 
ureter,  or  pressure  exerted  upon 
it  by  a  tumor  or  fibrous  band. 
Milder  grades  of  the  affection  may 
be  produced  by  an  enlarged  pros-  Hydronephrosi,  The  shell  of  kidney  sub. 

tate,    Or   Stricture   OI    the   Urethra.          stance    is    seen    to  the    left;     the    enormously 

The  Condition  begins  with  dila-       dilated    pelvis    to  the     rig  t.      The     ureter    is 

*     ,1  i    •  i  kinked     and     obstructed    by    a     fibrous     band. 

tation  of  the  pelvis   and  ureter,     (From    the   Pathological  ^useum   of  McGiii 
but  as  a  result  of  the  constantly    university.) 


Untersuchungen  iiber  den  Leprabacillus,  Berlin,  1898 :  80. 


760 


THE  KIDNEYS 


increasing  pressure,  the  kidney  substance  atrophies,  the  organ  dilates, 
and  is  eventually  converted  into  a  large,  loculated  sac,  formed  by  a 
thin  shell  of  kidney  substance,  containing  clear,  watery  fluid.  The  func- 
tion of  the  kidney  is  sooner  or  later  destroyed.  In  this  way  a  very  large 
tumor  may  be  formed  in  the  flank,  which  is  usually  crossed  in  front 
diagonally  by  a  coil  of  large  intestine. 

Degeneration. — Cloudy  Swelling  or  Albuminous  Degeneration. — Cloudy 
swelling,  or  albuminous  degeneration,  is  a  constant  occurrence  in  all 
infective  fevers,  but  particularly  in  diphtheria,  scarlatina,  typhoid, 
and  variola.  It  is  found  also  in  certain  forms  of  mineral  poisoning, 
diabetes,  hemoglobinemia,  gout,  and  is  invariably  present  in  Bright's 
disease  and  all  forms  of  local  inflammation.  Kidneys  so  affected  are 
usually  slightly  enlarged,  the  consistency  is  relaxed,  the  cortex  is  a  little 

Fio.  202 


Cloudy  swelling.  Leitz  obj.  No.  7,  without  ocular.  This  section  shows  the  swelling  of  the 
tubular  epithelium,  the  stellate  lumina,  and  the  badly-staining  nuclei.  (From  the  collection  of 
Dr.  A.  G.  Nicholls.) 

swollen  and  is  somewhat  paler  than  the  pyramids,  being  of  a  dull,  reddish- 
gray  color.  Microscopically,  the  secreting  cells  of  the  contorted  tubules 
are  the  parts  chiefly  involved.  They  are  somewhat  swollen,  turbid, 
and  the  nuclei  frequently  fail  to  stain.  The  lumina  of  the  tubules  are 
no  longer  circular  but  stellate  or  irregular.  When  a  fresh  section  is 
examined,  the  cells  are  opaque  and  granular,  being  filled  with  minute 
refractile  particles  which  obscure  the  nuclei.  When  treated  with  acetic 
acid  the  granules  disappear,  the  protoplasm  becomes  clear,  and  the 
nuclei  are  again  apparent.  The  condition  is  apt  to  pass  into  fatty 
degeneration  and  inflammation. 


PLATE  VIII 


FIG.   1 


FIG.  2 


Two   Sections  from  the   Same    Kidney  of  a   Rabbit  Treated 
with    Injections  of  Corrosive   Sublimate.      (Klotz.) 

I" ic..  1.— Section  stained  with  Sudan  III  to  demonstrate  fatty  degeneration  of  certain  tubules. 

FIG.  2.— Section  stained  with  silver  nitrate  to  demonstrate  calcareous  deposits  in  the  same  groups  of 
tubules.  (By  combined  staining  it  could  be  shown  that  the  identical  tubules  took  on  both  the  fatty  and 
the  calcareous  reactions.) 


AMYLOID  DEGENERATION  76i 

fatty  Degeneration. — Fatty  degeneration  is  a  common  sequel  of 
advanced  cloudy  swelling,  and  is  met  with  frequently  also  in  pernicious 
anemia,  acute  and  chronic  Bright's  disease,  amyloid  degeneration  of  the 
kidney,  and  in  poisoning  by  phosphorus  and  certain  other  mineral  sub- 
stances. In  this  condition  the  kidney  is  flabby,  and  in  well-marked 
cases  paler  than  normal.  The  cortex  is  the  part  chiefly  affected.  It  is 
swollen  and  of  a  uniform  pale,  yellowish  color,  or,  again,  blotched  with 
yellow,  presenting  a  marked  contrast  to  the  darker  red  of  the  medulla. 
The  fat  may  be  detected  microscopically  by  staining  the  tissue,  which  has 
previously  been  frozen  or  hardened  in  formalin,  with  Sudan  III,  or, 
again,  by  placing  it  in  Fleming's  solution.  By  the  first  method  the  fat 
appears  like  granules  or  droplets  of  a  golden-yellow  or  carmine-red 
color.  By  Fleming's  solution,  which  contains  osmic  acid,  the  fat  is 
stained  black  or  brown.  It  is  mainly  to  be  seen  in  the  secreting  cells  of 
the  contorted  tubules  and  the  lining  cells  of  Bowman's  capsules. 

Hyaline  Degeneration. — Hyaline  degeneration  affects  chiefly  the  glomer- 
uli  in  chronic  Bright's  disease.  The  globules  of  albumin  and  the 
desquamated  cells  within  the  tubules  often  fuse  into  hyaline  masses, 
thus  forming  casts. 

Amyloid  Degeneration. — This  is  a  frequent  accompaniment  of  general 
amyloid  disease.  A  local  amyloid  transformation  is  occasionally 
met  with  in  chronic  Bright's  disease.  The  condition  is  invariably 
associated  with  fatty  changes  and  diffuse  nephritis.  The  structures 
first  involved  are  the  capillaries  of  the  glomerular  tufts,  the  afferent 
arterioles,  the  interlobular  arteries,  and  the  vessels  of  the  medulla.  In 
advanced  cases  all  the  vessels  of  the  cortex,  and  even  the  basement 
membranes  of  the  tubules,  are  affected.  The  vessel  walls  are  thickened, 
presenting  a  homogeneous  translucent  appearance,  and  the  lumina  may 
become  impermeable.  In  advanced  cases  a  whole  glomerulus  may  be 
converted  into  a  structureless  mass.  The  specific  secreting  cells  show 
cloudy  swelling  and  fatty  degeneration,  while  there  is  frequently  an 
interstitial  round-celled  infiltration.  In  the  tubules  are  to  be  seen  cellular 
debris  and  hyaline-looking  casts.  It  is  doubtful  whether  the  amyloid 
casts,  so-called,  are  really  composed  of  amyloid  material. 

The  kidney  is  usually  enlarged,  very  firm,  and  of  a  consistency  sug- 
gesting india-rubber.  When  the  cut  edge  is  held  up  to  the  light  it 
presents  a  grayish,  translucent  appearance.  In  some  cases,  the  glomeruli 
are  sufficiently  enlarged  to  be  recognized  as  small,  grayish  dots.  Where 
there  is  much  fatty  change  the  kidney  may  be  pale  and  present  the 
gross  appearance  of  the  large  white  kidney,  or,  again,  it  may  resemble 
the  granular  contracted  kidney.  The  condition  may  be  recognized  in 
the  postmortem  room  by  the  application  of  a  watery  solution  of  iodine 
to  the  cut  surface  of  the  organ.  The  glomeruli  usually  then  appear 
as  small,  gelatinous-looking  points  of  a  mahogany  brown  color.  The 
test  may  fail,  however,  in  the  early  stages  of  the  disease.  In  micro- 
scopic sections  treated  with  anilin-gentian  violet  or  methylviolet,  the 
amyloid  appears  as  pinkish  masses  on  a  dull  bluish  or  greenish-blue 
background. 


762  THE  KIDNEYS 

Necrosis. — Necrosis  of  the  kidney  is  a  common  condition  due  to  the 
destructive  action  of  bacterial  or  mineral  toxins  or  the  products  of  disord- 
ered metabolism  circulating  in  the  blood.  Among  the  infective  diseases 
which  bring  it  about  may  be  mentioned  diphtheria,  scarlatina,  variola, 
septicemia,  typhoid,  and  tuberculosis.  It  is  met  with  also  in  diabetes, 
gout,  icterus,  carcinoma,  hemoglobinemia,  and  in  poisoning  with  sub- 
limate, phosphorus,  arsenic,  cantharides,  pyrogallic  acid,  and  salts  of 
chromic  acid.  A  local  necrosis  is  also  observed  in  acute  or  relapsing 
nephritis.  The  cells  chiefly  affected  are  those  of  the  contorted  tubules. 
The  cytoplasm  is  swollen,  the  nuclei  fail  to  stain,  the  lumina  are  irregular, 
and  the  whole  cell  has  a  diffuse,  opaque,  or  ground-glass  appearance. 
The  condition  closely  resembles  the  coagulation  necrosis  found  in  infarc- 
tion. In  cases  of  sublimate  poisoning,  deposits  of  lime  salts  may  be 
found  replacing  the  cells  of  the  degenerated  tubules.  The  parenchy- 
matous  degeneration  is  often  more  marked  than  in  the  case  of  nephritis, 
but  there  is  no  infiltration  of  the  connective  tissue  with  inflammatory 
products. 

Levaditi  and  Rehns,  from  Ehrlich's  laboratory,  have  described  a 
peculiar  form  of  renal  necrosis  limited  to  the  papillae,  brought  about  by 
vinylamin  and  tetrahydroquinoleme.1 


HEMATOGENEOUS  INFILTRATIONS. 

These  are  of  the  nature  of  corpuscular  elements,  pigments,  or  salts, 
brought  to  the  kidney  by  the  blood  and  deposited  either  within  the  inter- 
stitial substance  or  within  the  lumina  of  the  secreting  tubules.  Various 
soluble  salts,  the  result  of  pathological  processes  within  the  kidney  or 
elsewhere  in  the  body,  may  through  local  chemical  change  be  converted 
into  insoluble  products,  forming  the  so-called  "infarcts." 

Hemorrhage. — Hemorrhage  into  the  substance  of  the  kidney  is 
met  with  in  severe  passive  congestion,  embolism,  trauma,  certain  forms 
of  inflammation,  and  in  the  general  hemorrhagic  diathesis. 

The  effusion  takes  place  about  the  interlobular  vessels  or  into  the 
Bowman's  capsules.  Very  frequently,  the  tubules  become  filled  with 
blood  so  that  blood  casts  are  produced,  or  when  destruction  of  the  blood 
takes  place,  pigmentation  of  the  secreting  cells  occurs. 

In  acute  nephritis  and  in  the  hemorrhagic  diathesis,  small  petechial 
spots  are  seen  distributed  over  the  cortex  or  throughout  the  kidney 
substance. 

Leukocytic  Infiltration. — Leukocytic  infiltration,  apart  from  in- 
flammation, is  met  with  in  leukemia.  The  kidney  is  enlarged  and  pale, 
grayish-yellow  in  color,  due,  in  part,  to  the  leukocytic  accumulation  and 
in  part  to  the  associated  fatty  degeneration.  The  cortex  is  swollen. 
Whitish  streaks,  representing  the  overdistended  straight  vessels,  are  seen 
in  the  pyramidal  portion,  and  more  or  less  wedge-shaped  or  minute, 

1  Archives  Internat.  de  Pharmacodynamie  et  de  The"rapie,  8 : 1901 :  45  and  199. 


NEPHROLITHIASIS 


763 


FIG.  203 


rounded  areas  are  to  be  made  out  in  the  cortex,  the  so-called  "lympho- 
mata." 

Microscopically,  the  vessels  of  the  interstitial  substance  are  every- 
where greatly  distended  with  leukocytes,  so  that  the  secreting  tubules 
are  widely  dissociated,  and  there  may  be  extensive  leukocytic  infiltra- 
tion about  the  glomeruli. 

The  secreting  cells  are  found  in  all  stages  of  cloudy  swelling,  fatty 
degeneration,  and  atrophy,  owing  to  pressure  and  lack  of  nutrition. 

Pigments. — The  pigments  found  in  the  kidney  are  chiefly  those 
derived  from  the  blood,  as  hemoglobin,  methemoglobin,  hematoidin, 
hemosiderin;  bile  pigment;  melanin;  and  extraneous  substances,  like 
carbon  and  silver. 

Blood  Pigments. — The  blood  pigments  may  be  confined  to  the  vessels 
or  may  be  deposited  in  the  interstitial  substance  and  within  the  secret- 
ing cells. 

Bile  Pigments. — Bile  pigments  lead  to  a  diffuse  or  streaky  staining 
of  the  kidney  of  a  greenish  or  greenish-yellow  color.  Bile  pigment  can 
be  recognized  in  the  secreting  cells, 
which  are  often  degenerated  and  cast 
off,  so  that  a  form  of  cast  is  produced. 
Crystalline  deposits  are  seen. 

Argyriasis. — Argyriasis,  or  the  so- 
called  "silver  infarct,"  is  now  but 
rarely  seen.  The  kidney  has  a  dark 
gray  or  blackish  tint,  and  the  silver 
is  deposited  in  the  interlobular  con- 
nective tissue.  It  may  lead  to  fibroid 
changes. 

Uric  Acid. — Uric  acid  or  urates  may 
be  precipitated  within  the  kidney 
tubules  or  in  the  pelvis,  especially  in 
cases  of  gout  and  the  uric  acid  diath- 
esis, but  also  when  the  excretion  of  the 
urates  is  not  beyond  the  normal. 

Uratic  infarcts,  composed  of  acid 
sodium  urate,  are  sometimes  met 
with  in  cases  of  gout  when  they  form 
whitish  streaks  in  the  dilated  urinary 
tubules.  Similar  deposits  are  not 
infrequently  found  in  the  kidneys 
of  infants  dying  between  the  second 
and  fourteenth  day  after  birth. 
Here  the  salts  are  deposited  espe- 
cially in  the  lumina  of  the  collecting 
tubules  of  the  papillae  in  the  form  of  doubly  refractile  spheroliths. 

Nephrolithiasis. — When  the  salts  are  deposited  in  the  pelvis  of  the  kidney 
they  are  found  in  the  form  of  uratic  gravel,  or  as  calculi,  varying  in  size 
from  that  of  a  pea  to  a  large  branched  mass,  the  so-called  "coral  calculus/' 


A  coral  calculus  in  the  pelvis  of  the 
kidney.  (From  the  Pathological  Museum 
of  McGill  University.) 


764  THE  KIDNEYS 

which  may  occupy  the  whole  pelvis  of  the  organ.  The  smaller  calculi 
are  not  infrequently  found  within  the  calices,  or  may  be  impacted  in  the 
ureter. 

The  condition  often  leads  to  suppurative  pyelitis  or  pyonephrosis, 
and  the  whole  organ  may  be  destroyed,  being  finally  represented  only 
by  a  fibrous  sac  inclosing  the  stones. 

Deposits  of  carbonate  or  phosphate  of  lime  are  met  with  occasionally 
in  old  people  when  resorptive  processes  are  going  on  in  bone,  and  in 
cases  of  sublimate  poisoning  (see  vol.  i,  p.  853). 


PROGRESSIVE  METAMORPHOSES. 

The  kidney  substance  possesses  considerable  powers  of  regeneration. 
This  might  be  inferred  a  priori  from  the  fact  that  many  cases  of  acute 
inflammation  of  this  organ  heal  perfectly,  and  we  have  further  proof 
of  it  in  the  fact  that,  in  both  acute  and  chronic  nephritis  and  in  the 
neighborhood  of  wounds  and  infarcts,  the  nuclei  of  the  secreting  cells  of 
the  tubules  are  to  be  found  in  different  stages  of  mitosis.  Whether  these 
reparative  powers  are  sufficient  to  reproduce  whole  tubules  and  glomeruli 
is  still  in  question.  It  seems  probable,  however,  that  this  may  be  possible 
in  young  developing  individuals  of  certain  of  the  lower  animals.  As 
age  advances,  the  power  of  growth  inherent  in  the  cells  becomes  notice- 
ably weaker. 

Compensatory  Hypertrophy. — Compensatory  hypertrophy  is  found 
in  cases  where  one  kidney  is  congenitally  defective,  has  been  removed 
by  operation,  or  is  inefficient  from  disease.  The  remaining  kidney 
resembles  closely  the  normal  organ  save  that  it  is  much  larger  and  its 
cortex  is  somewhat  broader.  It  rarely,  however,  attains  the  weight  of 
two  normal  kidneys  combined.  The  pyramids  are  not  increased  in 
number.  Such  an  organ  is  specially  liable  to  disease,  inasmuch  as  its 
reserve  power  is  small.  Whether  the  condition  is  a  true  hypertrophy, 
or  not  rather  a  hyperplasia,  is  still  unsettled. 

Tumors. — For  purposes  of  description  it  is  convenient  to  classify 
tumors  of  the  kidney  according  to  their  histological  appearance.  On 
this  basis  we  may  recognize  tumors  of  epithelial  type,  meaning  by  this 
the  various  forms  of  adenoma  and  carcinoma,  and  those  of  connective- 
tissue  type,  which  would  include  such  forms  as  the  fibroma,  myoma, 
lipoma,  myxoma,  angioma,  and  sarcoma.  It  should  be  remarked,  how- 
ever, that  the  various  new-growths  of  the  kidney  have  much  more  in 
common  than  have  epithelial  and  connective-tissue  neoplasms  occurring 
elsewhere,  inasmuch  as  the  kidney,  both  in  its  secreting  mechanism 
and  supporting  framework,  is  derived  entirely  from  the  mesoblast.  We 
must,  therefore,  give  the  term  carcinoma,  if  we  apply  it  to  the  kidney, 
a  histological  sense,  rather  than  a  developmental  one.  In  addition, 
not  a  few  of  the  kidney  tumors  are  of  mixed  type  and  may  properly  be 
classed  as  teratoid  in  nature. 

The  commonest  tumors  of  the  kidney  are  the  sarcoma  and  carcinoma, 


TUMORS 


765 


the  former  being  somewhat  more  frequently  found  than  the  latter.  The 
benign  growths  are  usually  small  and  insignificant,  though  large  lipomas 
have  been  described  by  Warthin  and  others. 

Adenoma. — The  adenoma,  a  benign  tumor  of  epithelial  type,  varies 
in  size  from  that  of  a  millet-seed  to  that  of  a  walnut,  or  even  larger, 
and  is  usually  found  in  the  cortex.  It  forms  single  or  multiple,  soft, 
well-defined  nodules  of  whitish  color.  Histologically,  an  alveolar,  a 
tubular,  and  a  papillary  form  can  be  recognized.  The  cells  forming 
the  tumor  are  of  columnar  type.  Occasionally,  the  tubules  are  dilated 
into  cysts — cystadenoma.  Some  of  the  tumors  formerly  classed  with 
cystadenomas  may  possibly  have  been  hypernephromas  (q.  v.). 


FIG.  204 


Compensatory  hypertrophy:  R.  Kidney,  congenital  hypoplasia:  L.  Kidney,  compensatory 
hypertrophy  (length,  14.5  cm.);  Norm.,  a  normal  adult  kidney  (length,  11.0  cm.).  (Outlines 
made  to  scale  from  specimens  in  McGill  Medical  Museum.) 

Fibre  mas. — Fibromas  are  rather  common  in  the  kidney  but  are  insig- 
nificant in  importance.  They  form  small  masses,  from  a  microscopic 
nodule  to  one  the  size  of  a  pea,  rarely  larger. 

Leiomyomas  and  Fibroleiomyomas. — Leiomyomas  and  fibroleiomyomas, 
composed  of  unstriped  muscle,  or  an  admixture  of  muscle  and  connective- 
tissue  fibers,  are  met  with,  but  are  rare.  They  may  be  found  both  in  the 
cortex  and  in  the  pyramids. 

Rhabdomyomas  are  rare  -also. 


766  THE  KIDNEYS 

Lipomas. — Lipomas  are  found  beneath  the  capsule  and  in  the  cortex 
of  the  kidney.  They  are  single  or  multiple,  and  usually  of  small  size, 
although  exceptions  to  this  rule  occur.  Histologically,  they  are  encap- 
sulated and  consist  of  ordinary  adipose  cells. 

Certain  of  the  renal  fatty  tumors  are  considered  by  Grawitz  to  be  con- 
genital and  referable  to  overgrowths  of  misplaced  suprarenal  tissue 
(strumce  lipomatodes  aberrates  renis).  The  Grawitz  tumors  differ  from 
the  others,  however,  in  that  the  component  cells  contain  multiple  fat 
droplets  and  resemble  the  cells  of  the  suprarenal  cortex.  They  are  not 
true  fat  cells. 

Myxomas. — Myxomas  are  rare.  Most  of  the  growths  described  as 
myxomas  are  more  probably  to  be  regarded  as  connective-tissue  growths 
that  have  undergone  secondary  mucinous  degeneration.  Bezold  and 
Hollen,  however,  have  each  reported  a  case  of  true  myxoma. 

Chondromas  and  osteomas  have  been  met  with,  but  are  extremely 
rare. 

Angiomas,  more  correctly  teleangiectases,  are  occasionally  found. 
They  may  be  situated  in  the  pyramids  or  pelvis.  When  projecting  into 
the  cavity  of  the  kidney  they  may  give  rise  to  serious  hemorrhage. 

Sarcoma. — Sarcoma  is  the  most  common  tumor  found  in  the  kidney. 
It  is  met  with  both  in  childhood  and  in  adult  life.  New-growths,  often 
termed  sarcomas,  are  comparatively  common  in  early  life  and  may 
even  be  congenital.  Careful  study  of  these,  however,  will  show  that 
the  vast  majority  of  them  are  of  mixed  type,  containing  striped  muscle, 
cartilage,  or  bone.  They  are,  therefore,  more  properly  included  under 
the  teratoid  new-formations. 

Round-celled  and  spindle-celled  sarcomas  are  described,  the  former 
being  often  highly  vascular.  Alveolar  angiosarcoma,  really  either  an 
endothelioma  or  perithelioma,  is  occasionally  met  with.  Endotheliomas 
may  arise  from  the  lining  cells  either  of  bloodvessels  or  lymphatics. 
Giant  cells  are  at  times  found  in  renal  sarcomas. 

Carcinoma. — Carcinoma  of  the  kidney  is  somewhat  more  common 
on  the  right  side  than  on  the  left,  and  is  more  frequent  in  men  than  in 
women.  It  may  arise  from  the  secreting  cells  of  the  tubules  or  from  the 
epithelium  lining  the  pelvis.  It  is  generally  held  that  the  adenoma 
has  a  distinct  tendency  to  develop  into  carcinoma.  Carcinoma  of 
distinctly  glandular  type  is  known  as  adenocarcinoma. 

It  is  somewhat  olifficult  to  draw  the  line  between  the  simple  adenoma 
and  the  carcinoma.  Certain  authors,  notably  Pilliet,  Sottas,  and 
Albarran,  hold  that  tumors  which  histologically  resemble  pure  ade- 
nomas behave  at  times  like  malignant  growths,  as  evidenced  by  local 
infiltration  and  the  formation  of  metastases.  The  same  peculiarity  has 
been  observed  in  adenomas  elsewhere. 

Primary  carcinoma  may  be  nodular  or  diffuse,  and  is  of  the  scirrhous, 
simple,  or  medullary  type.  The  nodular  forms  are  well-defined,  and 
are  provided  often  with  a  more  or  less  perfect  capsule.  The  diffuse 
forms  lead  to  a  generalized  enlargement  of  the  organ  without  much 
deformity.  Carcinomas  frequently  attain  a  considerable  size,  but  are 


THE 

UNIVERSITY 

TERATOIDS  767 

not  usually  so  large  as  the  sarcomas.  The  growth  may  extend  into  the 
pelvis  and  ureter.  Degenerative  changes  not  infrequently  occur  in  the 
centre  of  the  growths,  leading  to  hemorrhagic  extravasation,  cyst-forma- 
tion, and  sometimes  calcareous  deposit.  Hematuria  is,  therefore,  a  not 
uncommon  manifestation  of  the  disorder. 

Carcinoma  is  much  more  often  secondary  in  the  kidney  than  primary. 
It  may  follow  cancer  of  the  testicle,  liver,  stomach,  uterus,  mamma, 
pancreas,  or  of  the  other  kidney. 

Sarcomas  may  also  form  metastatic  deposits  in  the  kidneys.  The 
melanotic  sarcoma  is  always  secondary. 

Teratoids. — The  teratoid  or  embryonal  mixed  tumors  of  the  kidney 
form  a  most  interesting  study.  They  include  the  large  majority  of  the 
so-called  sarcomas  and  carcinomas  found  in  childhood.  Careful  in- 
vestigations have  shown,  however,  that  they  have  distinct  features  of 
their  own  and  should  be  placed  in  a  class  by  themselves.  Inasmuch  as 
they  have  been  found  at  birth  or  shortly  after,  it  has  been  thought 
that  they  are  due  to  developmental  errors,  probably  being  present  in  a 
latent  form  at  a  very  early  period  of  life,  and  being  subsequently  excited 
into  activity  under  the  influence  of  some  stimulus,  notably  trauma. 
Very  exceptionally,  they  are  met  with  in  adults.  The  left  kidney  is 
involved  more  often  than  the  right,  but  both  organs  may  be  primarily 
affected.  These  tumors  often  attain  a  great  size,  one  being  on  record 
which  weighed  thirty-six  pounds.  The  general  shape  of  the  kidney  is 
not  greatly  altered,  though  the  surface  may  be  somewhat  nodular.  The 
kidney  substance  proper  usually  forms  a  more  or  less  complete  shell 
about  the  tumor.  On  section,  the  growth  may  be  homogeneous  and  of 
a  grayish-pink  color,  but  it  is  common  to  find  areas  of  degeneration, 
cysts,  and  hemorrhagic  extravasation  into  its  substance.  Extension 
usually  takes  place  into  the  veins  and  the  lungs  are  usually  secondarily 
involved. 

The  histological  structure  is  often  highly  complicated,  and  may  vary 
considerably  in  different  tumors,  and  in  different  parts  of  the  same  tumor. 
In  general,  it  may  be  said  that  there  are  to  be  seen  more  or  less  abundant 
epithelial  cells,  arranged  in  masses  or  in  tubules,  and  of  glandular  type, 
together  with  a  somewhat  cellular  stroma,  composed  of  round  or  spindle 
cells.  This  peculiarity  of  structure  has  led  to  these  growths  being 
termed  adenosar comas.  According  to  the  predominance  of  one  or  other 
type  of  cell,  however,  these  mixed  tumors  may,  at  one  time,  closely  re- 
semble the  adenomas  and  carcinomas,  with  which  they  have  often  been 
confounded,  and,  at  another,  the  sarcomas.  In  addition  to  the  features 
mentioned,  the  majority  contain  fibers  of  striped  and  unstriped  muscle 
(rhabdomyoma,  rhabdomyosarcoma,  myosarcoma),  islets  of  hyaline  carti- 
lage, and  even,  it  is  said,  ganglion  cells,  thus  indicating  their  teratomatous 
nature.  Rarely,  epithelial  "pearls"  surrounded  by  muscle-fibers  have 
been  observed. 

Considerable  divergence  of  opinion  has  been  expressed  in  regard  to 
the  origin  of  these  growths.  The  presence  of  striated  muscle-fibers  in 
many  of  them  suggested  the  idea  that  they  were  due  to  the  development 


768  THE  KIDNEYS 

of  misplaced  embryonic  tissue,  notably  portions  of  the  Wolffian  body. 
It  is  possible,  however,  as  Wilms  and  others  think,  that  the  fibers  in 
question  are  derivatives  of  the  primitive  myotomes  of  the  embryo. 

In  the  category,  also,  of  tumors  derived  from  embryonic  "rests"  is 
the  form  conveniently  termed  by  Birch-Hirschfeld  the  hypernephroma. 
This  is  a  tumor  believed  by  many  competent  pathologists  to  be  derived 
from  misplaced  suprarenal  tissue  (Grawitz,  Lubarsch,  Gatti,  Kelly). 
Accessory  suprarenals  or  misplaced  portions  of  suprarenal  tissue  have 
been  found  in  a  great  variety  of  situations,  such  as  the  immediate 
neighborhood  of  the  normal  site  of  the  suprarenal,  in  or  beneath  the 
capsule  of  the  kidney,  about  the  renal  vessels,  in  the  spermatic  cord, 
between  the  testis  and  epididymis,  in  the  inguinal  canal,  in  the  broad 
ligament,  in  the  solar  plexus,  the  cceliac  ganglion,  and  even  in  the  liver 
and  pancreas.  Tumors  may  originate,  conceivably,  therefore,  from  the 
proliferation  of  suprarenal  tissue  in  any  of  these  situations. 

The  most  common,  and  by  far  the  most  important,  of  such  new- 
growths  are  those  found  in  connection  with  the  kidney.  They  are  met 
with  usually  at  the  upper  end  of  the  organ,  in  the  cortex  just  beneath 
the  capsule.  The  growth  is  well  circumscribed,  and  usually  bounded 
by  a  more  or  less  perfect  connective-tissue  capsule.  It  is  made  up  of  a 
series  of  nodes,  varying  somewhat  in  size,  divided  one  from  the  other  by 
connective-tissue  septa,  and  composed,  in  turn,  of  a  series  of  smaller 
nodules,  which  contain,  as  a  rule,  but  little  connective  tissue.  Hyper- 
nephromas  are  moderately  firm,  of  a  yellowish,  yellowish-white,  or 
brownish-yellow  color.  There  is  a  marked  tendency  to  retrograde 
metamorphosis,  necrosis,  fatty  change,  hemorrhage,  and  cyst-formation. 
Rarely,  there  is  calcification.  As  they  grow  they  gradually  replace  the 
kidney  substance,  but  are  usually  bounded  by  a  zone  of  distorted  and 
compressed  renal  cells.  The  connective-tissue  capsule  is  often  invaded 
by  the  proliferating  cells,  but  the  remains  of  it  can  generally  be  detected 
on  careful  examination.  This  constitutes  one  of  the  characteristic 
features  of  the  hypernephroma.  The  larger  growths  tend  to  penetrate 
into  the  pelvis  of  the  kidney  and  invade  the  renal  veins.  Metastasis 
thus  takes  place  through  the  blood  circulation. 

Histologically,  the  structure  of  these  tumors  is  highly  complicated,  and 
it  is  not  surprising,  therefore,  that,  until  Grawitz  pointed  out  their  true 
nature,  many  of  them  had  been  described  variously  as  lipomas,  adenomas, 
carcinomas,  sarcomas,  adenosarcomas,  angiosarcomas,  endotheliomas, 
and  peritheliomas.  The  microscopic  examination  will,  as  a  rule,  reveal 
a  connective-tissue  capsule,  which  in  the  smaller  growths  is  often  com- 
plete and  in  the  larger  ones,  although  more  or  less  infiltrated,  can  usually 
be  traced  here  and  there.  In  brief,  a  hypernephroma  may  be  described 
as  a  tumor  composed  of  a  stroma  formed  of  a  rather  close  meshwork 
of  capillary  vessels,  and  of  cells  arranged  in  rows,  columns,  or  clusters 
closely  associated  with  these  capillaries.  The  character  and  arrangement 
of  the  cells  recalls  more  or  less  perfectly  the  appearance  of  the  cortex  of 
the  suprarenal  and  of  tumors  arising  therefrom.  This  resemblance  is 
most  striking  in  the  case  of  the  smaller  hypernephromas.  The  larger 


HYPERNEPHROMA 


769 


growths  are  apt  to  be  alveolar  in  type,  the  component  nodules  being 
separated  one  from  the  other  by  a  small  amount  of  connective  tissue, 
in  the  form  of  trabeculse  continuous  with  the  capsule.  The  endothelium 
lining  the  capillaries  is  usually  quite  distinct  and  may  even  be  proliferated. 
The  alveolar  appearance  may  cause  the  tumor  to  be  mistaken  for  a 
carcinoma  or  a  sarcoma.  The  tumor-cells  proper,  lying  between  the 
capillary  vessels,  are  arranged  in  rows  or  double  rows,  like  the  cells  of 
the  zona  fasciculata  of  the  suprarenal  cortex,  and  are  directly  continuous 
with  the  cells  of  the  vascular  endothelium.  They  are  of  epithelial  type, 
usually  polygonal,  but  may  be  rounded,  cubical,  columnar,  or  irregular, 
and  frequently  contain  multiple  vacuoles,  spaces  which  originally  con- 
tained fat.  The  fatty  infiltration  of  the  cells  is  a  noteworthy  feature  of 
the  hypernephroma.  Glycogen  is  also  commonly  present  in  relatively 
great  amount.  A  black  pigment  is  also  to  be  found,  similar  to  that 
normally  present  in  the  suprarenal  gland. 


FIG.  205 


FIG.  206 


fl." 


Section  of  portion  of  a  hypernephroma  of 
the  kidney.  A  characteristic  area  showing 
columns  of  clear  polygonal  cells:  a,  lying  in 
immediate  apposition  to  the  endothelium  (d) 
of  the  capillary  sinuses  (c).  At  6,  areas  of 
infiltration  and  degeneration. 


Section  from  another  portion  of  the  same 
tumor,  more  highly  magnified,  showing 
tubular  arrangement:  a,  swollen  translucent 
tumor  cells  surrounding  a  definite  lumen 
6,  capillary  c,  fat  droplets  in  tumor  cells. 
(Buday.) 


Besides  the  alveolar  form,  a  tubular  or  trabecular  arrangement  is 
occasionally  met  with;  or,  again,  the  type  may  vary  in  different  parts  of 
the  growth.  Tumors  of  great  complexity  may  thus  be  formed. 

Dermoid  Cysts. — Dermoid  cysts  have  been  found  in  the  kidney,  but 
are  very  rare. 

Parasites.— Among  the  animal  parasites  described  as  at  times  infesting 
the  kidney  may  be  mentioned  the  Echinococcus,  the  Cysticercus  cellulosce, 
the  Distoma  hematobium,  the  Eustrongylus  gigas,  the  Filaria  sanyuinis, 
and  the  Pentastomum  denticulatum.. 
49 


770  THE  URINARY  PASSAGES 

THE  PELVIS  OF  THE  KIDNEY  AND  URETERS, 

CONGENITAL  ANOMALIES. 

These  have  been  discussed  above  (see  p.  728). 

CIRCULATORY  DISTURBANCES. 

(Edema. — (Edema  is  found  associated  with  passive  congestion  and 
inflammation. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  of  the  mucous 
membrane  lining  the  pelves  and  ureters  is  met  with  in  cases  of  inflam- 
mation and  where  irritating  substances  have  been  excreted  by  the 
kidneys. 

Passive  Hyperemia. — Passive  hyperemia  is  observed  in  the  condition 
of  general  systemic  passive  congestion,  and  also  in  obstruction  to  the 
outflow  of  blood  from  the  renal  veins. 

Hemorrhage. — Hemorrhage  results  from  inflammation,  passive  con- 
gestion, ulceration,  traumatism,  the  hemorrhagic  diatheses,  parasites,  and 
tumors.  In  certain  cases,  also,  of  acute  and  chronic  nephritis,  blood 
may  escape  into  the  urinary  passages. 

ALTERATIONS  IN  THE  LUMINA  OF  THE  URINARY  PASSAGES. 

Narrowing  of  the  lumen  of  the  ureter  may  occur,  and  leads  to  a  more 
or  less  complete  obstruction  to  the  outflow  of  the  urine.  A  variety  of 
causes  may  bring  it  about.  Chief  among  these  are,  inflammatory  thick- 
ening of  the  mucosa,  the  presence  of  granulomata,  and  fibroid  induration 
of  the  ureteral  wall.  Owing  to  inflammatory  changes,  the  mucous 
membrane  lining  the  pelvis  may  encroach  upon  the  orifice  of  the  ureter 
after  the  fashion  of  a  valve,  and  lead  to  obstruction.  Swelling  of  the 
mucosa  of  the  bladder  may  also  shut  off  the  opening  of  the  ureter. 
Strictures  of  the  ureter  may,  however,  be  congenital  as  well  as  acquired. 
Possibly  both  are  inflammatory  in  their  origin. 

The  lumen  may  be  obstructed  by  foreign  substances  lodging  within 
it.  Calculi,  blood  clots,  necrotic  tissue,  portions  of  tumors,  and  parasites 
are  to  be  mentioned  in  this  connection. 

Pressure  from  without  is  also  an  important  etiological  factor.  It 
may  be  due  to  tumors,  enlarged  glands,  a  retroflexed  uterus,  or  an  over- 
filled rectum  or  bladder  in  a  contracted  pelvis,  inflammatory  bands, 
ligatures,  a  horseshoe  kidney,  an  anomalous  renal  artery,  or  an  accumu- 
lation of  extravasated  urine. 

Traction  upon  the  ureter,  and  kinking  or  torsion  from  a  movable 
kidney,  sometimes,  also,  bring  about  obstruction. 


PYELITIS  771 

The  effect  of  such  obstruction  is  the  same  in  all  cases.  The  urine 
is  dammed  back,  and  that  portion  of  the  urinary  passage  proximal 
to  the  point  of  obstruction  is  dilated.  When  the  pelvis  of  the  kidney 
is  distended  with  clear,  watery  fluid,  usually  a  modified  urine,  the  condi- 
tion is  known  as  hydronephrosis.  Hydronephrosis  is  more  likely  to 
occur  when  the  obstruction  is  brought  about  slowly  or  is  due  to  inter- 
mittently acting  causes.  Both  kidneys,  but  more  often  only  one,  may 
be  affected. 

In  a  moderately  advanced  case,  the  pelvis  is  dilated  and  the  kidney 
substance  is  somewhat  compressed  and  atrophic.  The  condition  will, 
under  ordinary  circumstances,  go  on  increasing  until  the  external 
pressure  is  equal  to  that  of  the  secretion.  In  time  the  kidney  may  be 
greatly  enlarged,  being  converted  into  a  thin  sac  containing  perhaps, 
several  liters  of  fluid.  The  fluid  in  question  is  low  in  specific  gravity 
(1002  to  1012),  contains  little  or  no  albumin,  and  is  deficient  in  urea, 
chlorides,  and  phosphates.  Microscopically,  it  may  contain  leukocytes, 
red  blood  corpuscles,  and  cholesterin.  Should  infection  take  place  the 
contents  become  intermingled  with  pus  (pyonephrosis).  (See  also  p.  759.) 

Solutions  of  continuity  of  the  ureters  are  usually  due  to  traumatism, 
ulceration,  or  new-growths. 


INFLAMMATIONS. 

Inflammation  of  the  pelvis  of  the  kidney  is  called  pyelitis ;  of  the  ureter, 
ureteritis. 

Pyelitis. — Pyelitis  most  commonly  results  from  an  ascending  infec- 
tion, microorganisms  reaching  the  pelvis  of  the  kidney  from  the  lower 
urinary  passages  by  way  of  the  urine.  The  possibility  of  this,  in  spite 
of  the  downward  current  of  the  urine,  has  been  amply  demonstrated. 
Any  condition  which  interferes  with  the  free  outflow  of  the  urine  and 
brings  about  its  decomposition  would  naturally  aid  in  the  production 
of  pyelitis.  Retention  of  urine,  cystitis,  and  stricture  of  the  urethra, 
are  the  conditions  of  most  importance  in  this  connection.  It  should 
be  mentioned,  however,  that  in  these  cases  the  inflammatory  process 
need  not  progress  by  contiguity,  for  pyelitis  may  result  from  a  preexisting 
cystitis  without  the  ureters  being  involved.  Moreover,  although  the 
infecting  agents  may  have  developed  within  the  bladder,  they  may  infect 
the  pelvis  of  the  kidney  without  setting  up  a  cystitis. 

Pyelitis,  again,  may  be  the  result  of  a  hematogeneous  infection  or  of 
inflammatory  processes  involving  the  kidneys  themselves.  In  this  form 
the  various  infections  and  intoxications  play  an  important  role.  Local 
irritation  within  the  pelvis  may  also  bring  about  pyelitis,  such  as  may 
be  produced  by  stones  and  parasites. 

In  rare  cases,  pyelitis  may  be  due  to  the  extension  of  inflammation 
from  some  of  the  parts  about  the  kidney. 

Although  in  many  instances  pyelitis  is  toxic  or  irritative  in  nature,  it 
is  usually  not  long  before  infection  is  superadded.  The  microorganisms 


772  THE  PELVES  AND  URETERS 

most  often  at  fault  are  the  B.  coli,  the  various  pyogenic  cocci,  and  the 
Gonococcus,  more  rarely  the  B.  proteus. 

Pyelitis  is  usually  bilateral.  Even  if  the  process  at  first  be  localized 
to  one  kidney  pelvis,  as  in  the  case  of  stone,  it  is  not  uncommon  for  the 
infective  agents  to  make  their  way  into  the  other  ureter  and  attack  the 
opposite  kidney.  According  to  the  anatomical  changes  produced,  we 
may  recognize  a  catarrhal,  a  suppurative,  a  membranous,  and  a  gangrenous 
pyelitis.  The  form  produced  depends  largely  upon  the  nature  and 
intensity  of  the  infection  and  the  mechanical  conditions  subsisting. 

In  catarrhal  pyelitis,  the  mucous  membrane  lining  the  pelvis  is 
swollen,  congested,  and  may  present  minute  hemorrhages.  The  urine 
contains  mucus,  leukocytes,  and  desquamated  epithelial  cells. 

In  the  purulent  form,  the  inflammation  is  more  intense  and  the  urine  is 
distinctly  purulent.  In  some  cases,  as  above  mentioned,  the  pelvis,  and 
even  the  kidney,  may  be  distended  with  pus  (pyonephrosis).  In  cases  of 
stone,  in  which  pressure  is  exerted  upon  the  inflamed  mucosa,  ulcera- 
tion  and  gangrene  may  result. 

Membranous  pyelitis  is  the  result  of  a  particularly  virulent  infection. 

In  cases  of  pyelitis  that  have  lasted  some  time,  the  mucosa  is  reddened 
and  thickened,  and  is  studded  with  a  number  of  grayish  prominences. 
These,  according  to  some  observers,  are  lymphadenoid  in  nature,  and 
are  due  either  to  hyperplasia  of  previously  existing  lymph-nodes  or  to  a 
new-formation  of  lymphoid  tissue  as  a  result  of  the  inflammation.  In 
other  cases  small  cysts  are  present  (pyelitis  cystica).  In  very  chronic 
pyelitis,  and  especially  in  the  tuberculous  variety,  the  lining  membrane 
becomes  thickened  and  horny,  and  of  a  pearly  white  color,  resembling 
cholesteatoma. 

Ureteritis. — Ureteritis  is,  in  most  respects,  similar  to  pyelitis,  and 
its  characters  may,  therefore,  be  inferred  from  what  has  just  been  said. 

Tuberculosis. — Miliary  tuberculosis  of  the  pelvis  of  the  kidney,  appar- 
ently hematogenic  in  origin,  has  been  met  with,  but  is  rare.  The  usual 
type  is  chronic  and  caseating  in  character.  This  form  usually  may  be 
traced  to  the  influence  of  a  tuberculous  kidney,  but  is  sometimes,  also, 
the  result  of  an  ascending  infection  from  the  lower  urinary  passages  or 
genitalia.  We  find  small,  elevated  tubercles  in  the  mucosa  which  tend 
to  coalesce.  These  are  often  found  in  the  upper  part  of  the  ureter  and 
near  its  entrance  into  the  bladder.  In  time  they  lead  to  thickening  of 
the  mucous  membrane  with  encroachment  upon  the  lumen  and  more  or 
less  obstruction  to  the  free  outflow  of  urine.  Ultimately  the  wall  of  the 
ureter  becomes  greatly  thickened  and  ulceration  takes  place.  In  an 
advanced  case,  we  have  seen  the  whole  of  the  urinary  passages,  from  the 
point  of  the  penis  to  the  kidney,  infiltrated  and  thickly  studded  with 
coarse  tubercles  tending  to  become  confluent. 

Parasites. — The  more  important  parasites  are  the  Filaria  sanguinis 
hominis,  the  Distoma  hematobium,  the  Eustrongylus  gigas,  and  the 
Ascaris  lumbricoides.  Filaria  in  the  urinary  passages  cause  hematuria. 
Chyluria  is  also  a  characteristic  symptom  of  filariasis. 


TUMORS  773 


RETROGRESSIVE  METAMORPHOSES. 

The  only  condition  worthy  of  note,  coming  under  this  category,  is 
ulceration.  This  is  sometimes  the  result  of  inflammation,  but  is  per- 
haps more  often  due  to  the  presence  of  a  calculus  (^pressure  necrosis). 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — The  most  frequently  occurring  tumor  of  the  pelvis  of  the 
kidney  is  the  villous  papilloma.1  It  may  give  rise  to  serious,  even  fatal, 
hemorrhage.  Malignant  papillomatous  epitheliomas  have  been  met  with, 
but  are  rare.2  Primary  carcinoma  has  been  observed,  but  is  rare.3  It  is 
occasionally  found  associated  with  the  presence  of  stones,  suggesting  an 
etiological  relationship.  Lymphosarcoma  of  the  pelvis  is  described.4  An 
adenoma  of  the  ureter  has  been  met  with.  Inasmuch  as  this  structure 
contains  no  glands,  it  has  been  supposed  that  the  tumor  originates  from 
remains  of  the  Wolffian  duct.  Hektoen5  has  recorded  an  instance  of 
primary  carcinoma  of  the  ureter. 

Sometimes  the  mucous  membrane  lining  the  urinary  passages  is 
the  site  of  multiple  small  cysts,  a  condition  called,  possibly  erroneously, 
pyelitis,  ureteritis,  cystitis,  urethritis  cystica,  as  the  case  may  be.  These 
have  been  thought  by  some  to  originate  in  downgrowths  of  the  superficial 
epithelium.  Others  think  that  they  are  due  to  proliferation  of  the  sub- 
epithelial  connective  tissue,  with  elevation  of  the  mucosa.  The  cysts 
develop  in  the  angle  thus  formed  by  the  prominence  and  the  general 
surface.  Still  others  would  attribute  them  to  the  activity  of  parasites. 

1  Savory  and  Nash,  Lancet,  London,  2:  1904:  1699;  Busse,  Virchow's  Archiv,  164: 
1901:  119. 

2  Kelly,  Proc.  Path.  Soc.  Phila.,  (N.  S.)  3:  1900:  217. 

3  Hektoen,  Jour.  Amer.  Med.  Assoc.,  26:  1896:  1115. 

4  White,  Trans.  Path.  Soc.  London,  49: 1898: 178. 

5  Jour.  Amer.  Med.  Assoc.,  26:  1896:  1115. 


CHAPTER    XXXVI. 

THE  BLADDER  AND  URETHRA. 

THE  BLADDER. 
CONGENITAL  ANOMALIES. 

THE  development  of  the  bladder  may  be  interrupted  at  any  stage  of 
its  progress.  Complete  defect  of  the  bladder  has  been  reported.  In  this 
case,  the  ureters  discharge  into  the  urethra.  Occasionally,  the  septum 
dividing  the  rectum  from  the  bladder  is  wholly  or  partially  lacking,  so 
that  the  ureters  and  the  rectum  empty  into  a  large  cloaca.  This  is  some- 
times associated  with  imperf orate  anus. 

Extroversion. — The  most  common  anomaly,  however,  is  extrover- 
sion. Here,  there  is  failure  of  union  between  the  two  halves  of  the 
body  along  the  median  ventral  line.  The  anterior  wall  of  the  bladder 
and  the  corresponding  portion  of  the  abdominal  wall  are  wanting. 
The  pubic  bones  are  also  often  separated  by  a  considerable  interval. 
The  condition  is  much  more  common  in  males  than  in  females.  Extro- 
version in  the  female  is  apt  to  be  associated  with  prolapse  or  procidentia 
of  the  uterus.  In  rarer  cases,  the  bladder  is  complete  but  prolapsed 
through  an  abdominal  fissure  (ectopia  vesicce). 

Diverticula. — Diverticula  may  be  found  near  the  point  of  junction 
with  the  urachus. 

Small  cysts  of  the  urachus  are  common. 


DISLOCATIONS. 

The  bladder  may  be  displaced  upward,  downward,  or  to  one  side. 
The  pregnant  uterus  in  the  course  of  its  enlargement,  tumors  of  the 
pelvis  and  pelvic  organs,  and  intestinal  adhesions,  may  carry  or  drag 
the  bladder  upward. 

Downward  dislocation  is  the  most  common  and  important  form.  It 
is  due  to  muscular  relaxation,  sagging  of  the  pelvic  floor,  destruction 
of  the  perineal  body,  and  malpositions  of  the  uterus.  Cystocele  is  a 
downward  pouching  of  the  floor  of  the  bladder.  Extroversion  of  the 
bladder  through  the  urethra  (female)  is  rare,  and  is  met  with  chiefly  in 
young  children.  It  is  sometimes  caused  by  the  prolapse  of  a  tumor 
of  the  trigone,  which  passes  into  and  through  the  urethra,  eventually 
dragging  the  bladder  after  it. 

Lateral  dislocation  may  be  caused  by  inflammatory  infiltration  or 
adhesions,  tumors;  in  rare  instances,  the  bladder  has  formed  part  of  the 


CYSTITIS  775 

contents  of  the  sac  in  inguinal  and  femoral  hernia.  It  is  hardly  necessary 
to  say  that,  for  anatomical  and  physiological  reasons,  dislocations  of 
the  bladder  are  almost  confined  to  the  female  sex. 


CIRCULATORY  DISTURBANCES. 

These  are  not  of  much  practical  importance. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  is  nearly  always 
an  inflammatory  manifestation,  and  is  met  with  in  cases  where  the 
urine  contains  irritating  substance,  as,  for  instance,  cantharidin,  or 
where  there  is  extension  of  inflammation  from  the  neighboring  parts. 

Passive  Hyperemia. — Passive  hyperemia  is  a  frequent  accompaniment 
of  general  systemic  congestion.  The  vessels,  particularly  those  of  the 
trigone  and  neck  of  the  bladder,  are  turbid  or  even  varicose,  standing 
out  in  marked  contrast  with  the  otherwise  pale  mucosa.  This  appear- 
ance is  all  the  more  characteristic  since  the  normal  vesical  mucosa  is 
strikingly  pale.  Passive  congestion  often  leads  to  cedema  of  the  bladder 
wall  and  finally  catarrh  of  the  mucosa. 

Hemorrhages. — Hemorrhages,  either  in  the  form  of  petechice  or 
suggillations,  are  met  with  in  cystitis,  ulceration,  tumors,  and  the  hemor- 
rhagic  diatheses. 

INFLAMMATIONS. 

Cystitis. — Inflammation  of  the  bladder — cystitis — is  of  frequent  occur- 
rence, and  is  due,  in  the  vast  majority  of  cases,  to  the  extension  of 
inflammation  from  other  parts  of  the  urinary  passages  (kidney,  etc.), 
or  to  abnormalities  in  the  contents  of  the  viscus.  Gonorrhceal  urethritis 
and  pyelitis  are  examples  of  the  first  class.  Of  the  second,  decomposing 
urine,  calculi,  and  foreign  bodies  may  be  cited.  The  irritation  set  up 
is  usually  aggravated  by  secondary  infection. 

Obstruction  to  the  outflow  of  urine,  as  in  stricture  of  the  urethra  or 
enlarged  prostate,  is  a  common  cause,  not  only  from  the  stagnation  of 
the  urine,  but  on  account  of  infection  which  invariably  takes  place. 
A  distended  bladder  is  particularly  liable  to  bacterial  invasion,  since  its 
resisting  power  is  diminished,  and  the  retained  urine,  undergoing  as  it 
does  marked  chemical  changes,  acts  as  a  direct  irritant  to  the  mucous 
membrane.  Moreover,  certain  bacteria,  which  reach  the  bladder  from 
outside,  induce  various  forms  of  fermentation  and  chemical  decomposi- 
tion so  that  the  original  disturbance  is  aggravated  and  perpetuated. 
The  microorganisms  in  question  reach  the  bladder  from  the  urethra,  or 
from  parts  adjacent  to  the  bladder.  They  are  also  introduced  through 
careless  instrumentation  or  the  use  of  a  dirty  catheter. 

The  urine  in  cystitis  is  often  alkaline  and  has  a  fcetid  ammoniacal 
odor.  The  reaction  and  the  fermentative  processes  going  on  depend, 
however,  on  the  nature  of  the  offending  microorganism.  The  ordinary 
ammoniacal  decomposition  of  the  urine  is  brought  about  by  the  Micro- 
coccus  urese.  When  the  B.  coli  is  present  alone,  according  to  Schmidt 


776  THE  URINARY  BLADDER 

and  Aschoff,  the  urine  is  acid.  If  the  urine  contain  staphyloccci, 
either  alone  or  associated  with  the  B.  coli,  the  reaction  is  alkaline. 

In  a  few  instances,  cystitis  can  be  traced  to  hematogenic  infection  of 
the  action  of  a  circulating  toxin,  or  to  the  extension  of  an  inflammatory 
process  in  adjacent  organs,  like  the  uterus  and  rectum. 

Cystitis  is  acute  or  chronic. 

Acute  Cystitis.— Catarrhal  Cystitis.— The  mildest  form  of  the  acute 
affection  is  catarrhal  cystitis.  As  people  do  not  die  from  this  disease, 
the  condition  is  only  discovered  in  the  routine  examination  of  those  who 
have  died  from  other  causes.  At  autopsy,  th  re  may  be  surprisingly 
little  evidence  of  its  presence,  even  where  the  signs  were  clear  during 
life.  At  most,  there  is  slight  redness  and  swelling  of  the  mucosa,  and 
any  urine  present  contains  a  little  mucus,  with  a  few  leukocytes  and 
some  degenerated  epithelium.  Small  grayish  blebs  may  be  found  about 
the  neck  and  trigone  (herpes  vesiccB). 

Acute  Suppurative  Cystitis. — A  more  serious  disturbance  is  acute 
suppurative  cystitis.  Here,  the  mucous  membrane  is  reddened  and 
swollen,  particular  y  about  the  fundus  and  trigone,  and  on  the  surface 
of  the  rugae.  If  the  urine  has  been  alkaline,  a  macerating  process  has 
been  going  on  and  the  epithelium  is  swollen,  soft,  and  desquamating  in 
large  flakes.  In  this  detritus  phosphates,  carbonates,  and  other  urinary 
salts  are  often  deposited,  giving  the  mucosa  a  dirty  whitish  or  whitish- 
brown,  gritty  appearance,  not  unlike  mortar.  Pus  can  frequently  be 
squeezed  out  of  the  lacunas,  and  hemorrhagic  patches  may  be  seen  here 
and  there. 

The  suppurative  process  may  extend  into  the  bladder  wall,  infiltrating 
the  interstitial  tissue  and  undermining  the  muscle,  which  may  thus  be 
dissected  off  and  float  free  in  the  vesical  cavity  in  the  form  of  soft, 
friable  tags.  When  the  suppuration  extends  diffusely  throughout  the 
muscular  wall,  the  condition  may  be  spoken  of  as  phlegmonous  cystitis. 
The  process  may,  however,  extend  still  deeper  to  the  surrounding 
connective  tissue — paracystitis,  or  even  to  the  peritoneum — pericystitis. 
Abscesses  of  considerable  size  may  form  in  the  perivesical  cellular  tissue. 
Provided  that  the  patient  survive,  they  may  become  circumscribed  and 
heal,  leading  to  fibrous  induration.  Perforation  of  the  abscess  into  the 
peritoneal  cavity,  intestine,  and  vagina  has  been  recorded. 

Membranous  Cystitis. — A  third  form  is  the  so-called  membranous 
cystitis.  This,  in  many  cases,  is  brought  about  by  chemical  changes  in 
the  urine,  particularly  by  the  action  of  ammonium  carbonate,  which 
produces  swelling,  desquamation,  and  maceration  of  the  tissues.  The 
condition,  however,  also  occurs  in  certain  of  the  infective  fevers,  typhoid, 
cholera,  the  exanthemata,  pyemia,  diphtheria,  dysentery,  and  in  second- 
ary carcinoma  derived  from  the  uterus.  The  bladder  is  intensely  con- 
gested and  of  a  deep  red  hemorrhagic  appearance,  while  upon  the  surface 
of  the  rugse,  particularly  in  the  posterior  part,  there  is  a  whitish-gray 
membrane,  more  or  less  firmly  adherent.  This  membrane  is  liable  to  be 
infiltrated  with  urinary  salts.  Microscopically,  the  mucosa  presents 
coagulation  necrosis. 


TUBERCULOSIS  777 

Chronic  Cystitis. — Chronic  cystitis  may  result  from  the  acute  form, 
or  may  be  chronic  from  the  start.  Depending  upon  the  cause,  the 
bladder  is  distended  and  thin-walled,  or  contracted  and  hypertrophic. 
The  mucous  membrane  is  usually  much  thickened,  and  lies  in  deep 
folds,  or  polypoid  outgrowths  may  be  formed.  The  mucosa  is  sometimes 
greatly  reddened,  but  more  often  is  of  a  slaty  color,  showing  incrustation 
with  salts  and  slight  superficial  erosion.  The  lymph-follicles  may  be 
enlarged  or  increased  in  numbers,  so  that  they  become  visible  and 
give  the  surface  a  granular  appearance. 

Syphilis. — Syphilis  but  rarely  affects  the  bladder;  little  or  nothing 
is  known  of  luetic  ulceration  in  this  locality. 

Tuberculosis. — The  mucous  membrane  of  the  urinary  bladder  is  nor- 
mally quite  resistant  to  tuberculous  infection.  Active  bacilli  may,  for 
a  long  time,  be  brought  in  contact  with  it  without  inducing  lesions.  A 
preexisting  cystitis  will,  however,  lessen  this  relative  immunity.  Vesical 
tuberculosis  is  rarely,  if  ever,  hematogenic,  but  the  infection  is  carried 
through  the  medium  of  the  urine  from  the  upper  urinary  passages,  or, 
much  less  often,  reaches  the  viscus  by  extension  from  the  genitalia. 
That  tuberculosis  of  the  bladder  is  rarer  in  femaLs  than  in  males  is 
explained  by  the  fact  that  genital  tuberculosis  is  not  so  often  met  with 
in  the  former.  In  the  male,  tuberculosis  of  the  kidney  or  of  the  prostate 
is  the  usual  cause.  Only  rarely  is  the  tuberculosis  primary,  and  in 
such  cases  the  bacilli  are,  possibly,  derived  from  the  blood,  but  more 
probably  from  the  outside  through  the  urethra.  That  the  urethra 
is  uninvolved  does  not  exclude  this  possibility,  for  it  is  particularly 
refractory  to  tuberculous  infection. 

Anatomically,  there  are  two  forms,  multiple  milia  and  the  caseous  ulcer. 

The  miliary  variety  takes  the  form  of  minute  grayish  nodules  situated 
just  beneath  the  epithelial  layer  of  the  mucosa.  In  descending  infection 
these  are  usually  most  thickly  grouped  about  the  orifices  of  the  ureters. 
In  the  ascending  form  they  are  found  at  the  trigone  and  neck  of  the 
bladder.  The  milia  are  surrounded  by  a  reddish  zone.  The  larger 
ones  present  central  caseation,  but  the  smaller  can  only  be  distinguished 
from  hyperplastic  lymph-follicles  by  microscopic  examination. 

When  the  nodules  coalesce,  large  granulomas  are  produced  which 
undergo  necrosis  and  ulceration.  These  tend  to  be  restricted  to  the 
mucosa,  and  spread  laterally  rather  than  into  the  muscular  wall.  It 
may  be  difficult  at  first  to  recognize  them  as  tuberculous,  since  the  bases 
may  be  quite  clean  and  free  from  necrotic  and  caseous  material.  Only 
in  the  more  extreme  cases  are  large  tuberculous  masses  produced,  with 
irregular  and  fissured  surfaces,  which  may  undermine  the  mucosa.  As 
in  the  case  of  other  ulcers,  the  surface  is  often  infiltrated  with  urinary 
salts. 

Secondary  tuberculosis  of  the  bladder,  derived  from  organs  not  in 
direct  contact  or  communication  with  the  viscus,  is  somewhat  rare. 
It  does,  however,  occur,  through  the  mediation  of  the  peritoneum. 
Tuberculosis  of  the  small  intestine,  cecum,  appendix,  or  Fallopian 
tubes,  plays  a  leading  role  here. 


778  THE  URINARY  BLADDER 

Parasites  and  Abnormal  Contents. — The  urine  which  reaches  the 
bladder  may  be  abnormal  in  its  secretion,  or  may  become  contami- 
nated by  contact  with  the  urinary  passages,  or,  again,  may  undergo 
chemical  decomposition  when  retained  within  the  bladder. 

Erythrocytes  or  their  derivatives  may  be  found  in  the  urine  in  certain 
cases  of  nephritis;  in  congestion  of  the  urinary  organs  and  passages;  in 
acute  inflammation  or  ulceration  of  the  pelvis  of  the  kidney,  ureter, 
or  bladder;  in  certain  intoxications;  and  in  the  hemorrhagic  diatheses. 
Tumors,  like  angiomas  or  carcinomas,  may  bleed  freely.  Blood  clots 
or  blood  casts  may  be  found. 

Leukocytes  result  from  inflammation  in  any  part  of  the  urinary  tract. 
They  may  form  casts. 

Epithelium  from  the  pelvis  of  the  kidney  and  bladder  is  often  met 
with,  and  is  of  considerable  importance  in  diagnosis. 

In  ulcerative  processes,  simple  or  cancerous,  necrotic  tissue  and  detritus 
may  be  found. 

Urinary  casts  are  derived  from  the  tubules  of  the  kidneys  in  various 
forms  of  nephritis.  They  may  be  hyaline  and  colorless,  amyloid  (colloid), 
fatty,  granular,  epithelial,  leukocytic,  fibrinous,  or  composed  of  red 
blood  cells.  Casts  should  be  carefully  differentiated  from  cylindroids, 
which  are  of  no  practical  importance. 

Bacteria  of  various  kinds  are  of  frequent  occurrence.  They  are  de- 
rived either  from  the  urethra,  or  are  eliminated  through  the  kidney. 
According  to  most  authorities  normal  urine  is  aseptic.  Enriquez1 
has  made  a  careful  study  of  this  point.  Whether  a  physiological  excre- 
tion of  microorganisms  through  the  kidney  is  possible,  in  the  absence  of 
a  lesion  of  the  secreting  epithelium,  is  still  a  moot  question.  The  majority 
of  observers,  following  Wyssokowitsch  and  Neumann  and  Konjajeff, 
seem  to  think  it  does  not  occur,  but  Schweitzer2  and  certain  others  hold 
that  bacteria  may  pass  the  renal  epithelium  in  the  absence  of  any  lesions 
that  it  is  possible  to  recognize  microscopically. 

The  chief  pathogenic  bacteria  found  in  the  urine  in  diseased  conditions 
are  the  B.  coli,  B.  typhi,  B.  tuberculosis,  staphylococcus,  streptococcus, 
Diplococcus  pneumonias,  Gonococcus,  and  B.  Friedlanderi. 

Yeasts  are  met  with  in  the  urine  of  diabetics. 

A  variety  of  foreign  bodies  have  been  found  in  the  urine.  In  fistulous 
communications  with  the  rectum,  feces  and  gas  may  enter  the  bladder. 
A  dermoid  cyst  may  open  into  the  organ  and  hairs  may  be  passed  in 
the  urine  (pilimictio). 

In  the  case  of  children  and  those  addicted  to  masturbation,  and  in 
attempts  to  relieve  itching,  foreign  bodies  of  all  kinds  have  been  passed 
into  the  urethra  and  may  slip  into  the  bladder.  Among  these  may  be 
mentioned  catheters,  hairpins,  hatpins,  needles,  matches,  straws,  candles, 
handles  of  parasols  or  toothbrushes,  glass.  Foreign  bodies  may  form  the 
nucleus  for  the  formation  of  concrements. 

1  Recherches  bact.  sur  1'urine  normale,  Semaine  m^dicale,  No.  57 : 1891 : 468. 
2Virchow's  Archiv,  110:  1887:255. 


PROGRESSIVE  METAMORPHOSES  779 

Concrements  and  calculi  are  produced  in  the  pelvis  of  the  kidney  or 
in  the  bladder  (see  vol.  i,  p.  864).  Stones  in  the  kidney  are  usually 
composed  of  urates,  uric  acid,  oxalates,  or  combinations  of  these  with 
phosphates.  Vesical  calculi  are  commonly  phosphatic.  It  is  obvious, 
however,  that  stones  of  varying  character  may  reach  the  bladder  from 
the  renal  pelvis  and  form  a  nucleus  for  a  much  larger  phosphatic 
calculus.  Certain  rare  forms,  cystin,  xanthin,  and  silicates,  may  just  be 
mentioned. 

The  parasites  found  are  the  Echinococcus,  Filaria,  Distoma,  and,  in 
cattle,  the  larvce  of  certain  flies. 

RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  bladder  is  met  with  in  old  age,  especially 
in  women,  and  in  marantic  and  cachectic  states.  The  mucosa  is  thinned, 
but  the  muscular  coat  is  the  portion  chiefly  involved.  The  bladder  wall 
is  sometimes  reduced  to  the  thickness  of  paper.  Long-continued  dis- 
tention,  such  as  is  met  with  in  paralysis  of  the  muscle,  is  an  important 
cause  of  atrophy. 

Necrosis. — Necrosis  of  the  bladder  wall,  at  times  leading  to  perfora- 
tion, is  commonly  due  to  injuries  during  parturition,  either  from  instru- 
mentation or  the  pressure  of  the  foetal  head.  It  may  also  result  from  the 
pressure  of  a  large  calculus. 

Fatty  and  amyloid  changes  are  common. 

PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Hypertrophy  of  the  vesical  muscle  is  of  frequent 
occurrence.  According  as  the  cavity  of  the  bladder  is  contracted  or 
dilated,  we  can  recognize  a  concentric  and  an  excentric  hypertrophy. 

The  most  common  cause  is  some  obstruction  to  the  free  outflow  of 
urine,  such  as  is  brought  about  by  an  enlarged  prostate,  stricture  of  the 
urethra,  the  pressure  of  a  prolapsed  uterus,  tumors  of  the  uterus  or 
bladder,  or  an  impacted  calculus.  A  second  form,  without  urinary 
obstruction,  is  found  in  cases  of  chronic  cystitis,  vesical  calculus,  and 
tumors.  Here,  it  is  supposed  that  the  constant  irritation  leads  to  in- 
creased activity  of  the  motor  nerves  and  functional  overwork  of  the 
muscle.  As  in  the  case  of  the  heart,  hypertrophy  may  in  time  give 
place  to  dilatation,  notably  in  the  obstructive  cases. 

Dilatation  of  the  bladder  with  hypertrophy  of  its  wall  is  frequently 
met  with  in  certain  affections  of  the  spinal  cord,  locomotor  ataxia,  mye- 
litis, and  the  like.  In  such  cases  there  can  be  no  question  of  obstruc- 
tion or  of  reflex  irritation.  The  dilatation  might  be  accounted  for 
on  the  score  of  diminished  sensibility  and  consequent  retention,  but  we 
may  consider  also  that  the  hypertrophy  is  due  to  some  trophic  disturb- 
ance of  the  sympathetic  nervous  system  or  of  the  nerves  in  the  muscular 
wall. 


780  THE  URINARY  BLADDER 

In  pure  hypertrophy  the  bladder  wall  is  thickened  and  of  firm  con- 
sistence. The  muscular  bands  of  the  inner  surface  are  greatly  enlarged, 
recalling  the  columnse  carnese  of  the  cardiac  ventricles.  When  obstruc- 
tion has  been  operative,  it  is  not  uncommon  to  find  smaller  or  larger 
sacculations  or  diverticula,  either  due  to  local  weakening  of  the  bladder 
wall,  or  to  hernial  protrusions  of  the  mucosa  through  weak  spots  in  the 
muscular  coat.  These  diverticula  are  most  frequent  at  the  fundus  of 
the  bladder,  while  the  trigone  is  usually  free. 

FIG.  207 


Hypertrophy  and  dilatation  of  the  urinary  bladder  due  to  an  enlarged  prostate.  The  saccula- 
tion  of  the  bladder  is  well  shown  and  also  the  encroachment  of  the  middle  lobe  of  the  prostate 
on  the  urethra.  (From  the  Pathological  Museum  of  McGill  University.) 

Tumors. — Primary  tumors  are  not  common.  Benign  vesical  growths 
are  rather  more  common  than  the  malignant.  In  640  cases  given  by 
Watson,1  60  per  cent,  were  non-malignant.  Males  are  more  frequently 
attacked  than  females,  the  proportion  being  about  3  to  2.  As  a  rule, 
the  base,  the  posterior  wall,  or  both,  are  involved. 

Papilloma. — The  most  common  growth  is  the  papilloma,  which  is 
often  benign  but  has  a  distinct  tendency  toward  malignancy,  and  hence 
may  develop  into  a  papillary  carcinoma.  It  appears  as  a  soft,  villous 
mass  attached  to  the  mucosa  by  a  fibrous  pedicle  of  varying  thickness. 

1  Morrow's  System  of  Genito-urinary  Diseases,  1 : 1893 : 565. 


TUMORS  781 

When  floated  out  in  water  the  tumor  has  a  shaggy,  tree-like  appearance. 
The  growth  is  generally  reddish,  but  may  present  paler  areas,  due  to 
necrosis  or  superficial  erosion. 

Microscopically,  the  tumor  consists  in  a  number  of  connective-tissue 
cores,  rather  rich  in  bloodvessels,  which  are  covered  with  simple  or 
stratified  polymorphous  or  columnar  cells.  If  the  tumor  be  malignant, 
the  wall  of  the  bladder  is  infiltrated  with  a  soft,  brain-like  substance,  from 
which  a  milky  juice  can  be  obtained  on  scraping.  Under  the  micro- 
scope, the  mucosa  and  muscularis  are  found  to  contain  masses  of  epi- 
thelial cells,  resembling  those  covering  the  papillae  of  the  original  growth. 

Fibroma. — Fibromas  or  fibrous  polyps  are  rather  more  uncommon. 
They  often  show  myxomatous  degeneration. 

Myoma  and  Fibromyoma. — Myomas  and  fibromyomas  are  among  the 
rarest  of  bladder  tumors.  They  vary  in  size  from  that  of  a  pea  to  that 
of  a  child's  head,  and  may  be  pedunculated  or  sessile. 

Adenoma. — Adenomas  are  quite  rare.  They  are  sessile  or  peduncu- 
lated, and  have  a  smooth,  lobulated,  or  papillary  surface.  They 
probably  arise  from  the  mucous  crypts. 

Angiomas  and  teratomas  are  excessively  rare. 

Carcinoma. — Carcinoma  originates  from  the  epithelium  lining  the 
bladder  or  that  of  the  mucous  crypts.  It  often  forms  a  rather  superficial, 
diffuse  growth,  projecting  only  slightly  into  the  cavity  of  the  bladder,  or 
presents  a  nodose,  somewhat  elevated  surface.  The  mucous  membrane 
is  sometimes  intact,  or  may  be  eroded  and  ulcerated.  Flattened  ulcers 
with  indurated  edges  may  be  formed.  In  other  cases,  as  before  men- 
tioned, carcinoma  takes  the  form  of  a  papillary  outgrowth,  or  cauliflower- 
like  mass.  Vesical  carcinomas  are,  as  a  rule,  somewhat  slow  growing, 
and  do  not  tend  to  invade  the  deeper  structures.  Metastases,  when 
present,  are  usually  strictly  local. 

Sarcoma. — Sarcomas  are  commonly  multiple,  sessile,  with  a  smooth 
surface.  In  color  they  are  red,  purplish,  or  almost  black.  They  are 
extremely  rare.  Myosarcoma,  chondrosarcoma,  and  osteoid  chondro- 
sarcoma1  have  been  recorded. 

Secondary  tumors,  usually  carcinomatous,  are  more  frequent  than 
primary  ones.  In  the  male  they  originate  in  the  prostate;  in  the  female, 
in  the  uterus  or  vagina.  In  both  sexes,  carcinoma  of  the  rectum  may 
extend  to  the  bladder. 


THE  URETHRA. 

CONGENITAL  ANOMALIES. 

Absence  of  the  urethra  is  met  with  associated  with  other  grave  defects. 
The  urethra  may  divide,  so  that  it  discharges  by  two  or  more  openings. 
In  the  male  the  passage  may  open  at  the  base  of  the  scrotum  instead  of 

1  Shattock,  Trans.  Path.  Soc.,  38:  1887:  183;  also  Beneke,  Arch.  f.  path,  Anat.  u, 
Phys.  u.  f.  klin.  Med.,  161: 1900:  70, 


782  THE  URETHRA 

passing  through  the  corpus  spongiosum,  and  in  the  female  it  may 
empty  into  the  vagina.  Local  obliteration  of  the  urethra  may  be  met 
with  at  the  meatus  and  in  other  parts,  due  to  defective  development  of 
the  corpus  spongiosum.  Valve-like  membranes  also  lead  to  partial 
or  complete  obstruction  of  the  canal. 

CIRCULATORY  DISTURBANCES. 

What  has  already  been  stated  with  regard  to  the  bladder  applies  with 
equal  force  to  the  urethra. 

In  females,  varices,  or  urethral  hemorrhoids,  are  met  with,  forming 
small  caruncles  or  polypoid  masses.  They  may  give  rise  to  serious 
hemorrhage  or  to  submucous  hematomas. 

INFLAMMATIONS. 

Urethritis. — Simple  Urethritis. — Simple  urethritis  is  commonly 
brought  about  by  irritation  from  unclean  habits,  careless  instrumen- 
tation, injections  of  fluids,  foreign  bodies,  calculi,  or  direct  violence. 
In  the  female,  inflammations  of  the  vulva  and  vagina  not  infrequently 
extend  to  the  urethra. 

A  simple  urethritis,  analogous  to  inflammation  of  other  mucous 
membranes,  may  arise  in  the  course  of  the  various  infective  fevers. 
It  is  also  believed  to  be  produced  by  coitus  with  a  woman  suffering  from 
a  leucorrhoeal  discharge,  or  who  is  menstruating. 

Gonorrhosal  Urethritis. — The  most  important  affection  is  specific 
urethritis  or  gonorrhoea,  which  is  due  to  a  particular  microorganism, 
the  Gonococcus  of  Neisser.  Gonorrhceal  urethritis,  as  a  primary 
disease,  is  more  frequent  in  males  than  in  females.  In  the  latter  it  is 
more  liable  to  spread  by  extension  from  a  previous  infection  of  the  vulva 
or  vagina.  The  condition  is  brought  about  by  contact  with  infective 
secretion  from  a  mucous  membrane,  usually  by  coitus,  although  instances 
of  mediate  contagion  are  not  uncommon.  Thus  the  infecting  agents 
may  be  carried  by  the  fingers,  towels,  sponges,  or  bed-linen. 

The  specific  microorganism  is  a  micrococcus,  usually  lying  in  pairs, 
the  opposed  surfaces  of  which  are  slightly  concave.  Single  cocci  and 
tetrads  are  also  met  with.  They  are  often  intracellular,  but  are  also 
found  lying  free.  They  stain  readily  with  aniline  dyes  and  are  decolor- 
ized by  Gram's  method.  Owing  to  the  fact  that  certain  other  micro- 
organisms, namely,  the  Trichomonas  vaginalis  and  some  aberrant  forms 
of  the  B.  coli,  resemble  Gonococci  rather  closely,  diagnosis  should  not 
be  made  from  stained  films  only,  unless  the  history  and  clinical  signs 
are  clear.  In  doubtful  and  especially  in  medicolegal  cases,  culture 
methods  should  also  be  resorted  to. 

Gonorrhoea  usually  starts  near  the  meatus  and  rapidly  spreads  to  the 
rest  of  the  anterior  urethra.  After  the  first  week  the  inflammation  may 
spread  to  the  posterior  urethra  and  the  prostate. 


PLATE  IX 


V- 


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Gonococci  in  pus. 


GONORRH(EAL   URETHRITIS  783 

The  disease  is  a  severe  purulent  catarrh,  leading  at  first  to  intense  con- 
gestion of  the  urethra,  followed  by  the  production  of  a  profuse  yellowish 
or  greenish-yellow  discharge,  occasionally  mixed  with  blood.  The  pre- 
puce and  glans  are  often  inflamed  (balanoposthitis),  and  there  may  be 
paraphimosis.  In  severe  cases  mild  constitutional  symptoms  arise, 
together  with  painful  erections  of  the  penis  (chordee).  The  secretion 
consists  of  pus,  blood,  and  desquamated  epithelial  cells,  and  contains 
the  specific  microorganisms. 

Microscopically,  the  superficial  epithelium  is  desquamating,  and 
leukocytes  can  be  seen  passing  between  the  cells  to  the  surface,  or  infil- 
trating the  periurethral  connective  tissue.  The  various  lacunae  and 
periurethral  glands  are  usually  distended  with  pus. 

After  a  few  weeks  the  intensity  subsides,  as  a  rule,  and  the  process  may 
end,  even  in  the  absence  of  treatment,  in  healing.  This  is,  however, 
not  often  the  case,  since  the  affection  tends  to  become  chronic,  and  certain 
complications  may  set  in.  If  the  inflammation  extend  to  the  prostatic 
gland,  the  disease  becomes  very  obstinate.  Ulceration  or  abscesses 
in  the  periurethral  connective  tissue  occur,  and  the  various  glands — 
prostatic,  Cowper's,  Tyson's,  and,  in  the  female,  Bartholin's  glands — 
may  retain  infective  pus  long  after  the  urethra  is  free.  This  local  suppu- 
ration may  be  due  to  the  Gonococcus  alone,  or  to  other  germs  associated 
with  it.  More  rarely,  acute  orchitis  or  epididymitis  results.  In  the 
female,  the  vagina,  particularly  about  the  cervix  uteri,  is  apt  to  be  involved. 
Gonorrhceal  endometritis  is  met  with,  but  more  often  the  uterus  escapes, 
while  the  Fallopian  tubes  become  diseased.  A  few  cases  of  gonorrhceal 
peritonitis,  due  to  extension  of  infection  from  the  tubes,  have  been 
reported.  In  both  sexes  the  bladder  is  not  infrequently  attacked, 
although  the  kidneys  are  but  rarely  involved.  Among  the  most  serious 
complications  are  conjunctivitis,  adenitis  and  periadenitis  (bubo), 
arthritis  and  tenosynovitis,  endocarditis,  and  septicemia. 

Gonorrhoeal  arthritis  and  tenosynovitis  usually  manifest  themselves 
weeks  or  months  after  the  first  infection,  and  are  most  obstinate  condi- 
tions. Suppuration  in  the  joints  and  fibrous  ankylosis  are  not  un- 
common. 

Chronic  gonorrhoea  is  usually  the  continuation  of  an  acute  attack  in 
a  less  florid  form.  It  may  be  catarrhal,  hyperplastic,  or  indurative. 

The  catarrhal  form  resembles  the  acute.  The  exudation,  however, 
is  less,  in  parts  there  are  superficial  erosions  of  the  epithelium,  and  the 
cylindrical  cells  may  be  converted  into  squamous  ones.  The  various 
crypts  and  glands  may  contain  pus  or  desquamated  cells,  and  show 
evidences  of  deeply  seated  inflammation.  Occasionally,  in  addition  to 
catarrh,  the  mucosa  is  thickened  and  studded  with  warty  or  polypoid 
excrescences. 

An  important  type  practically  is  the  indurative,  in  which  dense  fibrous 
tissue  is  produced,  leading  frequently  by  its  contraction  to  stricture  of 
the  urethra. 

Strictures  may  be  single  or  multiple,  and  are  usually  found  in  the 
membranous  urethra,  although  the  penile  portion  may,  at  times,  be 


784  THE  URETHRA 

affected.  The  condition  is  a  slowly  developing  one  and  is  important 
on  account  of  the  serious  disturbances  to  which  it  gives  rise,  obstruction 
to  the  free  discharge  of  urine,  hypertrophy  of  the  bladder  with  dilatation, 
hydronephrosis,  pyonephrosis,  rupture  of  the  urethra,  and  extravasation 
of  urine.  Behind  the  stricture  the  urethra  is  generally  in  a  state  of 
chronic  catarrh.  In  chronic  urethritis  there  is  usually  a  slight  discharge, 
generally  in  the  morning.  It  is  not  necessarily  purulent,  but  is  more  of 
a  mucous  nature  (gleet).  In  this  form,  the  urine  may  contain  a  few 
flocculent  shreds,  upon  which  may  be  detected  occasional  leukocytes  and 
gonococci. 

Membranous  Urethritis. — Membranous  urethritis  is  rare. 

Of  localized  inflammatory  lesions  may  be  mentioned  the  soft  chancre 
and  the  primary  syphilitic  sore.  They  are  found,  not  rarely,  just  within 
the  meatus,  to  which  they  give  a  peculiar  square  appearance  in  transverse 
section.  These  infections  may  be  inoculated  at  the  same  time  as  a 
gonorrhoea . 

Variolous  pustules  have  been  met  with  in  the  urethra. 

Among  the  chronic  inflammations  may  be  mentioned  polypoid  or  warty 
excrescences  (condylomas),  due  to  dirt  or  irritating  discharges.  They 
are  usually  found  at  the  meatus. 

Tuberculosis. — Tuberculosis  of  the  urethra  is  not  common.  Local 
foci  of  caseous  necrosis  are  sometimes  met  with  in  the  prostatic  portion, 
due  to  extension  from  the  bladder  and  prostate,  and  in  women,  at  the 
anterior  portion,  in  cases  of  lupus  of  the  vulva.  We  have  seen  the 
urethra  studded  with  coarse  granular  tubercles  along  its  whole  length, 
where  the  kidneys  and  urinary  tract  were  extensively  involved. 

Foreign  Bodies  and  Parasites. — What  has  already  been  remarked 
when  dealing  with  the  bladder  applies  also  to  the  urethra.  The  most 
important  foreign  bodies  are  calculi,  colloid  masses,  and  portions  of  bone, 
all  of  which  may  lead  to  obstruction. 

Of  the  parasites  may  be  mentioned,  by  way  of  curiosity,  the  Penicillium 
glaucum,  the  Eustrongylus  gig  as,  and  the  larvce  of  certain  flies.  In 
females,  especially  in  young  children,  thread  worms  may  reach  the 
urethra  from  the  anus,  and  set  up  pruritus  and  marked  irritation. 


RETROGRESSIVE  METAMORPHOSES. 

Ulceration  may  take  place  from  the  action  of  caustics  or  from  injuries 
inflicted  during  parturition  or  instrumentation. 


PROGRESSIVE  METAMORPHOSES. 

Tumors. — Primary  tumors  are  rare.  They  are  more  common  in  men 
than  in  women. 

In  females,  small  excrescences  or  caruncles  are  sometimes  found,  caused 
by  thickening  or  hypertrophy  of  the  normal  folds  of  the  mucosa. 


INJURIES  785 

Simple  retention  cysts,  arising  from  the  periurethral  glands,  are  occa- 
sionally met  with.  They  may  also  start  from  the  Cowper's  glands. 
Orth  has  recorded  a  cystadenoma. 

Fibrous  polyps  have  been  described. 

Carcinoma  is  rare.  It  may  originate  in  the  Cowper's  glands,  or  from 
periurethral  fistulae,  which  have  become  lined  with  squamous  epithelium. 

Sarcoma  is  still  rarer. 

As  a  rule,  malignant  growths  arise  by  extension  from  the  genitalia 
(glans  penis,  vagina,  vulva). 


INJURIES. 

The  urethra  may  be  injured  in  various  degrees  through  instrumenta- 
tion, or  parturition.  Falls  upon  the  perineum  may  produce  laceration. 
When  rupture  of  the  urethra  takes  place  and  the  urine  cannot  escape, 
extravasation  of  the  urine  occurs  into  the  layers  of  the  perineum  and 
abdominal  wall,  leading  to  inflammation,  and,  often,  widespread  necrosis 
and  death. 


50 


SECTION   VII. 
THE  REPRODUCTIVE  SYSTEM. 


CHAPTER    XXXVII. 

THE  MALE  SEXUAL  ORGANS. 

THE  PENIS. 
CONGENITAL  ANOMALIES. 

THESE  are  very  rare.  Complete  absence  of  the  penis,  doubling  of  the 
organ,  and  partial  defects  in  the  corpora  cavernosa  may  be  mentioned. 

A  congenital  fistula  has  been  described,  in  which  a  duct  connected 
with  the  prostate  opened  upon  the  dorsum  of  the  penis  or  at  the  glans. 

Cruveilhier1  figures  a  case  in  which  the  ejaculatory  duct  took  a  course 
independently  of  the  urethra  and  opened  on  the  glans. 

Somewhat  more  common  than  absence  of  the  penis  is  total  defect  or 
abnormal  shortness  of  the  prepuce.  Hypoplasia  of  the  external  genitalia 
is  rare  in  men  otherwise  well  developed,  but  is  more  frequent  in  crypt- 
orchids,  cretins,  idiots,  and  epileptics. 

Hyperplasia  has  been  observed.  Bifurcation  of  the  penis  may  simulate 
a  double  organ.  A  curious  malformation  is  one  in  which  the  organ 
resembles  the  tongue  of  a  bell.  Atresia  and  phimosis  of  the  prepuce 
are  not  uncommon.  Plates  of  bone  have  also  been  noted  in  the  penis. 

CIRCULATORY  DISTURBANCES. 

Passive  Congestion. — Passive  congestion  is  met  with  in  those  suffer- 
ing from  valvular  heart  disease  or  other  obstruction  in  the  systemic 
circulation.  The  condition  leads  to  enlargement  of  the  corpora  caver- 
nosa. A  similar  effect  is  produced  by  the  relaxation  of  the  supporting 
structure  of  the  corpora  in  those  addicted  to  sexual  excess.  Passive 
congestion  is  sometimes  also  due  to  paraphimosis  or  other  causes  that 
lead  to  constriction  of  the  organ,  such  as  strings  tied  around  it  or  rings 
slipped  over  it.  The  last-mentioned  conditions  are  occasionally  met  with 
in  young  boys.  The  result  is  often  serious,  as  gangrene  may  set  in. 

1  Atlas,  Lfg.  39:  Taf.  2:  Fig.  3. 


788 


THE  PENIS 


Gangrene. — Gangrene  may  also  be  due  to  embolism  of  the  dorsal 
artery  or  to  thrombosis  in  the  corpora. 

Varicosity. — Varicosity  of  the  dorsal  veins  is  not  uncommon. 

Hemorrhage. — Owing  to  the  loose  structure  of  the  corpora  and  skin 
of  the  prepuce  and  penis,  effusions  of  blood  readily  take  place.  These 
may  be  due  to  rupture  of  the  corpora  or  the  bloodvessels,  and  may 
attain  considerable  size  (hematoma).  This  accident  may  occur  during 
coitus.  Dilatation  of  the  larger  lymph-channels  is  rare. 


INFLAMMATIONS. 

Inflammatory  processes  of  various  types  affect  the  penis.  The  parts 
attacked  are  the  skin,  prepuce,  glans,  and  corpora.  We  can  thus  speak 

of  a  dermatitis,  posthitis,  balani- 

FIG.  208  tis,  and  cavernitis.     As  a  rule, 

both  prepuce  and  glans  are  af- 
fected together  (balanoposthitis). 
The  preputial  sac  is  particularly 
liable  to  inflammation,  since 
there  is  a  tendency  to  accumu- 
lation of  smegma,  pus,  dirt,  and 
urinary  salts,  which  form  a 
suitable  culture  medium  for 
many  germs.  Chemical  and 
mechanical  irritation,  however, 
also  play  a  part.  If  the  swell- 
ing of  the  parts  be  great,  it 
may  be  impossible  for  the  patient 
to  draw  the  prepuce  forward 
(paraphimosis).  Passive  con- 
gestion, ulceration,  and  even 
gangrene  may  then  be  the  result. 
Catarrhal  Balanoposthitis.  — 
Simple  catarrh,  which  leads 
merely  to  slight  reddening  of 

Paraphimosis:  penis  curved  nearly  at  a  right  the  HlUCOSa,  Swelling  of  the  pre- 

angie.  (Taylor.)  puce,  secretion,  and  desquama- 

tion  of  cells  is  a  trifling  affection. 

Suppurative  Balanoposthitis. — More  important  is  suppurative  balano- 
posthitis, such  as  often  occurs  during  the  course  of  gonorrhoea  and 
phimosis.  Here  there  is  an  abundant  purulent  secretion,  great  redness 
and  swelling,  together  with  shallow  erosions  due  to  maceration  and 
desquamation  of  the  epithelium.  The  process  sometimes  leads  to 
induration  of  the  affected  parts  with  fibrosis,  and  to  union  of  the  two 
layers  of  mucous  membrane.  Both  acute  and  chronic  forms  may  be 
the  cause  of  phimosis. 

Balanoposthitis  Aspergillina. — A  mycotic  form — balanoposthitis  asper- 
gillina — has  been  met  with  in  diabetics. 


SYPHILIS  789 

Membranous  Balanoposthitis. — Membranous  inflammation  is  due  to 
the  irritation  of  retained  secretion  or  to  wound  infection,  and  occurs 
also  in  the  infective  fevers,  diphtheria,  typhoid,  variola,  scarlatina,  and 
measles. 

Herpes  Progenitalis. — Herpes  progenitalis  begins  with  the  formation 
of  one  or  more  groups  of  small  vesicles  that  rupture  and  form  superficial 
erosions,  surrounded  by  a  scanty,  whitish  border  of  epithelium.  From 
infection  larger  ulcers  may  result.  The  condition  is  possibly  neuro- 
trophic,  since  in  one  case  at  least  fibrosis  of  the  nerves  of  the  penis  was 
discovered. 

Cavernitis. — Cavernitis  is  either  acute  suppurative  or  chronic  productive 
in  character.  It  affects  the  corpora  in  whole  or  in  part.  Local  abscesses 
may  be  formed  which  perforate  into  the  urethra  or  a  diffuse  affection 
results,  with  fibrous  induration. 

Syphilis. — The  primary  lesion  of  syphilis  ("hard"  chancre)  is  by  far 
the  most  important  affection.  The  initial  sore  makes  its  appearance 
on  an  average  from  three  to  four  weeks  after  infection,  usually  upon  the 
prepuce  near  the  raphe  or  on  the  corona,  sometimes  within  the  urethra 
or  on  the  skin.  It  begins  as  a  minute  vesicle,  which  in  time  ruptures, 
leaving  a  superficial  erosion  surrounded  by  a  reddish  border.  In  a 
short  time  the  ulcer  becomes  indurated  and  hard,  so  that  when  pinched 
between  the  finger  and  thumb  it  feels  as  if  a  small  bit  of  parchment 
were  inserted  in  the  base.  The  amount  of  ulceration  and  inflammatory 
reaction  is  often  trifling  and  the  lesion  is  frequently  overlooked.  The 
sore  is  infective,  but  indolent  and  not  auto-inoculable. 

Microscopically,  the  part  is  infiltrated  for  the  most  part  with  small, 
round  cells,  but  occasional  epithelioid  elements  and  giant  cells  are  seen. 
The  round  cells  are  chiefly  aggregated  about  the  smaller  vessels,  the 
walls  of  which  are  also  thickened  and  infiltrated.  The  endothelium  of 
the  capillaries  is  proliferated.  In  the  periphery  numerous  "Mast-zellen" 
may  be  made  out.  The  connective  tissue  shows  a  progressing  fibrosis. 
The  superficial  epithelium  presents  a  loss  of  substance  that  extends 
more  or  less  deeply  into  the  underlying  strata. 

The  Spirochseta  pallida  has  now  been  repeatedly  found  in  the  syphilitic 
chancre  (Schaudinn,  Levaditi,  Buschke  and  Fischer,  Burlat  and  Vin- 
cent), usually  in  the  lymphatic  spaces  and  between  the  epithelial 
cells. 

Sooner  or  later  the  infection  becomes  systemic,  an  early  sign  of  which 
is  hyperplasia  of  the  inguinal  lymphatic  glands,  forming  what  is  known 
as  the  "indolent  bubo." 

In  the  second  stage  of  syphilis  small,  reddish,  moist  nodules  form  on 
the  mucosa  which  may  fuse  together,  and  from  the  effect  of  warmth  and 
moisture  form  the  broad  condyloma.  As  in  the  case  of  the  primary 
chancre,  these  may  ulcerate. 

In  tertiary  syphilis,  small  gummas  (syphilomata)  may  be  formed  in 
any  part  of  the  penis.  They  are  usually  deeply  situated  and  may  cause 
extensive  ulceration.  When  they  heal,  dense  and  deforming  scars  may 
result. 


790  THE  PENIS 

Chancroid. — As  contradistinguished  from  the  primary  syphilitic  sore 
or  "hard"  chancre  we  have  to  recognize  a  non-specific  sore  or  "soft" 
chancre  (chancroid). 

This  develops  in  from  one  to  five  days  after  exposure,  in  the  form  of 
a  small  vesicle  or  pustule  which  rapidly  breaks  down  into  an  ulcer, 
having  a  sharply  defined  or  undermined  edge  and  an  angry  reddish- 
yellow  base  secreting  pus.  A  necrotic  membrane  is  often  formed  on  the 
surface. 

The  ulcer  is  usually  found  on  the  inner  surface  of  the  prepuce, 
especially  about  the  frenum,  and  on  the  glans.  It  differs  from  the  true 
syphilitic  sore  in  the  shorter  incubation  period,  the  more  rapid  and 
severe  erosion,  to  a  certain  extent  in  its  position,  and  on  the  fact  that 
it  is  auto-inoculable,  so  that  multiple  ulcers  are  frequently  observed. 
Secondary  syphilides  do  not,  of  course,  develop.  A  certain  amount  of 
inflammatory  induration  may  be  produced,  but  this  is  rarely  to  be  con- 
fused with  the  parchment-like  feel  of  the  syphilitic  sore,  which  may 
persist  long  after  the  ulcer  has  disappeared. 

Microscopically,  the  vessels  are  dilated,  the  papillae  in  the  neighbor- 
hood are  proliferating,  and  the  base  and  edge  of  the  sore  are  markedly 
infiltrated  with  inflammatory  products.  The  exact  cause  of  the  soft 
chancre  is  not  certainly  known,  whether  it  is  due  to  the  ordinary  pus 
germs  or  to  a  specific  microorganism  acting  with  the  pus  germs.  It 
should  not  be  forgotten  that  a  -mixed  infection  with  syphilis  and  soft 
chancre  occasionally  occurs.  Here  what  appears  to  be  an  ordinary  soft 
chancre,  eventually  becomes  indurated  and  is  followed  by  the  ordinary 
secondary  manifestations  of  syphilis.  Soft  chancre  often  leads  to  swell- 
ing of  the  prepuce,  lymphangitis  in  the  penis,  and  to  suppurative  inflam- 
mation of  the  inguinal  glands  (virulent  bubo)  and  the  neighboring  tissues. 
In  persons  of  low  vitality  it  may  become-phagedenic. 

Tuberculosis. — Tuberculosis  is  rare  in  adults,  although  caseous 
ulceration  of  the  glans  is  recorded.  Microscopically,  it  does  not  differ 
from  tuberculosis  elsewhere. 

Tuberculous  infiltration  of  the  prepuce  is  commoner  and  is  met  with 
in  children  as  a  result  of  the  ritual  practice  of  circumcision,  in  cases 
where  the  saliva  of  the  operator  contained  tubercle  bacilli. 

Foreign  Bodies  and  Parasites. — Retained  smegma  and  dirt  may 
become  inspissated  and  infiltrated  with  lime  salts,  so  that  a  concre- 
ment  is  formed.  Phimosis  (tight  prepuce)  is  a  strong  predisposing 
cause  of  this  condition.  Urinary  calculi  may  also  be  arrested  by  a 
tight  foreskin  and  form  a  nidus  for  further  accretion  (preputial  calculus). 
Of  parasites  may  be  mentioned  bacteria,  yeasts,  spores  of  fungi,  and 
mycelial  threads.  The  most  important  form  is  the  smegma  bacillus, 
inasmuch  as,  morphologically  and  tinctorially,  it  resembles  closely  the 
tubercle  bacillus.  As  it  is  contained  in  most  urines,  it  will  be  seen  how 
important  it  is  to  differentiate  between  the  two  microorganisms  (vide 
p.  757). 


CONDYLOMA  791 


RETROGRADE  METAMORPHOSES. 

Senile  atrophy  of  the  corpora  is  common.  As  the  prepuce  is  less 
affected,  it  appears  to  be  relatively  long.  Necrosis  and  ulceration  may 
lead  to  deformity  of  the  organ.  In  some  cases,  supposed  to  be  due  to 
a  lack  of  resisting  power  on  the  part  of  the  individual,  ulceration  may  be 
rapid  and  destructive  (phagedena).  This  is  apt  to  occur  in  alcoholics, 
syphilitics,  diabetics,  and  in  tuberculous  persons. 


PROGRESSIVE  METAMORPHOSES. 

Condyloma  Acuminata. — Papillomatous  outgrowths,  due,  for  the 
most  part,  to  inflammation  or  chronic  irritation,  are  not  uncommon  on 
the  glans  and  prepuce.  The  most  frequent  form  is  the  condyloma 
acuminata,  which  develops  characteristically  on  an  inflammatory  basis, 
and  is  generally  due  to  an  irritating  discharge  (gonorrhoea)  or  retained 
secretion.  Phimosis  is  a  potent  predisposing  cause.  Condylomas  take 
the  form  of  larger  or  smaller  multiple  papillary  excrescences  on  the 
glans  and  prepuce  not  unlike  a  cock's  comb.  From  the  pressure  of  a 
contracted  prepuce  the  growths  are  often  somewhat  flattened.  In 
aggravated  cases,  large  masses,  the  size  of  a  fist,  having  a  cauliflower 
appearance,  may  be  produced.' 

Microscopically,  the  outgrowths  consist  of  a  fibrous  vascular  core 
covered  with  stratified  squamous  epithelium.  The  fibrous  tissue  is 
richly  branched,  so  that  papillomas  are  produced,  although  this  arrange- 
ment is  somewhat  masked  by  the  proliferation  of  the  epithelial  covering. 
Inflammatory  infiltration  is  generally  also  to  be  observed.  The  diagnosis 
between  condyloma  and  carcinoma  is  not  always  easy.  Condylomas 
are  usually  soft  and  freely  movable  upon  the  subjacent  tissues,  unless 
ulceration  has  taken  place.  In  this  case,  inflammatory  induration  is 
apt  to  impair  the  mobility.  Carcinoma  is  usually  hard  and  infiltrated. 

Keratosis. — Another  form  of  hypertrophy  is  seen  in  the  heaping  up 
of  the  superficial  epithelium  known  as  keratosis. 

Tumors. — Carcinoma. — Carcinoma  of  the  penis,  usually  epithelioma, 
forms,  according  to  Orth,  2.8  per  cent,  of  all  cancers,  and  is  met  with 
generally  between  the  ages  of  fifty  and  seventy.  Phimosis,  as  it  con- 
duces to  the  retention  of  decomposing  secretion,  appears  to  be  an 
important  predisposing  cause.  Epithelioma  may  also  arise  in  the 
condylomas  just  described  and  in  keratosis.  The  growth  may  begin 
in  any  part,  but  usually  at  the  edge  of  the  prepuce,  the  sulcus,  and  the 
inner  surface  of  the  prepuce.  It  begins  ordinarily  as  a  small  wart  that 
gradually  extends  over  the  surface  of  the  organ,  forming  a  papillomatous 
mass  that  may  erode  through  the  prepuce.  The  surface  is  moist,  the 
folds  contain  a  foul,  whitish,  greasy  secretion.  Ulceration  occurs  in 
advanced  cases. 

Microscopically,  processes  of  squamous  epithelium  are  seen  to  extend 


792  THE  PENIS 

deeply  into  the  underlying  structures  from  the  superficial  strata,  often 
forming  branching  masses.  "Cell-nests"  of  keratohyaline  material  are 
often  present.  When  ulceration  takes  place  the  growth  becomes  very 
granular,  owing  to  infiltration  with  inflammatory  products.  Extension 
takes  place  through  the  lymphatic  system,  and  the  inguinal  glands  are 
first  and  most  strikingly  involved.  Much  rarer  is  medullary  carcinoma 
of  the  adenomatous  type.  Still  rarer  are  melanotic  forms. 

Melanotic  sarcoma  occurs  and  is  liable  to  be  confounded  with  carcinoma. 
Unpigmented  sarcoma  has  been  found  originating  in  the  corpora,  and  also 
intravascular  endothelioma.  Secondary  growths,  carcinoma,  and  sarcoma, 
usually  are  metastatic  or  arise  by  extension  from  neighboring  parts. 

Fibroma,  lipoma,  and  neuroma  have  also  been  described.  Cysts  due 
to  obstruction  of  the  sebaceous  glands  are  fairly  common. 

Elephantiasis. — Somewhat  allied  to  tumor-formation  is  elephantiasis, 
which  chiefly  affects  the  prepuce,  either  alone  or  together  with  the  whole 
penis,  and  sometimes  the  scrotum.  The  extent  of  the  disease  may  be 
trifling  or  a  large  tumor  may  be  produced.  One  is  described  that  weighed 
more  than  25  kilos.  The  growth  is  easily  distinguished  from  elephantiasis 
of  the  scrotum  in  that  the  opening  of  the  urethra  is  at  the  base  of  the 
tumor. 

Microscopically,  the  mass  consists  chiefly  of  fibrous  tissue,  containing 
in  the  superficial  layers  many  "Mast-zellen."  In  the  preputial  portion 
bundles  of  unstriped  muscle  have  been  noted.  There  may  be  diapedesis 
of  leukocytes  about  the  vessels.  The  superficial  papillae  of  the  skin  are 
often  unaltered,  but  may  show  signs  of  overgrowth,  and  even  may  form 
papillomatous  warts. 

INJURIES. 

The  most  striking  injury  is  the  so-called  luxation  of  the  penis,  in  which 
the  main  substance  of  the  organ  is  separated  from  the  prepuce  and 
overlying  skin  and  is  found  beneath  the  skin  of  the  trunk,  the  original 
covering  hanging  like  an  empty  sausage  skin.  Fracture  of  the  penis, 
usually  of  the  corpora  cavernosa,  occurs  from  striking  the  organ  when 
in  an  erect  condition  against  some  hard  substance.  It  is  sometimes 
caused  by  intentional  violence,  as  in  the  dangerous  practice  among 
the  lower  orders  of  "breaking  the  cord"  in  chordee.  Injuries  also  occur 
during  attempts  at  coitus,  from  falls,  tying  strings  around  the  organ,  or 
inserting  foreign  substances  (catheters,  etc.). 


THE  PROSTATE. 


MALFORMATIONS. 

These  are  not  common.     Complete  absence  of  the  prostate  is  met  with 
in  association  with  other  grave  defects  of  the  genito-urinary  apparatus. 


TUBERCULOSIS  793 

Unilateral  aplasia  and  hypoplasia  is  exceptionally  observed  in  unilateral 
hypoplasia  of  the  sexual  organs.  Aberrant  prostates  have  been  found. 
Unilateral  or  complete  defect  of  the  colliculus  seminalis,  and  dilatation 
of  the  prostatic  sinus  have  been  described.  Cysts  are  sometimes  found 
along  the  course  of  the  Miillerian  duct,  due  to  imperfect  closure  of  the 
same. 

CIRCULATORY  DISTURBANCES. 

Hyperemia  is  a  common  but  comparatively  unimportant  condition. 
Repeated  or  continued  congestion  has  been  by  some  regarded  as  a  cause 
of  hypertrophy  of  the  organ.  The  prostatic  plexus  is  often  found  dilated, 
and  thrombosis  and  phlebolith  formation  are  comparatively  common. 


INFLAMMATIONS. 

Prostatitis. — Catarrhal  Prostatitis. — Simple  catarrhal  prostatitis  is 
described,  in  which  there  is  an  accumulation  of  inflammatory  products 
and  desquamated  cells  within  the  tubules,  together  with  hyperemia  and 
oedema  of  the  supporting  structure.  Little  is  known,  however,  about 
this  affection. 

Suppurative  Prostatitis. — More  important  is  suppurative  prostatitis, 
in  which  the  glands  are  filled  with  pus  and  abscesses  are  produced. 
This  is  not  an  infrequent  sequel  of  gonorrhoea,  but  is  also  a  result  of 
severe  cystitis  and  injuries  to  the  urethra.  The  process  in  the  first 
instance  begins  in  the  gland-tubes  but  soon  spreads  to  the  neighboring 
tissues  where  several  foci  may  coalesce  to  form  abscesses.  The  abscesses 
are  multiple  and  are  scattered  irregularly  through  the  gland,  which  may 
be  almost  entirely  destroyed.  They  may  perforate  into  the  bladder, 
urethra,  scrotum,  perineum,  rectum,  or  through  the  abdominal  wall.  A 
paraprostatitis  may  lead  to  general  peritonitis.  Should  the  abscesses 
heal,  fibroid  scars  are  the  result  or  perhaps  calcification.  Suppurative 
prostatitis  is,  rarely,  metastatic  in  septicemia. 

Chronic  Prostatitis. — In  chronic  prostatitis,  prostatorrhcea  may  be  a 
chief  symptom,  and  Rokitansky  has  described  a  condition  in  which  the 
secretion  was  quite  milky  from  the  presence  of  lecithin.  The  condi- 
tion may  be  simple  or  suppurative.  The  organ  is  swollen  and  of  a  dirty 
brownish  color.  The  gland-tubules  are  dilated  and  may  coalesce, 
forming  cysts. 

Tuberculosis. — Tuberculosis  takes  the  form  of  multiple  caseous 
nodules  in  one  or,  more  frequently,  in  both  lobes,  which  may  lead  to 
considerable  enlargement  of  the  gland.  In  advanced  cases  the  whole 
prostate  may  be  destroyed.  The  caseous  foci  sometimes  soften,  leading 
to  the  formation  of  abscesses  which  may  burst  into  the  urinary  passages 
or  into  the  rectum.  As  with  suppurative  inflammation,  the  process 
begins  in  the  gland-tubules,  both  in  tuberculosis  of  urogenital  and  hema- 
togenic  origin.  As  a  rule,  tuberculosis  is  part  of  an  extensive  urogenital 
infection.  Primary  tuberculosis  of  the  organ  is  decidedly  rare. 


794 


THE  PROSTATE 


Foreign  Bodies. — Concretions,  corpora  amylacea,  are  found  commonly 
in  the  prostate,  especially  of  old  people.     On  cutting  into  the  gland 


FIG.  209 


Caseous  tuberculosis  of  the  prostate.     Leitz  obj.  No.  3,  without  ocular.     The  necrotic  area  is  shown 
to  the  right,  with  giant-cells  toward  the  margin.     (From  the  collection  of  Dr.  A.  G.  Nicholls.) 

FIG.  210 


a  <a&\      \»  >. 


Corpora  amylacea  in  the  prostate.    Leitz  obj.  No.  3.     (From  the  collection  of  Dr.  A.  G.  Nicholls.) 


HYPERTROPHY  795 

they  appear  like  grains  of  black  pepper,  but  may  be  large  and  gritty, 
resembling  grape-seeds.  The  latter  peculiarity  is  due  to  infiltration  with 
salts.  The  corpora  are  found  chiefly  in  the  neighborhood  of  the  colliculus 
seminalis,  but  also  throughout  the  gland. 

Microscopically,  the  smaller  ones  are  oval  or  rounded,  more  rarely 
triangular,  showing  concentric  lamination,  giving  them  a  general  Resemb- 
lance to  starch  granules,  whence  their  name.  In  many  cases  two  or 
three  cells  in  process  of  fusion  and  disintegration  may  often  be  seen 
with  beginning  hyaline  transformation,  showing  that  the  process  has  its 
origin  in  catarrh  and  degeneration  of  the  lining  epithelium.  The  larger 
granules  show  but  little  lamination,  and  are  merely  amorphous  masses 
of  brownish  mineral  matter.  Posner  has  suggested  that  two  bodies  are 
present  in  the  corpora,  a  hyaline,  albuminous  substance  and  lecithin. 
The  condition  is  of  little  pathological  significance. 

Parasites. — Echmococcus  cysts  have  been  found  in  the  prostate. 


RETROGRADE  METAMORPHOSES. 

Simple  atrophy  of  the  gland  occurs  in  from  20  to  30  per  cent,  of  old 
men.  It  is,  however,  occasionally  met  with  in  young  people,  as  a  result 
of  wasting  disease,  cachexia,  castration,  the  impotence  of  the  tubercu- 
lous, the  pressure  of  retained  urine,  pent-up  secretion,  and  concretions. 
In  the  form  due  to  constitutional  causes,  the  glandular  portion  is  the 
one  chiefly  affected,  while  in  that  due  to  concrements  the  stroma 
suffers  most.  In  the  latter  case,  however,  the  epithelial  cells  may  be 
flattened  and  fattily  degenerated  from  pressure. 

Pigmentation  of  the  epithelial  cells  is  found  in  advanced  age  and  in 
the  cachexias. 

Hyaline  degeneration  of  the  muscle  bundles  and  the  glandular  epithe- 
lium is  met  with.  Fatty  degeneration  is  also  met  with.  The  so-called 
amyloid  bodies,  above-mentioned,  have  nothing  to  do  with  amyloid 
disease. 

PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Hypertrophy  of  the  prostate  takes  two  forms,  the 
most  common  being  perhaps  a  condition  of  multiple  fibromyoma,  analo- 
gous to  the  fibroids  of  the  uterus,  and  a  more  diffuse  glandular  or  adeno- 
matous  overgrowth.  In  both  varieties  the  gland  is  enlarged,  either  as 
a  whole  or  in  part,  and  may  attain  the  size  of  the  fist.  The  outer  surface 
is  usually  smooth,  but  may  be  rough  and  warty.  On  section,  in  the 
fibromyomatous  form,  the  gland  is  studded  with  nodules  varying  in  size 
from  that  of  a  pin-head  to  a  large  bean,  which  are  firm,  projecting,  and 
more  or  less  fasciculated.  These  nodules  are  of  a  grayish  or  grayish- 
white  color.  In  adenomatous  enlargement  the  tissue  is  softer  and  some- 
what spongy,  of  a  yellowish-red  color.  There  may  be  cystic  condition 
of  the  glands. 


796 


THE  PROSTATE 


The  enlargement  may  affect  one  or  both  sides,  but  of  most  importance 
is  enlargement  of  the  so-called  "  middle"  lobe,  which  may  form  a  broad 
sessile  mass  or,  again,  a  pedunculated  growth  projecting  into  the  urethra 
and  even  the  bladder.  This  condition  is  of  great  practical  moment  in 
that  it  leads  to  obstruction  to  the  free  discharge  of  urine  with  all  its 
effects  on  the  bladder,  ureters,  and  kidneys.  Exceptionally,  obstruction 
does  not  occur  in  the  prostatic  urethra,  which,  on  the  contrary,  is  rather 
dilated,  presumably  from  the  effects  of  the  pressure  of  the  retained 
urine.  In  cases  of  long  standing,  cystitis  is  usually  present  with  hyper- 
trophy and  dilatation  of  the  bladder.  Hydronephrosis  and  septic  pyelo- 
nephritis are  not  infrequent  complications.  Where  catheterization  has 
been  practised,  false  passages  and  abscesses  may  be  formed  in  the 
prostate,  or,  again,  cicatrices.  The  ejaculatory  duct  is  frequently  found 
obstructed. 


FIG.  211 


Myomatous  enlargement  of  the  prostate,  obstruction  to  the  outflow  of  urine  caused  by  the 
overgrowth  of  the  so-called  middle  lobe;  consecutive  hypertrophy  of  the  walls  of  the  bladder. 
(From  the  Pathological  Museum  of  McGill  University.) 

Hypertrophy  of  the  prostate  is  rather  common  in  old  men.  The  reason 
for  this  is  not  quite  clear.  As  an  expression  of  a  senile  change  one  would 
expect  atrophy  rather  than  hypertrophy.  We  know,  however,  that  the 
process  begins  in  the  neighborhood  of  the  glandular  elements,  and 
consequently  some  observers  have  insisted  upon  the  action  of  preexisting 
irritation,  such  as  passive  congestion  and  chronic  inflammation,  as  the 


CARCINOMA  797 

exciting  causes.  Gonorrhoea  is  mentioned  in  this  connection.  Prob- 
ably the  myomatous  form,  if  not  the  adenomatous  enlargement,  is 
a  true  tumor,  and  as  such  depends  upon  the  same  factors  that  are  at 
work  in  the  production  of  tumors  elsewhere,  whatever  they  may  be. 

Microscopically,  in  the  first  variety,  the  nodules  are  composed  largely 
of  muscular  tissue  or  mixed  fibrous  tissue  and  muscle  arranged  in  inter- 
lacing bundles,  exactly  as  in  uterine  fibroids.  In  the  adenomatous 
type  the  glands  are  apparently  increased  in  number,  dilated  and  saccu- 
lated,  while  the  fibromuscular  stroma  is  correspondingly  diminished. 
The  secreting  cells  are  often  flattened  or  desquamated,  and  may  undergo 
various  forms  of  disintegration  and  hyaline  change. 

FIG.  212 


So-called  "adenoma"  of  the  prostate  gland.  An  amyloid  body  can  be  seen  at  the  upper 
right-hand  edge  of  the  section.  Winckel  obj.  No.  3,  without  ocular.  (From  the  collection  of 
Dr.  A.  G  Nicholls.) 

Tumors. — Of  malignant  growths  carcinoma  and  sarcoma  should  be 
mentioned. 

Carcinoma. — Primary  carcinoma  is  not  uncommon,  and  originates, 
in  many  cases  at  least,  in  a  previously  existing  adenomatous  enlarge- 
ment. One  case  has  come  under  our  observation  in  which  there  were 
symptoms  of  urinary  obstruction  due  to  enlarged  prostate  for  eleven 
years  before  the  development  of  adenocarcinoma.  Carcinoma  of  the 
prostate  takes  the  form  of  a  rather  soft,  nodular  tumor  in  one  or  both 
lobes  of  the  prostate,  which  rapidly  infiltrates  the  capsule  of  the  gland, 
the  mucosa  at  the  neck  of  the  bladder,  and  the  prostatic  urethra.  It 
may,  indeed,  extend  to  the  penis  and  rectum.  Occasionally  it  assumes 
a  papillomatous  appearance. 


798  THE  PROSTATE 

Microscopically,  the  growth  is  composed  of  cylindrical  or  polyhedral 
cells,  arranged  more  or  less  in  glandular  fashion  or  in  solid  bands  or 
masses.  The  stroma  is  normal  or  infiltrated  with  round  cells.  Cases 
occur  in  which  the  growth  resembles  sarcoma  closely  until  more  careful 
study  shows  it  to  be  carcinomatous  (Adami).  Metastases  are  insignifi- 
cant, as  a  rule,  and  occur  late.  The  retroperitoneal  lymph-glands  and, 
exceptionally,  the  inguinal  glands  and  peritoneum  are  the  parts  attacked. 
Carcinoma  is  found  at  all  ages,  but  naturally  much  more  commonly  in 
old  people. 

FIG.  213 


Carcinoma  of  the  prostate.     Zeiss  obj.  DD,  ocular  No.  1.      (From  the  Pathological  Laboratory 

of  McGill  University.) 

Sarcoma. — Sarcomas  are  usually  round-celled  or  spindle-celled.  About 
10  per  cent,  of  all  malignant  tumors  of  the  prostate,  according  to  Orth, 
occur  in  childhood.  The  large  majority  of  these  are  sarcomas. 

Secondary  tumors  of  metastatic  origin  are  rare.  More  frequent  are 
those  due  to  extension  from  the  bladder  or  rectum. 


THE  TESTES  AND  EPIDIDYMES. 


CONGENITAL  ANOMALIES. 

These  consist  in  defects  of  development  and  irregularities  in  the 
process  of  descent.  An  increase  in  the  number  of  the  testes — poly- 
orchidism — is  occasionally  met  with.  Usually  two  on  one  side  are  found. 


ORCHITIS  AND  EPIDIDYMITIS  799 

One  case  is  on  record1  where  both  testes  were  fused  into  one — synorchid- 
ism;  complete  absence  of  the  testes — anorchidism — is  more  usual.  The 
absence  may  be  unilateral.  In  such  cases,  as  a  rule,  there  is  defect  of 
the  epididymis  and  vas  deferens  on  the  same  side.  Incomplete  develop- 
ment— hypoplasia — is  not  uncommon.  The  condition  is  bilateral  or 
unilateral,  and  is  due  usually  to  retardation  in  the  descent  of  the  organ. 

Microscopically,  a  retained  testicle  shows  a  relative  increase  in  the 
amount  of  the  stroma,  while  the  gland-tubes  are  atrophic  or  badly  de- 
veloped. Hypoplasia  is  exceptionally  met  with  in  children  and  at  the 
age  of  puberty.  In  such  cases  the  testes  may  have  descended  properly 
but  have  lagged  behind  in  the  general  growth  of  the  sexual  organs. 

Abnormal  position  of  the  testis  (ectopia)  is  not  uncommon.  The 
testis  is  either  retained  at  some  point  in  the  canal  or  is  actually  dislocated 
out  of  its  natural  passage.  The  testis  may  be  retained  in  the  abdomen 
opposite  the  lumbar  vertebrae,  in  the  inguinal  canal,  or  at  the  fold  be- 
tween the  scrotum  and  thigh.  In  dislocation,  dystopia,  the  testis  is 
outside  its  normal  surroundings,  and  is  found  in  the  abdominal  wall 
in  a  pouch  leading  from  the  inguinal  canal,  at  the  femoroscrotal  fold. 
The  testis  may  also,  as  in  a  case  once  observed  by  us,  be  found  in  an 
artificial  sac  on  the  inner  side  of  the  thigh  (hernia  cruralis  testicularis). 
A  great  rarity  is  the  presence  of  both  testes  in  the  same  half  of  the 
scrotum.  Dystopia  is  usually  unilateral,  but  may  be  bilateral.  A 
dislocated  testis  is  particularly  liable  to  disease,  and  generally  shows 
atrophy,  fatty  degeneration  of  the  secreting  cells,  and  fibroid  induration. 
It  may  also  be  infarcted  and  inflamed.  Dystopia  is  not  always  congenital 
but  may  be  due  to  trauma,  as  sometimes  happens  in  gymnasts. 

An  interesting  anomaly  is  the  presence  of  aberrant  suprarenal  tissue 
between  the  testis  and  the  head  of  the  epididymis. 


CIRCULATORY  DISTURBANCES. 

Anemia  of  the  testis  is  usually  due  to  pressure,  as  in  hydrocele  and  hema- 
tocele.  Anemic  and  hemorrhagic  infarcts  are  also  occasionally  met 
with.  Necrosis  is  apt  to  supervene  in  such  cases  and  when  phlebitis  or 
thrombosis  of  the  pampiniform  plexus  is  present.  Hemorrhage  into  the 
testis  is  generally  a  result  of  traumatism,  but  is  also  met  with  in  leukemia. 


INFLAMMATIONS. 

Orchitis  and  Epididymitis. — Inflammation  of  the  testis — orchitis— 
and  of  the  epididymis — epididymitis — may  occur  independently  of  each 
other,  but  are  usually  associated.  The  tunica  vaginalis,  vas  deferens, 
and  the  spermatic  cord  are  frequently  involved  as  well. 

As  a  rule,  the  condition  is  brought  about  by  infection  through  the 

1  Cruveilhier,  Traite  d'anat.  path,  i:  301. 


800  THE  TESTES  AND  EPIDIDYMES 

spermatic  ducts  (gonorrhceal)  or  from  contiguity  with  the  tunica  and 
spermatic  vessels.  Trauma  is  the  next  most  important  cause.  Hemato- 
genic  infection  is  not  so  common.  In  cases  where  the  infection  has 
spread  from  the  spermatic  ducts,  the  epididymis  is  usually  first  involved. 
The  character  of  the  inflammation  varies  somewhat  in  the  case  of  the 
two  organs.  In  the  testicle  the  inflammation  is  at  first  interstitial,  while 
in  the  epididymis  it  is  more  likely  to  be  catarrhal  and  desquamative. 
Orchitis  is  often  met  with  in  gonorrhoea,  less  frequently  in  typhoid, 
mumps,  and  variola.  It  has  also  been  observed  after  abdominal 
operations.  The  gonorrhoeal  form  begins  usually  as  a  catarrhal  and 
interstitial  epididymitis  which  frequently  extends  to  the  testis.  The 
affection,  as  a  rule,  arises  from  the  second  to  the  sixth  week  of  the 
urethritis. 

Macroscopically,  the  epididymis  is  enlarged  and  its  consistency 
increased.  On  section  it  is  reddened,  the  tubules  are  frequently  dilated 
and  filled  with  grayish-yellow  material,  composed  of  pus,  mucus,  and 
desquamated  epithelium.  The  testis  may  show  merely  reddening  and 
oedema,  but  may  be  actually  inflamed.  Here  the  inflammation  tends 
to  be  interstitial,  and  abscess-formation  is  more  common  than  in  the 
epididymis. 

Microscopically,  in  the  epididymis,  the  vessels  are  congested,  and 
the  epithelial  lining  of  the  tubules  is,  in  part,  converted  into  goblet-cells 
secreting  mucus.  The  connective  tissue,  the  walls  of  the  tubules,  and 
the  epithelium  are  all  infiltrated  with  inflammatory  products.  The 
looser  portions  are  cedematous.  If  the  process  last  for  a  sufficiently 
long  time,  fibroid  induration  sets  in,  which  may  lead  to  obstruction  and 
cystic  dilatation  of  the  tubules. 

In  suppurative  orchitis  the  organ  is  swollen  and  its  capsule  tense. 
On  section,  it  is  of  a  yellow  color,  pulpy,  and  cedematous,  the  tubules 
showing  as  yellowish- white  streaks.  Large  or  smaller  abscesses  are 
often  found.  Microscopically,  the  tubules  are  distended  with  pus  cells 
and  the  interstitial  stroma  is  either  diffusely  infiltrated  or  presents  foci 
of  suppuration.  In  chronic  cases  fibrosis  may  result. 

Chronic  Fibroid  Orchitis. — Chronic  fibroid  orchitis  may,  as  has  just 
been  remarked,  terminate  a  simple  or  suppurative  inflammation,  but  is 
most  strikingly  found  in  syphilis. 

Tuberculosis. — Tuberculous  orchitis  and  epididymitis  are  fairly 
frequent.  The  affection  is  rarely  primary,  but  is  usually  associated 
with  tuberculosis  of  the  seminal  vesicles,  vasa  deferentia,  prostate, 
bladder,  and  ureters.  In  adults  the  process  generally  begins  in  the 
epididymis  and  involves  the  testis  secondarily.  In  children  before  the 
age  of  puberty  the  reverse  is  the  case.  The  infection  is  either  hemato- 
genic  or  from  extension  along  the  vas  deferens.  Infection  from  the 
urethra  is  rare.  Nakarai1  has  shown  recently  that  tubercle  bacilli  may 
be  found  in  the  normal  testicle  and  epididymis  of  a  tuberculous  person. 

It  is  the  rule  for  one  or  more  large,  caseous  foci  to  be  formed  with 

1  Ziegler's  Beitrage,  24: 1898:327. 


SYPHILIS  801 

subsidiary  smaller  tubercles,  but  occasionally  there  are  numerous  foci 
of  about  equal  size.  When  the  epididymis  is  involved  it  is  converted 
into  a  caseous  mass,  often  bounded  by  fibrous  tissue.  Here  the  process 
begins  in  the  walls  of  the  tubules  in  the  globus  major.  Next  to  the  epi- 
didymis, the  corpus  Highmori  is  the  most  seriously  affected,  being 
either  filled  with  small,  caseous  nodules  or  totally  destroyed.  In  time 
the  infection  spreads  to  the  testicle,  which  contains  a  few  small,  gray 
or  yellowish-white  tubercles.  These  start  in  the  tubules  or,  occasion- 
ally, in  the  interstitial  tissue.  Inflammatory  infiltration  takes  place 
with  catarrh  and  proliferation  of  the  tubular  epithelium,  and  the  process 

FIG.  214 


Tuberculosis  of  the  epididymis.     Zeiss  obj.  DD,  ocular  No.  1.     (From  the  Pathological 
Laboratory  of  McGill  University.) 

gradually  spreads  along  the  lymphatics  and  tubules.  Caseation  rapidly 
supervenes  and  giant  cells  are  numerous.  Later,  the  testis  is  filled  with 
large  caseous  or  caseofibroid  masses  tending  to  coalesce.  The  disease 
may  gradually  extend  to  the  tunica  propria  and  tunica  communis,  and 
finally  to  the  skin.  When  softening  occurs  fistulse  may  be  formed. 
Fungoid  granulations  may  appear  externally.  Fibrosis  is  never  suffi- 
cient to  overbalance  the  destructive  process. 

Syphilis. — Syphilis  is  not  uncommonly  found  in  the  testicle  in  the 
later  stages  of  the  disease.  As  a  rule,  the  process  begins  in  the  testis 
and  spreads  thence  to  the  epididymis.  Two  forms  are  met  with,  a 
diffuse  fibroid  induration  and  gumma. 

In  the  first  type  the  testis  on  section  presents  delicate,  pearly  white 
bands  of  connective  tissue,  not  infrequently  extending  from  the  rete  to 
the  tunica  albuginea.  These  are  pathognomonic  of  syphilis.  Micro- 
51 


802 


THE  TESTES 


scopically,  the  process  is  seen  to  be  primarily  an  interstitial  one,  the 
intertubular  stroma  containing  a  few  leukocytes  and  being  greatly 
thickened,  while  the  tubules  are  atrophic  and  hyaline.  The  arteries  are 
also  thickened.  Federmann1  has  pointed  out  as  a  further  diagnostic 
point  that  in  syphilis  the  elastic  tissue  of  the  testis  is  preserved,  while 
in  tuberculosis  it  disappears  even  before  caseation  has  set  in. 


FIG.  215 


Healed  syphilis  of  the  testis.  Leitz  obj.  No.  3,  without  ocular.  There  is  a  marked  over- 
growth of  connective  tissue.  Many  of  the  tubules  have  disappeared  entirely.  Some  are  repre- 
sented merely  by  rings  of  nuclei.  Those  still  existing  are  compressed  and  atrophic.  (From  the 
collection  of  Dr.  A.  G.  Nicholls.) 

In  the  gummatous  form  the  testicle  contains  firm  nodules  enclosed  in 
fibrous  tissue.  As  a  rule,  only  one  organ  is  affected.  In  many  cases 
the  tunica  albuginea  becomes  involved  so  that  a  serous  or  serofibrinous 
exudation  is  produced,  which  may  lead  to  thickening  and  adhesion  of 
the  membranes.  Occasionally,  the  skin  is  involved  and  gummy  foci 
may  discharge  externally.  Fungoid  excrescences  about  the  sinuses 
occur,  as  in  tuberculosis,  but  are  rare. 

Lepra. — Leprosy  occurs  in  the  testis  and  epididymis  in  the  form  of 
granulomas,  leading  to  necrosis.  Should  healing  take  place  the  organ 
remains  permanently  atrophic. 

Glanders. — In  the  human  subject  glanders  rarely  affects  the  testicle. 
In  guinea-pigs,  however,  that  have  been  injected  intraperitoneally  with 
the  B.  mallei,  the  testes  and  scrotum  become  acutely  and  markedly 
infiltrated  with  inflammatory  products  (Strauss'  phenomenon).  This 
fact  is  taken  advantage  of  in  the  laboratory  diagnosis  of  the  disease. 

1  Inaug.  Diss.,  Gottingen,  1900. 


CYSTS  803 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — The  condition  of  atrophy,  so-called,  is  found  as  a  senile 
change  and  in  wasting  diseases.  Pressure  may  bring  it  about,  as  in  hydro- 
cele,  hematocele,  varicocele,  hernias,  and  tumors.  A  similar  condition 
is  met  with  in  injuries  to  the  cerebellum,  concussion  of  the  brain,  and 
paraplegia. 

The  most  common  retrograde  change  is  a  diminution  in  the  size  of 
the  testis,  in  which  there  is  a  more  or  less  complete  destruction  of  the 
secreting  cells.  It  is  not  to  be  regarded  entirely  as  an  atrophic  process, 
however,  inasmuch  as  there  is  in  many  cases,  although  not  in  all,  a 
progressive  fibrosis  of  the  stroma  with  thickening  of  the  walls  of  the 
seminiferous  tubules.  Not  infrequently,  the  walls  appear  swollen,  trans- 
parent, and  hyaline,  so  that  folds  project  into  the  lumen  of  the  tubules, 
which  in  advanced  cases  may  be  obliterated.  The  secreting  cells  show 
fatty  degeneration,  and,  with  the  cells  of  the  interstitial  substance,  are 
pigmented. 

Calcification  occurs  in  tumors,  abscesses,  and  fibrous  scars. 

Gangrene  of  the  testis  occurs  after  trauma. 


PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Compensatory  hypertrophy  of  one  testis,  when  the 
other  is  deficient,  probably  exists,  although,  contrary  to  what  one  would 
expect,  it  is  very  rare.  A  form  of  enlargement  due  to  overgrowth  of 
the  connective  tissue  has  been  met  with  in  the  Tropics,  associated  with 
elephantiasis  of  the  scrotum. 

Tumors. — The  tumors  occurring  in  the  testis  are  striking  for  their 
great  variety  and  for  certain  special  forms  of  a  heterotopic  nature. 

Cysts. — Simple  retention  cysts  are  not  infrequent.  They  develop 
about  puberty  and  are  attributable  to  defects  in  development.  The 
seminiferous  glands  and  their  discharging  ducts  are  developed  inde- 
pendently; consequently,  should  they  not  unite  in  the  normal  manner, 
the  glands  form  a  closed  cavity  which  will,  in  time,  become  enormously 
distended  with  fluid.  A  similar  condition  may  be  brought  about  by 
obstruction  of  the  ducts  from  fibrous  induration  or  tumors.  The  cysts 
of  the  epididymis  are  due  in  part  to  secretion  with  retention,  spermato- 
cele,  and  to  aberrant  growth  of  certain  embryonic  "rests,"  hydatids, 
anomalies  of  the  Miillerian  duct  and  paradidymis. 

The  most  interesting  forms  of  cyst  of  the  testis  are,  however,  to  be 
regarded  as  examples  of  true  tumor-formation.  Of  these  should  be 
mentioned  the  cystoma  (?),  in  which  numerous  cysts  are  present  without 
any  glandular  structure,  the  cystadenoma,  and  epidermoid  cysts.  Some 
of  these  cysts  are  of  mixed  type  and  may  contain  cartilage  and  muscular 
tissue — teratomas.  The  epithelium  lining  them  varies  considerably, 
being  ciliated,  cylindrical,  or  stratified  pavement.  The  contents  of  the 


804  THE  TESTES 

cysts  may  be  mucinous  (cystoma  mucosum),  porridgy  (c.  atheroma- 
tosum),  or  cholesteatomatous.  The  simplest  of  the  teratomas  are  the 
dermoids,  but  many  are  extremely  complicated,  containing  bone,  carti- 
lage, epithelia,  glands,  and  nerves.  The  higher  types  approach  closely 
twin  malformations,  or  the  so-called  foetus  in  fcetu. 

A  point  of  much  interest  and  importance  in  regard  to  teratomas  is 
that  certain  malignant  forms  have  been  observed  by  Breus  and  Schlagen- 
haufer1  which  contain  syncytial  masses  and  cells  corresponding  with 
those  of  the  Langhans'  layer.  The  resemblance  to  deciduoma  malignum 
and  the  hydatidiform  mole  is  close,  and  opens  up  much  room  for  specula- 
tion. Such  cases,  at  all  events,  support  the  view  that  deciduoma  is  a 
growth  of  foetal  and  not  of  uterine  origin. 

Chondroma. — A  good  example  of  heterotopic  tumor-formation  is 
afforded  by  the  chondroma  of  the  testis.  It  forms  nodular  growths  or 
abundant  cylindrical  branching  masses.  In  the  last  type  there  is  a 
development  of  cartilage  within  the  lymphatic  vessels. 

They  may  reach  a  considerable  size  and  form  hard,  warty  growths. 
On  section,  softening,  cystic  degeneration,  and  calcification  are  fre- 
quently to  be  seen.  The  new-growth  usually  begins  in  the  neighborhood 
of  the  rete. 

Microscopically,  it  consists  of  hyaline  or  fibrocartilage.  A  fibrous 
perichondrium  is  common. 

These  tumors  are  met  with  in  children,  and  are  therefore  presumably 
due  to  congenital  defects,  probably  cell-inclusion.  A  striking  pecu- 
liarity is  a  tendency  to  form  metastases,  so  that  the  tumor  is  to  be 
regarded  as  relatively  malignant. 

Myomas. — Myomas,  composed  of  smooth  or  striated  muscle  are 
occasionally  observed.  They  arise  from  preexisting  muscle,  as  from  the 
so-called  "inner"  cremaster,  and  from  remains  of  the  gubernaculum. 

Fibroma,  osteoma,  and  angioma  are  rare. 

Carcinoma. — By  far  the  most  important  malignant  growth  is  carcinoma. 
It  is  most  common  between  the  ages  of  thirty  and  forty,  but  is  met  with 
in  childhood.  There  is  some  difference  of  opinion  as  to  whether  it 
originates  in  the  tubules  or  in  epithelial  cell  "rests."  The  growth  is 
usually  encephaloid,  but  scirrhous  forms  occur.  Colloid  carcinoma  is 
excessively  rare. 

Encephaloid  carcinoma  begins  about  the  centre  of  the  organ  and 
infiltrates  either  as  a  diffuse  growth  or  in  the  form  of  multiple  nodules. 
The  whole  testis  may  be  destroyed,  but,  while  enlarged,  may  for  long 
preserve  a  smooth  surface,  owing  to  the  fact  that  the  tunica  albuginea 
is  very  resisting.  On  section,  the  growth  is  soft  and  brain-like  and  of 
a  grayish  or  yellowish-gray  color.  Fatty  degeneration,  necrotic  and 
hemorrhagic  areas  are  often  seen. 

Microscopically,  the  cells  are  large,  polyhedral,  having  a  pale,  delicate 
protoplasm.  According  to  Langhans,  glycogen  is  abundant.  The 

1  Centralbl.  f.  Gyn.,  27:82:1903;  see,  also,  Carey,  Johns  Hopkins  Hosp.  Bull., 
13: 1902; 275, 


PERIORCHITIS  805 

vessels  of  the  stroma  are  often  numerous  and  dilated  (car.  teleangiec- 
taticum).  Cysts  are  also  sometimes  found. 

Sarcoma. — Sarcoma  is  much  less  common  than  carcinoma,  but  also 
forms  a  rapidly-growing  soft  tumor.  It  is  most  frequently  bilateral. 
On  section,  it  is  more  homogeneous,  smooth,  and  grayish-red  in  color, 
somewhat  like  bacon  in  appearance.  It  is  often  lobulated.  The 
growth  does  not  tend  to  infiltrate  to  the  same  extent  as  carcinoma  and 
in  the  course  of  growth  pushes  the  testis  to  one  side.  Microscopically, 
sarcoma  is  usually  round-celled,  but  spindle-celled  forms  are  seen. 
Plexiform  angiosarcoma  has  been  described. 

A  peculiar  tumor,  somewhat  suggesting  the  intracanalicular  fibroma 
of  the  breast,  is  the  intracanalicular  cystosarcoma,  in  which  the  inter- 
stitial tissue,  in  a  state  of  sarcomatous  proliferation,  grows  into  the 
dilated  tubules. 

Chondrosarcoma  and  myxosarcoma  as  well  as  malignant  glioma  are 
met  with. 

Sarcoma  is  most  frequent  in  childhood  and  in  early  manhood. 


The  Membranes  of  the  Testes. 

The  membranes  surrounding  the  testes  and  spermatic  cords  are  formed 
by  the  evagination  of  the  peritoneal  sac  (processus  vaginalis  peritonei). 
Occasionally,  from  defective  evolution,  the  portion  about  the  testis  does 
not  become  separated  as  it  should,  so  that  there  persists  a  more  or  less 
perfect  communication  with  the  peritoneal  cavity.  This  has  an  im- 
portant bearing  on  the  subject  of  hernia.  In  some  cases  fluid  collects 
in  the  scrotum  about  the  testicle,  forming  one  variety  of  hydrocele. 

Hemorrhage,  usually  between  the  tunica  vaginalis  and  the  tunica 
communis,  may  occur,  forming  a  hematoma.  It  is  usually  due  to  external 
violence,  heavy  muscular  work,  or  coughing,  or  to  the  hemorrhagic 
diatheses. 

Hydrops. — Simple  hydrops  may  be  found  in  general  anasarca. 
In  the  Tropics,  in  association  with  filarial  disease,  a  milky  fluid  is  trans- 
uded into  the  cavity,  the  so-called  "  galactocele,"  or,  more  correctly, 
chylocele. 

Periorchitis. — By  far  the  most  important  affection  of  the  membranes 
is  inflammation,  pericrchitis  or  vaginitis  testis.  This  may  be  acute  or 
chronic,  and  is  usually  secondary  to  lesions  of  the  testis  or  epididymis. 
Occasionally,  it  is  primary  and  due  to  trauma,  and  may  occur  apparently 
spontaneously.  As  might  be  supposed,  periorchitis  is  more  common 
during  the  earlier  period  of  sexual  activity.  It  is  frequent  in  the  Tropics. 
Congenital  malformations  predispose  to  the  condition.  According  to 
the  nature  of  the  process  we  can  recognize  an  exudative  and  a  productive 
form. 

Serous  Periorchitis. — Serous  or  serofibrinous  periorchitis  is  characterized 
by  the  exudation  of  a  serous  or  serofibrinous  fluid  that  often  collects 
in  considerable  quantity,  and  leads  to  great  distension  of  the  sac.  The 


806 


THE  MEMBRANES  OF  THE  TESTES 


FIG.  216 


process  may  be  acute  or,  again,  insidious  in  its  onset.  The  fluid  is  clear 
or  slightly  turbid  from  the  presence  of  leukocytes  and  epithelial  cells 
(acute  hydrocele),  or  may  contain  blood  (hematocele).  The  fibrin  that 

is  formed  tends  to  be  deposited  on 
the  walls  of  the  sac.  Both  the 
epithelium  and  the  superficial  layers 
of  the  sac  may  undergo  fibrinoid 
transformation.  The  subserous  con- 
nective tissue  is  usually  cedematous. 
As  the  process  goes  on,  fibrous 
adhesions  may  be  formed,  binding 
together  more  or  less  firmly  the  two 
layers  of  the  sac  (adhesive  periorchitis) 
In  long-standing  cases  the  exudate  is 
usually  clear  or  tinged  with  blood. 
Not  infrequently,  it  contains  numer- 
ous crystals  of  cholesterin,  giving  it 
a  milky  glistening  appearance.  The 
more  fluid  portions  are  in  some 
cases  absorbed,  leaving  behind  a 
whitish  or  pigmented,  mushy-looking 
mass,  containing  cholesterin. 

Among  the  chief  causes  may  be 
mentioned,  gonorrhoea,  traumatism, 
and  the  infectious  fevers,  such  as 
scarlatina.  Occasionally  in  children 
it  appears  independently  of  any  of 
these  causes. 

A  special  form  of  fibrinous  peri- 
orchitis  is  the  periorchitis  villosa  or 
verrivcosa,  in  which  polypoid  excres- 
cences spring  up,  usually  upon  the 
epididymis.  These  may  be  torn  off 
and  form  free  bodies. 

Suppurative    Periorchitis.  —  Suppu- 
rative    periorchitis    is    characterized 
by  a  purulent  or  fibrinopurulent  exu- 
dation with  much  congestion  of  the 
membranes.     In  some  cases   putrid 
decomposition   of   the   fluid   occurs. 
The  inflammation  may  extend  up  the 
spermatic   cord   to   the   peritoneum, 
or    may   involve    the    scrotum.     In 
some  cases  healing  takes  place  with 
adhesion  of  the  two  layers  of  the  sac. 
The  process  may  be  metastatic,  but  much  more  commonly  is  due  to 
a  preexisting  inflammation,  to  injuries,  gonorrhoea,  or  to  suppurative 
orchitis  or  epididymitis. 


Hydrocele  of  the  tunica  vaginalis.  The 
testicle  is  seen  at  the  lower  portion  of  the 
sac.  (From  the  Pathological  Museum  of 
McGill  University.) 


PERIORCHITIS  807 

Chronic  Serous  Periorchitis. — Chronic  serous  periorchitis  usually 
originates  in  an  acute  affection,  but  may  begin  insidiously.  It  leads  to 
a  considerable  outpouring  of  fluid,  as  much  as  three  liters  in  some  cases, 
which  contains  about  5  per  cent,  of  albumin  and  tends  to  clot  on  standing. 
It  may  contain  blood  and  cholesterin.  In  some  cases,  where  there  is 
spermatocele  or  an  aberrant  vas  deferens,  spermatozoa  may  be  found  in 
the  fluid  (Roth).  The  walls  of  the  sac  are  often  thickened  and  pigmented 
(productive  periorchitis).  There  may  also  be  adhesions. 

In  all  effusions  into  the  tunica  the  testicle  is  usually  situated  in  the 
posterior  portion  of  the  dilated  sac,  and  while  for  some  time  it  may 
retain  fairly  well  its  normal  condition,  it  later  becomes  indurated  and 
compressed,  so  as  to  be  with  difficulty  recognizable.  The  testis  and 
epididymis  are  frequently  atrophic,  not  only  from  pressure,  but  often 
from  preexisting  disease,  the  cause  of  the  original  hydrocele.  When  old 
adhesions  are  present  a  loculated  collection  of  fluid  results. 

Tuberculosis. — This  is  by  no  means  common,  except  when  secondary 
to  tuberculosis  of  the  testis  and  epididymis.  As  a  primary  affection  it 
takes  the  form  of  disseminated  foci  or  large  granulomas. 

Syphilis. — Syphilis  is,  as  a  rule,  met  with  as  an  adhesive  periorchitis 
extending  from  the  testicle.  It  may  be  complicated  with  hydrocele. 
Gummas  are  rare. 

Tumors. — Primary  tumors  of  the  tunica  vaginalis  testis  are  rare. 
Among  them  may  be  mentioned  fibroma,  leiomyoma,  rhabdomyoma, 
lipoma,  myxoma,  chondroma,  dermoid  cysts,  and  sarcoma. 

Parasites. — Echinococcus  disease  is  found  in  the  membranes. 


THE  SCROTUM. 

The  scrotum  is  composed  of  a  modified  skin,  and  the  diseases  affecting 
it,  for  the  most  part,  resemble  those  of  the  skin.  Only  a  few  of  the  more 
important  conditions,  therefore,  will  be  referred  to  here. 

The  structure  is  very  elastic  and  contractile,  owing  to  the  presence  of 
unstriped  muscle  fibers  composing  the  tunica  dartos.  Subcutaneous  fat 
is  absent,  but  bloodvessels  and  lymphatics  are  abundant. 

The  most  important  malformations  are  the  fission  that  occurs  in  hypo- 
spadias  and  hermaphroditism,  and  hypoplasia. 

Owing  to  the  elastic  texture  of  the  scrotum  and  its  great  vascularity, 
hemorrhage  and  oedema  occur  readily  and  are  often  of  extreme  degree. 
(Edema  is  frequently  present  in  cases  of  chronic  valvular  disease  of  the 
heart.  A  local  form  is  also  described,  which  is  possibly  due  to  neuro- 
pathic influences. 

INFLAMMATIONS. 

The  inflammations  are  usually  due  to  external  irritation,  local  infection, 
parasites,  or  extension  from  the  testicle  or  epididymis. 


808  THE  SPERMATIC  CORD 


RETROGRESSIVE  METAMORPHOSES. 

Gangrene. — Among  retrogressive  changes,  gangrene  is  of  frequent 
occurrence.  It  follows  oedema,  extravasation  of  urine,  erysipelas, 
phagedena,  and,  rarely,  infectious  diseases. 


PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — The  most  important  progressive  disorder  is  hyper- 
trophy or  elephantiasis.  Frequently  the  penis  and  scrotum  are  involved 
together.  Two  forms  exist,  the  one  a  diffuse  fibrosis,  and  the  other 
in  which  the  more  or  less  indurated  tissue  contains  numerous  dilated 
lymphatics,  sometimes  forming  on  the  surface  nodular  tumors  or  vesicles 
(lymph  scrotum;  pachydermia  lymphangiectatica).  The  disease  is  most 
common  in  the  Tropics,  and  some  of  the  cases,  at  least,  are  due  to  filariasis. 

Tumors. — Of  the  tumors  may  be  mentioned  fibroma,  lipoma,  fibro- 
myoma,  angioma,  teratoma,  sarcoma,  carcinoma,  and  various  forms  of  cysts. 

The  most  important  new-growth  is  carcinoma,  interesting  chiefly  on 
account  of  its  peculiar  etiology.  It  is  due  apparently  to  irritation,  and 
is  found  in  chimney-sweeps  and  those  working  in  tar  and  paraffin. 
Melanotic  carcinoma  is  described,  but  is  excessively  rare. 

Kocher1  has  described  a  remarkable  giant-celled  sarcoma. 

Parasites. — The  animal  parasites  found  are  the  Filaria  sanguinis 
hominis  and  the  Echinococcus. 


THE  SPERMATIC  CORD,  VAS  DEFERENS,  AND  VESICUL^S 

SEMINALES. 

(Edema. — (Edema  may  lead  to  swelling  of  the  cord  or  diffuse 
hydrocele. 

Varicocele. — A  common  affection  is  dilatation  of  the  veins,  or 
varicocele.  The  disease  is  commonest  in  early  adult  life.  It  is  occasion- 
ally due  to  the  obstruction  to  the  free  outflow  of  blood  by  tumors  or 
hernia,  but  frequently  appears  without  obvious  cause.  It  may  be  that 
there  is  some  congenital  weakness  of  the  vessels  that  predisposes.  The 
veins  in  question  are  poor  in  valves,  and  thus  a  long  column  of  blood  has 
to  be  supported.  Among  the  causes  to  which  the  condition  is  attributed 
are  prolonged  standing,  violent  muscular  exertion,  sexual,  excess,  gonor- 
rhoea, and  traumatism.  The  affection  is  usually  found  on  the  left  side, 
owing  to  the  fact  that  the  left  spermatic  vein  does  not  empty  directly 
into  the  vena  cava  inferior  but  into  the  left  renal  vein,  and  also  lies 
behind  the  rectum. 

1  Deutsche  Chir.,  506:  1887. 


SPERM  A  TOCYSTITIS  809 

Funiculitis. — Inflammation  of  the  cord — funiculitis — is  due  to  the 
extension  of  a  posterior  urethritis  or  to  traumatism. 

Tumors. — Of  primary  tumors  should  be  mentioned  lipoma,  fibroma, 
myxoma,  my xo fibroma,  and  sarcoma.  They  are  rare.  Metastatic  deposits 
may  occur  in  sarcoma  or  carcinoma  of  the  testis. 

Cysts  are  due  to  localized  hydroceles  of  the  cord,  or  develop  from 
remains  of  the  Wolffian  body  (?),  duct. 

Deferenitis. — The  most  important  affection  of  the  vas  deferens  is 
inflammation,  deferenitis  or  spermatitis.  This  is  due  to  the  extension 
of  inflammation  from  the  urethra,  bladder,  prostate,  or  epididymis. 
Rarely  it  is  idiopathic  or  due  to  trauma.  The  usual  cause  is  gonorrhoea. 
Obliteration  of  the  vas  and,  if  the  condition  be  bilateral,  sterility  is  the 
result. 

Tuberculosis. — Tuberculous  deferenitis  arises  by  extension  from 
the  associated  organs. 

Syphilis. — Gummas  have  been  occasionally  observed. 

The  seminal  vesicles  may  be  absent  on  one  or  both  sides,  or  may  be 
fused.  The  first  condition  is  usually  associated  with  unilateral  defect 
of  the  kidney,  vas,  and  epididymis. 

Hypoplasia  occurs  in  anorchidism. 

Spermatocystitis. — Inflammation  of  the  seminal  vesicles,  Sperma- 
tocystitis, is  usually  an  extension  from  the  vas.  Rarely,  it  occurs  from 
trauma,  or  even  without  obvious  cause.  It  is  usually  due  to  gonorrhoea. 
The  inflammation  may  be  simple,  mucoid,  mucopurulent,  purulent, 
hemorrhagic,  or  caseous.  The  walls  of  the  vesicles  are  infiltrated  with 
inflammatory  products,  and  the  cavities  are  filled  with  leukocytes, 
chiefly  mononuclear,  desquamated  epithelium,  debris,  and,  if  the  duct 
be  not  occluded,  with  spermatozoa. 

In  tuberculosis  there  is  a  caseous  detritus  and  the  walls  show  tuber- 
culous infiltration. 

In  advanced  cases  of  Spermatocystitis  the  walls  are  thickened  and  the 
cavities  may  be  contracted.  In  old  men  who  have  suffered  from  chronic 
gonorrhoea,  hypertrophy  of  the  prostate,  vesical  calculus,  or  stricture, 
diverticula  are  sometimes  found,  owing  to  irregular  proliferation  of 
fibrous  tissue.  Occasionally  cystic  dilatation  is  the  result.  The  contents 
of  the  vesicles  may  in  some  cases  become  inspissated  and  infiltrated  with 
salts,  so  that  concretions  are  produced. 


CHAPTER    XXXVIII. 

THE  FEMALE  SEXUAL  ORGANS. 

THE  female  organs  of  generation  include  the  vulva  (external  genitalia), 
the  vagina,  the  uterus,  Fallopian  tubes,  ovaries,  and  broad  ligaments 
(internal  genitalia).  Inasmuch,  however,  as  the  mammae  are  closely 
connected  with  the  function  of  reproduction  and  attain  their  full  develop- 
ment only  in  the  female,  it  is  convenient  to  discuss  diseases  of  the  breast 
also  under  this  category.  Affections  of  the  placenta  and  foetal  mem- 
branes will  also  be  treated  here. 

A  great  variety  of  malformations  affecting  the  genital  tract  in  whole 
or  in  part  are  described.  Hermaphroditism  is  discussed  in  another  place 
(see  vol.  i,  p.  257).  Doubling  of  the  genital  organs  as  a  whole,  including 
also  the  bladder  and  urethra,  is  recorded  in  one  case.1 

A  more  common  anomaly  is  hypoplasia,  either  where  the  usual  changes 
incident  to  puberty  are  retarded,  or  where  the  organs  remain  small  or 
imperfect  throughout  life. 

THE  EXTERNAL  GENITALIA. 

These  are  the  vulva,  including  the  clitoris,  labia  majora,  labia  minora, 
the  hymen,  and  certain  associated  glands — the  Cowper's  or  Bartholini's 
glands. 

CONGENITAL  ANOMALIES. 

Complete  defect  of  the  vulva  is  found  in  acephalic  monsters  and  siren 
deformities.  More  common  are  defects  of  certain  parts,  either  bilateral 
or  unilateral.  Such  are  absence  of  the  labia  minora,  the  labia  majora, 
the  clitoris,  or  the  labia  minora  and  clitoris  together.  Besides  aplasia, 
hypoplasia  exists,  generally  associated  with  retarded  development  of  the 
internal  organs.  The  vulva  in  the  adult  may  present  the  characteristics 
of  infancy  (vulva  infantilis). 

Fission  of  the  clitoris  simulating  reduplication  has  been  met  with.  It 
is  usually  associated  with  epispadias.  Hypertrothy  of  the  clitoris  affects 
the  prepuce  alone  or  the  organ  as  a  whole.  The  affection  is  more 
common  in  tropical  countries.  It  is  found  occasionally  in  the  black 
races,  in  pseudohermaphroditism,  and,  rarely,  in  prostitutes.  In  certain 
cases,  hypertrophy  of  the  clitoris  is  combined  with  hyperplasia  of  the  labia. 

1  V.  Engel,  Arch.  f.  Gyniik.,  29:1887:  43. 


VULVITIS  811 

In  certain  races,  as  Bushmen  and  Hottentots,  the  labia,  particularly 
the  labia  minora,  are  excessively  enlarged,  sometimes  almost  reaching 
the  knees  (Hottentot  apron).  Enlargement  of  the  labia  majora  is  said 
by  some  to  be  due  always  to  new-growth. 

An  increase  in  the  number  of  the  labia  is  rare,  less  so  in  the  case  of 
nymphse.  Congenital  adhesions  of  the  labia,  especially  the  labia  minora, 
exist  and  may  lead  to  interference  with  the  function  of  urination. 

Hernia  into  the  labium  majus  through  the  inguinal  canal  may  occur 
(hernia  inguinalis  labialis),  or  may  extend  from  beneath  the  ramus  of 
the  pubes  into  the  lower  part  of  the  labium  (hernia  labialis  inferior). 

The  urogenital  sinus  may  fail  to  develop  naturally,  and  thus  per- 
sistence of  an  embryonic  condition  may  occur,  such  as  epispadias, 
hypospadias,  and  anus  preternaturalis  vestibularis. 

The  hymen  presents  great  variation  in  its  form.  It  may  be  com- 
pletely occluded  or  may  possess  merely  a  small  opening.  Or,  again,  the 
opening  may  be  double  (hymen  septus),  sieve-like  (hymen  cribriformis), 
or  serrated  (hymen  fimbriatus). 


CIRCULATORY  DISTURBANCES. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  is  due  to  inflam- 
mation, or  to  mechanical  irritation,  as  in  the  sexual  act. 

Passive  Hyperemia. — Passive  hyperemia  is  commonly  due  to  pregnancy, 
or  to  general  blood  stasis,  as,  for  instance,  in  valvular  disease  of  the 
heart. 

If  the  condition  persist,  the  vessels,  especially  those  of  the  labia  majora, 
become  dilated  and  varicose.  Thrombosis  then  may  occur  with  possibly 
phlebolith  formation,  or  a  venule  may  burst,  leading  to  large  extrav- 
vasations  of  blood  (hematoma).  Rupture  of  the  veins  may  also  take 
place  from  external  traumatism  or  from  parturition. 

Hemorrhage. — Hemorrhage  from  tearing  of  the  hymen  at  the  first 
coitus  is  rarely  dangerous.  It  is  most  likely  to  be  severe  when  the 
orifice  is  small  or  the  hymen  thick  and  fleshy. 

(Edema. — (Edema  is  common  and  may  be  associated  with  inflam- 
matory hyperemia,  passive  congestion,  or  vascular  changes.  It  is  found 
especially  in  valvular  heart  affections  and  in  nephritis. 


INFLAMMATIONS. 

The  inflammatory  affections  of  the  vulva  are  practically  those  found 
on  any  skin  surface.  Among  them  may  be  mentioned  erythema,  eczema, 
herpes,  acne,  furunculosis,  hard  and  soft  chancre,  condylomas,  impetigo, 
phlegmon,  erysipelas,  diphtheritis,  gangrene,  and  lupus. 

Vulvitis. — Acute  Vulvitis. — Acute  vulvitis  or  vulvovaginitis  is  com- 
monly due  to  gonorrhoea  and  occurs  chiefly  in  children  from  mediate 


812 


THE  VULVA 


infection.  It  is,  however,  also  met  with  in  adults.  Cases  are  some- 
times due  to  uncleanliness  or  the  irritation  of  thread-worms.  The  labia 
and  clitoris  are  reddened,  oedematous,  and  bathed  in  pus.  The  follicles 
may  also  be  involved.  Excoriation  is  common. 

Catarrhal  Vulvitis. — Simple  catarrhal  vulvitis  may  be  due  to  dirt, 
mechanical  irritation,  or  irritating  discharges. 

Phlegmonous  Vulvitis. — Phlegmonous  vulvitis  has  been  known  to 
follow  injuries  in  labor. 

Membranous  Vulvitis. — Membranous  vulvitis  may  be  due  to  diphtheria, 
or  may  be  merely  diphtheroid.  The  latter  form  occurs  in  many  of  the 
infections,  as  puerperal  sepsis,  measles,  typhoid,  scarlatina,  and  cholera. 
It  may  also  originate  in  the  extension  of  inflammation  from  the  bowel 
or  vagina  and  may  terminate  in  gangrene. 


FIG.  217 


Condylomas  of  the  vulva.     (From  the  Gynecological  Clinic  of  the  Montreal  General  Hospital.) 

Inflammation  of  the  Bartholini's  glands  is  commonly  due  to  gonorrhoea, 
but  may  be  an  extension  from  vulvitis  of  other  forms.  It  gives  rise  to 
catarrh  of  the  duct,  with  retention  of  the  secretion.  Abscess  frequently 
results.  Inflammation,  when  of  some  standing,  leads  to  productive 
changes  and  often  adhesion  of  the  nymphse.  Induration  of  the  Bartho- 
lini's glands  also  occurs. 

Condyloma. — Condylomas  are  inflammatory  outgrowths  on  the  mucous 
membrane  of  the  vulva  and  vagina  or  the  skin  near  by.  They  are 
either  acuminate  or  nodular,  and  tend  to  be  produced  wherever  there  is 
heat  and  moisture.  They  are  common  in  syphilis,  but  may  also  be 
due  to  irritating  discharges,  gonorrhoea,  etc. 


KRAUROSIS   VVLVJE  813 

Chancroid. — Chancroid  or  ulcus  molle  is  found  upon  the  labia  majora 
and  minora,  the  fourchette,  and  meatus  urinarius.  The  ulcers  tend  to 
be  multiple,  as  they  are  auto-inoculable.  They  take  the  form  of  punched- 
out  excavations  secreting  pus. 

Syphilis. — The  lesions  of  syphilis  are  protean.  We  may  have  the 
primary  sore,  secondary  eruptions,  mucous  patches,  condylomas,  and, 
rarely,  gummas. 

Tuberculosis. — Tuberculosis  assumes  the  form  of  lupus  or  rapid 
caseous  ulcer ation. 

It  should  be  mentioned  that  there  has  been  described  a  form  of  chronic 
ulceration,  very  refractory  to  treatment,  leading  to  inflammatory  hyper- 
plasia,  that  has  been  regarded  by  some  as  tuberculous,  by  others  as 
syphilitic.  Koch  believes  that  it  is  due  to  lymph  stasis  following  destruc- 
tion of  the  inguinal  glands.  Microscopically,  the  appearances  are  those 
of  simple  inflammation. 

Actinomycosis. — Actinomycosis  of  the  vulva  is  very  rare,  only  two 
cases  being  on  record.1 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — After  the  climacteric  the  labia  majora  undergo  atrophy, 
owing  to  the  absorption  of  the  fat,  and  become  small  and  relaxed,  tending 
to  expose  the  vulvar  orifice.  Atrophy  of  the.  nymphse  also  occurs. 

Kraurosis  Vulvae. — A  curious  and  rare  form  of  atrophy  and  contraction 
of  the  vulva,  first  described  by  Breisky,2  is  the  so-called  kraurosis  vulvae. 
The  etiology  of  the  condition  is  still  doubtful  but  it  may  be  inflammatory 
in  origin.  The  disease  involves  the  vestibule,  vagina,  labia  majora, 
clitoris,  and  the  inner  part  of  the  labia  minora.  The  mucous  membrane 
is  stretched,  smooth,  glistening,  dry,  inelastic,  and  pale  reddish-gray  in 
color.  The  surface  is  often  fissured  and  enlarged  bloodvessels  can  be  seen. 

Microscopically,  according  to  Peter,3  there  is  at  first  chronic  inflam- 
mation with  round-celled  infiltration  and  inflammatory  oedema  of  the 
corium  and  epidermis,  and  later  atrophy  of  the  upper  layers  of  the  corium, 
especially  the  papillae.  With  this  there  is  a  marked  tendency  to  con- 
traction with  hyperplasia  of  the  connective  tissue.  Very  little  can  be 
seen  of  the  sebaceous  and  sudoriparous  glands,  and  there  is  loss  of  the 
elastic  tissue. 

Gangrene. — Gangrene  of  the  vulva  results  from  simple  or  inflam- 
matory oedema,  hemorrhage,  thrombosis,  and  from  traumatism.  It 
is  found  in  certain  infectious  diseases,  as  typhoid,  measles,  scarlatina, 
and  variola. 

Certain  special  forms  deserve  mention.  These  are  phagedena  from 
chancroid  and  noma.  Noma  vulvse  occurs  under  the  same  circum- 

1  Bongartz,  Monatssch.  f.  Geb.  u.  Gyn.,  3:  1896:  4. 

2Zeit.  f.  Heilk.,  6:  1885:  69. 

3  Monatsschr.  f,  Geb.  u.  Gynak.,  3:1896:297. 


814  THE  VULVA 

stances  as  noma  of  the  cheek.     It  is  found  in  anemic  and  debilitated 
children. 

Concretions. — Concretions  may  form  behind  the  prepuce  of  the 
clitoris. 

SOLUTIONS  OF  CONTINUITY. 

These  occur  during  coitus  and  parturition.  Small  fissures  may  be 
produced,  or,  again,  extensive  lacerations.  The  most  important  form 
is  laceration  of  the  perineum  during  labor. 


PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Cutaneous  horns  due  to  hypertrophy  of  the  epithe- 
lium have  been  met  with  on  the  clitoris. 

Elephantiasis. — The  most  important  and  interesting  of  the  hyper- 
trophies is  elephantiasis.  The  etiology  is  by  no  means  perfectly  under- 
stood and  undoubtedly  varies  in  different  cases.  Some  cases  are  con- 
genital, in  the  sense,  at  least,  that  at  birth  there  is  a  hyperplastic 
enlargement  of  the  parts  that  subsequently  becomes  excessive,  or  that 
there  is  an  inherited  predisposition  to  the  disease  (pachydermatocele, 
eleph.  mollis).  Other  cases  are  undoubtedly  due  to  chronic  inflamma- 
tion or  to  some  affection  of  the  lymph-glands  and  vessels.  The  latter 
is  generally  caused  by  obstruction  to  the  outflow  of  the  lymph  and  may 
be  caused  by  filarise  and  inflammatory  fibrosis.  The  condition  has  been 
known  to  follow  suppuration  of  the  inguinal  glands.  The  disease  may 
be  unilateral  or  bilateral,  and  affects  the  labia  majora,  less  frequently 
the  nymphse  and  clitoris.  The  surface  is  either  smooth  (eleph.  glabra) 
or  nodular  (eleph.  tuberosa).  The  anatomical  forms  vary.  In  one  type 
the  parts,  as  a  whole,  are  enlarged  and  the  normal  contour  of  the  tissues 
is  destroyed  by  a  subcutaneous  oedema  or  connective-tissue  hyperplasia. 
In  whatever  way  the  process  starts  it  is  liable  to  be  complicated  by  in- 
flammatory changes,  such  as  induration,  ulceration,  and  gangrene. 

Tumors. — Fibromas. — Fibromas  have  been  found  in  the  labia  majora, 
less  commonly  in  the  labia  minora  and  clitoris.  They  are  either  massive 
or  pedunculated,  and  may  weigh  several  pounds.  They  originate  in 
the  subcutaneous  connective  tissue,  in  the  fascia,  or  in  the  periosteum 
of  the  pelvis  (Kiwisch).  Structurally,  fibromas  are  composed  of  a  loose, 
often  oedematous  connective  tissue,  or  occasionally  are  in  part  mucoid 
(myxo fibroma).  Owing  to  hemorrhage  or  degeneration  they  may  become 
cystic.  Myoma  and  fibromyoma  have  also  been  described.  Rarely,  they 
arise  from  the  end  of  the  round  ligament.  Lipomas  are  rather  un- 
common, and  develop  in  the  mons  veneris  and  greater  labia.  They  may 
be  combined  with  angioma.  Hemangioma  and  lymphangioma  are  also 
met  with.  Among  the  greatest  rarities  is  chondroma  of  the  clitoris, 
occasionally  associated  with  softening,  calcification,  or  true  bony  growth. 
Pigmented  nevi  are  met  with  in  children  on  the  labia.  Adenoma  of  the 
Bartholini's  glands  13  rare. 


CONGENITAL  ANOMALIES  815 

Carcinoma. — Carcinoma  of  the  vulva  is  relatively  common.  It  arises 
usually  from  the  labia,  clitoris,  commissures,  or  urethra.  It  occurs  as 
a  papillary  or  nodular  outgrowth  or  as  a  diffuse  infiltration.  Extensive 
ulceration  may  occur.  The  growth  is,  as  a  rule,  of  the  type  of  horny 
epithelioma  or  cancroid,  but  scirrhous  and  soft  forms  are  also  met  with. 
Carcinoma  is  commonest  in  the  later  years  of  life,  and  appears  to  be  re- 
lated to  chronic  thickening  of  the  epithelium,  such  as  occurs  in  pruritus. 
Carcinoma  may  also  arise  from  the  Bartholini's  glands. 

Sarcoma. — Much  rarer  than  carcinoma  is  sarcoma,  which  usually  takes 
the  form  of  melanosarcoma.  Round-  and  spindle-celled  forms  as  well  as 
myxosarcoma  have  been  described. 

A  secondary  melanotic  hypernephroma  of  the  labium  minus  has  been 
described.1 

Cysts  are  of  various  kinds,  either  degenerative,  retention,  or  develop- 
mental. Some  arise  from  a  local  collection  of  fluid  in  the  canal  of 
Nuck,  or  from  remains  of  Gartner's  duct;  others  from  obstruction 
to  the  duct  of  a  Bartholini's  gland.  They  may  be  found  on  the  labia 
and  hymen. 

THE  VAGINA. 

The  vagina  in  adults  is  a  potential  tube,  the  walls  of  which  are  normally 
in  contact,  composed  of  connective  tissue  in  which  are  numerous  bands 
of  unstriped  muscle.  It  is  lined  with  a  mucous  membrane,  consisting 
of  stratified  pavement  epithelium,  the  cells  in  the  lower  layers  of  which, 
however,  tend  to  be  cylindrical.  The  mucous  membrane  is  not  smooth 
but  thrown  up  into  papillae  and  transverse  ridges  or  rugae.  As  a  rule,  it 
contains  no  glands,  but  there  are  certain  lacunae  or  crypts  opening  be- 
tween the  papillae  and  folds.  In  a  few  cases  glands  lined  with  ciliated 
epithelium  have  been  observed  (v.  Preuschen2).  In  the  submucous 
connective  tissue  small  clumps  of  lymphoid  cells  are  to  be  found. 

By  repeated  coitus  or  the  act  of  parturition  the  rugse  become  gradually 
obliterated,  and  as  old  age  comes  on  involution  takes  place.  The 
mucosa  becomes  atrophic  and  the  lumen  more  or  less  contracted. 


CONGENITAL  ANOMALIES. 

The  vagina  may  be  completely  absent  or  represented  only  by  a  fibrous 
cord.  In  other  cases  it  is  more  or  less  rudimentary  and  contracted  for 
its  full  length  (total  atresia).  Partial  atresia  also  occurs  in  the  lower 
portion  of  the  vagina.  It  may  consist  in  a  membranous  occlusion 
(atresia  vaginalis)  or  in  an  imperf orate  hymen  (atresia  hymenalis). 
When  the  vagina  is  completely  absent,  there  is  defect  of  the  uterus 
and  generally  of  the  adnexa.  Partial  atresia  after  the  establishment  of 

1  Grafenberg,  Virch.  Archiv,  194:  1908: 17. 
2Virch.  Archiv,  70:1877:111. 


818  THE  VAGINA 

injuries  during  parturition,  the  irritation  of  foreign  bodies,  puerperal 
sepsis,  discharges  from  cancers  or  fistulse,  rarely  gonorrhoea,  polypi. 
True  diphtheria  is  rare. 

Phlegmonous  Vaginitis. — Phlegmonous  or  erysipelatous  vaginitis  has 
been  described.  In  this  there  is  diffuse  suppuration  in  the  vaginal  wall 
that  may  lead  to  exfoliation  of  tissue. 

Aphthous  Vaginitis. — Aphthous  vaginitis  is  due  to  a  microorganism 
resembling  the  oi'dium  albicans.  The  mucosa  is  reddened  and  studded 
with  white,  elevated  patches. 

Chancroids. — Chancroids  are  not  common  in  the  vagina. 

Emphysematous  Vaginitis. — A  curious  affection  is  the  so-called  emphy- 
sematous  vaginitis  (colpohyperplasia  cystica,  emphysema  vaginci).  This 
disease  is  met  with  usually  in  pregnant  women  or  shortly  after  con- 
finement, but  has  been  found  in  others.  In  the  vaginal  wall  are 
numerous  small  vesicles  or  cysts,  varying  in  size  from  that  of  a  millet- 
seed  to  that  of  a  hazelnut.  Otherwise  the  mucosa  appears  to  be 
normal.  Numerous  opinions  (Winckel,  Schroeder,  Eppinger,  Zweifel, 
Klebs,  Chiari)  have  been  expressed  as  to  the  cause.  The  most  recent 
investigator,  Lindenthal,1  finds  that  it  is  due  to  an  anaerobic  gas- 
producing  microorganism,  belonging  to  the  malignant  oedema  group. 
The  bacillus  coli  is  not  a  cause,  except  possibly  in  diabetic  individuals. 

Inflammatory  processes  may  extend  from  the  vagina  or  rectum  into 
the  perivaginal  connective  tissue  and  lead  to  abscess  formation  or  fibrosis 
(perivaginitis  suppurativa,  perivag.  fibrosa). 

Tuberculosis. — Tuberculosis  is  uncommon,  and  as  a  primary  affec- 
tion, excessively  rare.  As  a  rule,  it  extends  from  the  uterus,  vulva,  or 
anus,  but  exceptionally  is  metastatic.  It  takes  the  form  of  shallow 
ulcers  with  irregular  edges  and  nodular  bases  that  tend  to  coalesce. 
Less  commonly,  one  finds  grayish  tubercles  or  caseating  granulomas. 

Syphilis. — The  primary  papule  or  sclerosis  of  syphilis  is  rare  in  the 
vagina.  It  is  found  at  the  entrance  or  near  the  posterior  commissure. 
Erythema,  psoriasis,  condylomas,  and  gummas  are  also  met  with. 
Birch-Hirschfeld2  has  described  a  perivaginitis  gummosa,  in  which  the 
vagina  is  converted  into  a  stiff,  fibrous  tube. 

RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  rugae  and  of  the  mucous  membrane  in 
general  has  already  been  referred  to  (p.  815). 

In  prolapse  of  the  vagina  the  mucosa  becomes  converted  into  a  struc- 
ture closely  resembling  skin,  with  stratum  granulosum  and  keratiniza- 
tion. 

Gangrene. — Gangrene  may  result  from  traumatism,  pressure,  in- 
flammation, carcinoma,  etc. 

1  Beitr.  zur  Aetiol.  u.  Histol.  der  Colpohyperplasia  cystica;  Zeit.  f.  Geb.  u.  Gyn., 
40: 1899:375. 

2  Lehrbuch,  2:1887:794. 


FOREIGN  BODIES  AND  PARASITES  819 

Circumscribed  ulcers  due  to  ischemic  necrosis  have  been  described  by 
Zahn.1 

PROGRESSIVE  METAMORPHOSES. 

These  are  rather  uncommon. 

Hypertrophy. — Hypertrophy  of  the  vaginal  wall  from  muscular 
overactivity  is  found  in  association  with  atresia  and  retained  fluid. 
Mucous  polyps,  sometimes  of  considerable  size,  are  met  with.  They  are 
usually  found  on  the  posterior  wall.  The  inflammatory  new-growths, 
such  as  hyperplastic  papillae  and  condylomas  have  been  referred  to  already. 

Tumors. — Myoma. — Sessile  or  pedunculated  myomas,  composed  either 
of  smooth  or  striped  muscle,  grow  occasionally  from  the  anterior  vaginal 
wall.  They  may  be  of  several  pounds'  weight.  In  contradistinction  to 
myomas  of  the  uterus  they  are  rarely  multiple. 

Sarcoma. — In  young  children  multiple  papillary  or  polypoid  sarcomas 
or  fibrosarcomas  (see  vol.  i,  p.  607),  often  containing  muscle,  are  some- 
times found.2  They  appear  to  be  congenital.  The  neoplasms  may 
remain  latent  for  years  and  then  take  on  rapid  growth.  Microscopically, 
the  growths  consist  of  round  or  spindle  cells.  In  adults,  round-,  spindle-, 
and  giant-celled  sarcomas  are  met  with,  and  also  a  diffuse  sarcomatous 
infiltration  of  the  vaginal  wall.  Myxomatous  degeneration  may  occur. 
Secondary  sarcoma  has  been  observed. 

Carcinoma. — Primary  carcinoma  (epithelioma)  occurs  not  infrequently, 
and  takes  the  form  of  a  cauliflower-like  mass  starting  from  the  posterior 
wall.  Exceptionally,  a  more  diffuse  or  ring-like  infiltration  of  a  scirrhous 
or  medullary  character  is  met  with. 

More  common  are  secondary  carcinomas  that  have  extended  from 
the  uterus,  rectum,  bladder,  urethra,  or  vulva.  Metastatic  growths 
originating  from  the  uterus  or  ovaries  are  rare,  as  are  the  cases  of 
implantation  of  cancer  cells  from  the  uterus. 

Carcinoma  of  the  vagina  extends  rapidly  and  is  liable  to  ulcerate,  so 
that  fistulse  are  quickly  produced. 

Cysts. — Cysts  originate  in  remains  of  the  Wolffian  or  Gartner's  ducts, 
from  implantation  of  epithelium,  or  from  dilated  lymphatics. 

Foreign  Bodies  and  Parasites.— Apart  from  bacteria,  may  be 
mentioned  the  larva?  of  certain  flies,  Oxyuris  vermicularis,  Ascaris, 
Trichomonas  vaginalis,  O'idium  albicans,  Manila  albicana,  Manila 
Canada,  and  various  yeasts. 

Echinococciis  cysts  may  penetrate  the  vagina  from  the  intestine. 

A  variety  of  foreign  bodies  have  been  found  in  the  vagina,  as,  for 
instance,  feces,  urinary  calculi,  calcified  myomas  from  the  uterus, 
pessaries,  catheters,  tampons,  portions  of  instruments,  and  objects 
introduced  for  purposes  of  masturbation. 

1  Virch.  Archiv,  95:  1884:  388;  and  95:  1889:  167. 

2Kolisko,  D.  polypose  Sarkom  der  Scheide  im  Kindesalter,  Wien.  klin.  Woch., 
6:1889:119. 


820  THE  UTERUS 

Foreign  bodies,  if  they  cannot  be  absorbed  or  discharged,  become 
encrusted  with  triple  phosphates  and  carbonates. 


THE  UTERUS. 

The  uterus  is  a  hollow  viscus,  composed  chiefly  of  unstriped  muscle, 
with  a  certain  admixture  of  connective  tissue,  and  lined  with  a  mucous 
membrane  of  peculiar  type.  The  organ  is  formed  by  the  fusion  of  the 
Miillerian  ducts  and  undergoes  certain  minor  modifications  of  its  form 
in  passing  from  the  infantile  to  the  adult  and  functioning  type. 

In  the  adult  nullipara,  the  uterus  measures  from  5.5  to  8  cm.  in 
length,  and  in  the  parous  woman,  9  to  9.5  cm.  Its  breadth  varies 
from  3.5  to  4  and  5.5  to  6  cm.  respectively.  The  organ  contains 
abundant  lymphatics,  of  which  those  of  the  cervix  discharge  into  the 
iliac,  and  those  of  the  corpus  into  the  lumbar  and  inguinal  glands.  The 
mucosa  is  about  1  mm.  thick,  and  is  composed  of  a  delicate,  reticulated, 
fibrous  stroma,  containing  numerous  lymphoid  cells.  In  this  are 
abundant  spiral  and  branching  glands  or  crypts  (glandulse  utriculares), 
composed,  like  the  mucosa  lining  the  cavity,  of  a  single  layer  of  ciliated, 
cylindrical  cells.  The  mucosa  of  the  cervix  is  thinner  and  more  compact, 
containing  short  gland-tubes,  many  of  the  cells  of  which  are  mucin- 
producing.  The  vaginal  portion  is  covered  externally  with  squamous 
epithelium  like  the  vagina.  A  submucosa  does  not  exist  in  the  uterus, 
but  the  glands  pass  directly  down  to  the  muscle,  or  even  into  it. 

During  menstruation  the  uterus,  being  to  some  extent  an  erectile  organ, 
is  congested.  The  mucous  membrane  is  especially  hyperemic,  and 
hemorrhage  takes  place  from  its  surface.  Not  only  this,  but  there  is 
hyperplasia  both  of  the  stroma  and  of  the  glands,  which  become  larger 
and  more  elongated.  Later,  the  superficial  epithelium,  and  to  some 
extent  that  of  the  glands,  is  cast  off.  Finally,  the  remaining  cells  pro- 
liferate and  the  mucosa  is  restored  to  its  former  condition. 

In  pregnancy  the  uterus  becomes  greatly  enlarged,  relatively  more  so 
in  the  corpus.  In  the  first  half  of  gestation  there  is  said  to  be  a  true  new- 
formation  of  muscle  fibers,  but  later  on  there  is  simple  hypertrophy. 
In  the  cervical  portion,  in  addition  to  some  hypertrophy,  there  is  a  notable 
increase  in  the  elastic  fibers.  The  vessels  become  thickened  and  greatly 
lengthened,  and  the  veins  and  lymphatics  are  dilated.  The  mucous 
membrane  is  converted  into  decidua,  which,  in  the  later  stages,  undergoes 
fatty  and  necrotic  changes. 

After  delivery  the  uterus  becomes  greatly  reduced  in  size,  at  first  from 
contraction,  and  later  from  atrophy  of  the  muscle  fibers,  which  also 
show  cloudy,  hyaline,  and  fatty  degeneration.  The  process  of  involu- 
tion usually  takes  about  six  weeks.  The  involuted  uterus  never  quite 
regains  its  former  appearance.  The  corpus  remains  relatively  large 
and  thick-walled,  and  the  cavity  is  enlarged.  The  os  is  almost 
invariably  fissured  and  the  vessels  are  large,  tortuous,  and  may  show 
signs  of  endarteritis. 


CONGENITAL  ANOMALIES  821 


CONGENITAL  ANOMALIES. 

Two  great  classes  of  anomalies  may  be  recognized,  the  first  due  to 
nutritive  disorders  (dystrophies),  resulting  in  abnormality  in  the  size  of 
the  organ;  the  second,  due  to  eccentricities  of  development  (dysplasias). 
To  a  certain  extent  both  types  may  be  associated. 

Hyperplasia  of  the  uterus  is  occasionally  observed,  and  enlargement  of 
the  organ  is  described  in  connection  with  many  of  the  dysplasias.  The 
uterus  may  be  completely  wanting  (aplasia)  or  diminutive  in  size  (hypo- 
plasia).  Hypoplasia  is  symmetrical  or  asymmetrical,  according  as  the 
Miillerian  ducts  are  equally  or  unequally  involved. 

Transverse  fission  of  the  external  os  is  sometimes  met  with  and  may 
be  confused  with  that  resulting  from  childbirth. 

The  dysplasias  are  to  be  divided  into  five  classes:  (1)  partial  or 
complete  separation  of  the  two  Miillerian  ducts;  (2)  imperfect  fusion 
of  the  ducts;  (3)  imperfect  development  of  the  fundus;  (4)  anomalies  of 
the  cavity;  (5)  faulty  relationship  with  neighboring  structures. 

Complete  aplasia  of  the  uterus  is  very  rare.  As  a  rule,  on  careful 
examination,  scanty  rudiments  can  be  discovered.  In  these  cases  the 
vagina  and  external  genitals  may  be  present.  The  tubes  and  ovaries 
may  be  present  or  absent.  When  the  uterus  is  partly  formed,  the  cervical 
portion  is  often  absent  or  represented  by  a  solid  mass  of  muscle. 

Another  class  of  cases  is  that  in  which  there  is  an  unequal  develop- 
ment of  the  Miillerian  ducts  leading  to  asymmetry  of  the  uterus.  The 
most  marked  example  of  this  is  where  one  duct  almost  completely  fails 
to  develop.  This  results  in  the  uterus  unicornis.  On  the  affected  side 
the  ovary  may  be  well-formed,  but  cases  are  on  record  where  it  was 
defective  as  well  as  the  tube,  together  with  absence  of  the  ureter  and 
kidney  of  the  same  side. 

Development  of  the  Miillerian  ducts  without  fusion  results  in  the  forma- 
ation  of  two  separate  uteri  and  vaginae  (uterus  didelphys).  In  certain 
rare  instances  both  uteri  may  open  into  one  vagina. 

In  another  class  of  cases,  fusion  of  the  two  ducts  takes  place  only 
in  the  lower  portion  of  the  uterus,  while  above  the  ducts  remain  separate 
(uterus  bicornis).  The  amount  of  separation  is  very  variable.  The  line 
of  division  may  be  indicated  by  a  mere  depression  (uterus  arcuatus  sive 
bifundalis).  In  other  cases  it  is  anvil-shaped  (uterus  incudiformis). 
In  the  most  extreme  form  the  line  of  cleavage  extends  down  to  or  even 
into  the  cervical  portion.  Sometimes  between  the  halves  there  is  found 
a  vesicorectal  ligament.  Rudimentary  and  asymmetrical  forms  of 
uterus  bicornis  also  exist.  The  uterine  cavities  may  be  entirely  separate 
(uterus  bicornis  duplex)  or  may  unite  at  the  cervix  (uter.  bicorn.  unicollis). 
In  the  first  case  there  may  be  a  double  vagina  or  one  with  a  septum, 
but  this  is  not  invariable. 

When  the  cavity  is  more  or  less  perfectly  divided  by  a  septum  the  con- 
dition is  called  uterus  bilocularis  or  septus.  The  septum  may  vary  in 
completeness.  When  it  extends  from  the  fundus  only  a  short  distance 


822  THE  UTERUS 

into  the  cavity  we  speak  of  uterus  bilocularis  subseptus;  when  it  reaches 
the  cervix,  uter.  biloc.  unicollis;  in  other  cases  the  septum  is  only  found 
at  the  external  os,  uterus  biforis;  in  still  others,  the  septum  is  only  present 
in  the  cervix,  uter.  bicollis  unicorporeus. 

Abnormalities  in  the  formation  of  the  cavity  are  common  in  the 
rudimentary  uterus.  The  cavity  may  be  completely  absent  or  there 
may  be  one  or  more  rudimentary  cavities.  It  may  be  narrower  than 
normal  or  obstructed  (stenosis  uteri). 

There  may  be  abnormal  union  with  neighboring  structures.  Thus,  the 
uterus  may  be  connected  with  the  bladder  by  a  tube,  or  with  the  rectum, 
either  directly  or  indirectly  (congenital  uterorectal  fistula;  anus  uterinus). 

Several  forms  of  anomalous  development  that  occur  subsequently 
to  birth  should  be  referred  to.  The  uterus  may  never  progress  beyond 
the  stage  to  which  it  attained  during  foetal  life  (uterus  foetalis),  or  it  may 
preserve  its  infantile  characteristics  beyond  the  period  of  puberty 
(uterus  infantilis).  A  peculiar  form  of  hypoplasia  is  that  in  which  the 
form  of  the  uterus  is  normal  but  its  muscular  elements  are  greatly 
lacking.  The  uterine  wall  may  be  not  more  than  0.5  to  1  mm.  thick 
(uterus  membranaceus). 

Precocious  development  of  the  uterus  may  also  occur.  This  condition 
may  be  associated  with  the  early  onset  of  menstruation  and  enlarge- 
ment of  the  breasts,  while  the  rest  of  the  body  remains  infantile  in  type. 

The  uterus  is  occasionally  congenitally  retroflexed,  retroverted,  or 
anteflexed.  In  children  with  spina  bifida  of  the  lumbosacral  region  the 
uterus  had  been  found  prolapsed.  In  some  few  instances  the  uterus 
has  formed  part  of  the  contents  of  a  crural  or  inguinal  hernia  (uterocele; 
hysterocele). 


ACQUIRED  MALPOSITIONS  OF  THE  UTERUS. 

In  children  and  young  women  the  uterus  lies  in  contact  with  the  pos- 
terior wall  of  the  bladder.  In  the  parous  woman,  it  may  occupy  this 
position  or  may  be  inclined  backward,  so  as  to  form  almost  a  right  angle 
with  the  plane  of  the  posterior  wall  of  the  bladder.  The  latter  position 
is,  however,  generally  regarded  as  an  abnormal  one. 

The  uterus,  as  a  whole,  may  be  misplaced  forward  (anteposition), 
backward  (retroposition),  to  the  side  (lateroposition),  upward  (elevation), 
downward  (prolapsus).  It  may  be  turned  inside  out  (inversion),  or  may 
form  part  of  the  contents  of  a  hernial  sac  (hysterocele).  The  chief  causes 
of  these  unusual  positions  are  pressure,  as  from  tumors,  hemorrhage, 
and  exudation;  weight,  as  from  uterine  growths;  and  tension,  from  the 
contraction  of  ligaments  or  inflammatory  bands. 

Anteposition  is  usually  due  to  the  action  of  tumors  in  the  posterior 
wall,  collections  of  fluid  or  misplaced  organs  behind  the  uterus.  Less 
commonly,  it  arises  from  the  contraction  of  inflammatory  adhesions 
between  the  uterus  and  the  bladder  or  abdominal  wall. 

Retroposition  is  most  frequently  the  result  of  contracting  bands  of 


ACQUIRED  MALPOSITIONS  823 

adhesion  traversing  the  Douglas'  pouch,  less  frequently  it  is  caused  by 
tumors  of  the  anterior  wall  or  of  the  bladder,  or  a  distended  bladder. 

Lateroposition  is  due  to  tumors  or  cysts  in  the  broad  ligament,  to  exu- 
dates  in  the  parametrium  pushing  the  organ  to  one  side,  or  to  the  traction 
of  inflammatory  adhesions. 

Another  important  class  of  malpositions  is  that  in  which  there  is  an 
alteration  in  the  direction  of  the  axes  of  the  uterus.  The  organ  may  be 
rotated  in  its  transverse  axis  (version);  forward  (anteversion) ;  backward 
(retro version).  Rotation  on  the  anteroposterior  axis  leads  to  latero- 
version;  on  its  long  axis,  to  torsion. 

Retroversion  is  the  most  common  form.  The  degree  of  retroversion 
varies.  The  long  axis  of  the  uterus  may  form  an  angle  of  45°  with  the 
plane  of  the  superior  strait  of  the  pelvis  (first  degree),  or  both  cervix 
and  fundus  may  lie  in  the  same  plane  across  the  pelvis  at  an  angle 
of  90°  (second  degree),  or,  again,  the  axis  lies  at  an  angle  of  135°  (third 
degree).  The  dislocation  is  commonly  the  result  of  childbirth  or  abor- 
tion, and  is  due  to  increase  in  the  weight  of  the  uterus,  disturbance  of  its 
normal  balance,  or  relaxation  of  its  supports.  Potent  causes  are  sub- 
involution,  relaxation  of  the  round  ligaments,  laceration  of  the  cervix 
and  perineum,  and  tumors  in  the  anterior  wrall  of  the  uterus.  The  con- 
traction of  inflammatory  bands  in  Douglas'  sac  may  also  bring  it  about. 
On  account  of  the  impairment  of  the  circulation  induced  thereby,  the 
uterus  is  congested,  enlarged,  and  cedematous,  and  metritis  and  endo- 
metritis  are  frequently  set  up. 

In  the  etiology  of  anteversion,  pregnancy  is  not  of  such  great  impor- 
tance, although  pregnancy  and  subinvolution  may  play  a  part  if  the 
abdominal  walls  be  lax.  More  important  are  inflammatory  changes  in 
the  body  of  the  uterus  or  in  the  neighboring  connective  tissue. 

Lateral  version  is  usually  combined  with  torsion.  It  may  be  con- 
genital, but  is  usually  due  to  adhesions  or  tumors  of  the  ovary. 

Inversion  of  the  uterus  generally  occurs  in  the  puerperal  uterus. 
Three  grades  exist:  (1)  Incomplete,  where  the  invaginated  fundus 
lies  within  the  uterine  cavity;  (2)  complete,  where  the  fundus  lies  in  the 
vagina;  and  (3)  inversion  with  prolapse,  where  the  uterus  and  vagina 
are  turned  completely  inside  out  and  the  uterus  appears  at  the  vulvar 
orifice.  In  this  position  the  puerperal  uterus  may  undergo  involution 
and  become  firm  and  hard.  Often,  however,  it  is  soft,  congested,  and 
mucous  membrane  thickened,  and  there  may  be  ulceration  or  polypoid 
outgrowths.  The  interstitial  tissues  show  inflammatory  hyperplasia, 
the  glands  gradually  atrophy,  at  least  in  the  more  superficial  parts,  while 
they  proliferate  deeper  down.  In  long-standing  cases,  the  epithelium 
of  the  mucosa  is  converted  into  a  horny  layer  of  squamous  cells  resembling 
skin.  As  will  readily  be  understood,  circulatory  disturbances  may  be 
extreme,  so  that  ulceration,  necrosis,  and  gangrene  may  result,  with 
complete  separation  of  the  part. 

Prolapse  is  the  condition  in  which  the  uterus  as  a  whole  occupies  a 
lower  position  in  the  pelvis  than  normal.  Varying  grades  exist.  When 
the  external  os  reaches  no  farther  than  the  floor  of  the  pelvis,  we  speak 


822  THE  UTERUS 

into  the  cavity  we  speak  of  uterus  bilocularis  subseptus;  when  it  reaches 
the  cervix,  uter.  biloc.  unicollis;  in  other  cases  the  septum  is  only  found 
at  the  external  os,  uterus  biforis ;  in  still  others,  the  septum  is  only  present 
in  the  cervix,  uter.  bicollis  unicorporeus. 

Abnormalities  in  the  formation  of  the  cavity  are  common  in  the 
rudimentary  uterus.  The  cavity  may  be  completely  absent  or  there 
may  be  one  or  more  rudimentary  cavities.  It  may  be  narrower  than 
normal  or  obstructed  (stenosis  uteri). 

There  may  be  abnormal  union  with  neighboring  structures.  Thus,  the 
uterus  may  be  connected  with  the  bladder  by  a  tube,  or  with  the  rectum, 
either  directly  or  indirectly  (congenital  uterorectal  fistula;  anus  uterinus). 

Several  forms  of  anomalous  development  that  occur  subsequently 
to  birth  should  be  referred  to.  The  uterus  may  never  progress  beyond 
the  stage  to  which  it  attained  during  foetal  life  (uterus  fo3talis),  or  it  may 
preserve  its  infantile  characteristics  beyond  the  period  of  puberty 
(uterus  infantilis).  A  peculiar  form  of  hypoplasia  is  that  in  which  the 
form  of  the  uterus  is  normal  but  its  muscular  elements  are  greatly 
lacking.  The  uterine  wall  may  be  not  more  than  0.5  to  1  mm.  thick 
(uterus  membranaceus). 

Precocious  development  of  the  uterus  may  also  occur.  This  condition 
may  be  associated  with  the  early  onset  of  menstruation  and  enlarge- 
ment of  the  breasts,  while  the  rest  of  the  body  remains  infantile  in  type. 

The  uterus  is  occasionally  congenitally  retroflexed,  retroverted,  or 
anteflexed.  In  children  with  spina  bifida  of  the  lumbosacral  region  the 
uterus  had  been  found  prolapsed.  In  some  few  instances  the  uterus 
has  formed  part  of  the  contents  of  a  crural  or  inguinal  hernia  (uterocele; 
hysterocele). 


ACQUIRED  MALPOSITIONS  OF  THE  UTERUS. 

In  children  and  young  women  the  uterus  lies  in  contact  with  the  pos- 
terior wall  of  the  bladder.  In  the  parous  woman,  it  may  occupy  this 
position  or  may  be  inclined  backward,  so  as  to  form  almost  a  right  angle 
with  the  plane  of  the  posterior  wall  of  the  bladder.  The  latter  position 
is,  however,  generally  regarded  as  an  abnormal  one. 

The  uterus,  as  a  whole,  may  be  misplaced  forward  (anteposition), 
backward  (retroposition),  to  the  side  (lateroposition),  upward  (elevation), 
downward  (prolapsus).  It  may  be  turned  inside  out  (inversion),  or  may 
form  part  of  the  contents  of  a  hernial  sac  (hysterocele).  The  chief  causes 
of  these  unusual  positions  are  pressure,  as  from  tumors,  hemorrhage, 
and  exudation;  weight,  as  from  uterine  growths;  and  tension,  from  the 
contraction  of  ligaments  or  inflammatory  bands. 

Anteposition  is  usually  due  to  the  action  of  tumors  in  the  posterior 
wall,  collections  of  fluid  or  misplaced  organs  behind  the  uterus.  Less 
commonly,  it  arises  from  the  contraction  of  inflammatory  adhesions 
between  the  uterus  and  the  bladder  or  abdominal  wall. 

Retroposition  is  most  frequently  the  result  of  contracting  bands  of 


ACQUIRED  MALPOSITIONS  823 

adhesion  traversing  the  Douglas'  pouch,  less  frequently  it  is  caused  by 
tumors  of  the  anterior  wall  or  of  the  bladder,  or  a  distended  bladder. 

Lateroposition  is  due  to  tumors  or  cysts  in  the  broad  ligament,  to  exu- 
dates  in  the  parametrium  pushing  the  organ  to  one  side,  or  to  the  traction 
of  inflammatory  adhesions. 

Another  important  class  of  malpositions  is  that  in  which  there  is  an 
alteration  in  the  direction  of  the  axes  of  the  uterus.  The  organ  may  be 
rotated  in  its  transverse  axis  (version);  forward  (anteversion) ;  backward 
(retroversion).  Rotation  on  the  anteroposterior  axis  leads  to  latero- 
version;  on  its  long  axis,  to  torsion. 

Retroversion  is  the  most  common  form.  The  degree  of  retroversion 
varies.  The  long  axis  of  the  uterus  may  form  an  angle  of  45°  with  the 
plane  of  the  superior  strait  of  the  pelvis  (first  degree);  or  both  cervix 
and  fundus  may  lie  in  the  same  plane  across  the  pelvis  at  an  angle 
of  90°  (second  degree),  or,  again,  the  axis  lies  at  an  angle  of  135°  (third 
degree).  The  dislocation  is  commonly  the  result  of  childbirth  or  abor- 
tion, and  is  due  to  increase  in  the  weight  of  the  uterus,  disturbance  of  its 
normal  balance,  or  relaxation  of  its  supports.  Potent  causes  are  sub- 
involution,  relaxation  of  the  round  ligaments,  laceration  of  the  cervix 
and  perineum,  and  tumors  in  the  anterior  wall  of  the  uterus.  The  con- 
traction of  inflammatory  bands  in  Douglas'  sac  may  also  bring  it  about. 
On  account  of  the  impairment  of  the  circulation  induced  thereby,  the 
uterus  is  congested,  enlarged,  and  oedematous,  and  metritis  and  endo- 
metritis  are  frequently  set  up. 

In  the  etiology  of  anteversion,  pregnancy  is  not  of  such  great  impor- 
tance, although  pregnancy  and  subinvolution  may  play  a  part  if  the 
abdominal  walls  be  lax.  More  important  are  inflammatory  changes  in 
the  body  of  the  uterus  or  in  the  neighboring  connective  tissue. 

Lateral  version  is  usually  combined  with  torsion.  It  may  be  con- 
genital, but  is  usually  due  to  adhesions  or  tumors  of  the  ovary. 

Inversion  of  the  uterus  generally  occurs  in  the  puerperal  uterus. 
Three  grades  exist:  (1)  Incomplete,  where  the  invaginated  fundus 
lies  within  the  uterine  cavity;  (2)  complete,  where  the  fundus  lies  in  the 
vagina;  and  (3)  inversion  with  prolapse,  where  the  uterus  and  vagina 
are  turned  completely  inside  out  and  the  uterus  appears  at  the  vulvar 
orifice.  In  this  position  the  puerperal  uterus  may  undergo  involution 
and  become  firm  and  hard.  Often,  however,  it  is  soft,  congested,  and 
mucous  membrane  thickened,  and  there  may  be  ulceration  or  polypoid 
outgrowths.  The  interstitial  tissues  show  inflammatory  hyperplasia, 
the  glands  gradually  atrophy,  at  least  in  the  more  superficial  parts,  while 
they  proliferate  deeper  down.  In  long-standing  cases,  the  epithelium 
of  the  mucosa  is  converted  into  a  horny  layer  of  squamous  cells  resembling 
skin.  As  will  readily  be  understood,  circulatory  disturbances  may  be 
extreme,  so  that  ulceration,  necrosis,  and  gangrene  may  result,  with 
complete  separation  of  the  part. 

Prolapse  is  the  condition  in  which  the  uterus  as  a  whole  occupies  a 
lower  position  in  the  pelvis  than  normal.  Varying  grades  exist.  When 
the  external  os  reaches  no  farther  than  the  floor  of  the  pelvis,  we  speak 


824 


THE  UTERUS 


of  descent;  when  it  protrudes  through  the  vulvar  opening,  it  is  incomplete 
prolapse;  when  the  uterus  is  entirely  outside  the  body,  it  is  complete 
prolapse.  Some  writers  consider  as  prolapse  a  condition  in  which 
the  corpus  remains  in  its  normal  site  but  the  cervix  is  elongated.  It 
is  more  correct,  however,  to  term  this  hypertrophy  of  the  cervix.  It 
may  exist  both  with  and  without  true  prolapse  or  descent. 

FIG.  218 


I 


Complete  prolapse  of  the  uterus.     From  the  Gynecological  Clinic  of  the  Montreal 
General  Hospital.) 

The  causes  ofprolapse  are  numerous  and  usually  several  are  operative 
at  the  same  time.  The  chief  are,  retroversion  and  retroflexion,  with  the 
conditions  leading  to  them;  lack  of  tone  of  or  injury  to  the  pelvic  floor; 
relaxation  of  the  uterine  ligaments  from  frequent  childbearing;  increased 
weight  of  the  uterus;  weakness  of  the  abdominal  wall.  Rarely,  no  obvi- 
ous cause  can  be  made  out,  as  in  a  case  occurring  in  a  virgin,  aged  sixteen 
years,  recorded  by  Duncan.1 

In  prolapse,  the  circulation  is  markedly  interfered  with,  leading  to 
congestion  and  oedema  of  the  organ  and  hyperplasia  both  of  the  muscle 
and  the  endometrium,  together  with  chronic  metritis  and  endometritis. 
There  may  be  ectropion  of  the  mucosa,  with  erosion.  As  a  rule,  the 
bladder  or  rectum  or  both  accompany  the  uterus  in  its  descent  (cystocele, 
rectocele). 

Elevation  of  the  uterus,  apart  from  the  general  increase  in  size  due  to 
the  presence  of  a  foetus  or  the  accumulation  of  fluid  in  the  cavity  (hydro- 

j    *  Brit.  Med.  Jour.,  1 : 1899 : 404. 


FLEXION  825 

metra,  hematometra),  may  be  caused  by  tumors  growing  in  the  cavity 
or  vagina,  extravasations  of  blood  in  the  Douglas'  pouch,  or  by  tumors 
in  the  uterine  wall,  ovaries,  and  ligaments.  The  puerperal  uterus  may 
also  become  attached  to  the  abdominal  wall,  so  that  when  parturition 
takes  place  involution  cannot  take  place  normally  and  the  uterus  re- 
mains permanently  in  a  high  position.  The  uterus  is  also  attached  high 
up  in  the  operation  of  ventral  fixation.  The  condition  may  lead  to  elonga- 
tion of  the  organ,  atresia  of  the  cavity,  and  atrophy  of  the  cervix. 

One  of  the  rarest  anomalies  of  position  is  hysterocele.  Not  only  may 
the  quiescent  but  also  the  pregnant  uterus  be  involved.  As  a  rule,  the 
tubes  and  ovaries  are  first  engaged  in  the  hernia!  sac  and  the  uterus  follows 
owing  to  the  traction.  The  uterus  has  been  found  in  inguinal,  crural,  and 
ventral  hernias. 

When  the  body  of  the  uterus  is  bent  upon  the  cervical  portion  we 
speak  of  flexion.  Anteflexion,  retroflexion,  and  lateral  flexion  are  described. 

Retroflexion  is  the  commonest,  and  is  often  associated  with  retro- 
version.  In  severe  cases  the  fundus  is  found  in  the  hollow  of  the  sacrum. 
When  pregnancy  occurs  in  such  a  uterus  the  organ  may  be  incarcerated 
in  its  false  position.  Retroflexion  has  been  known  to  occur  in  nullipara 
and  newborn  children,  but  pregnancy  is  the  most  important  predisposing 
cause.  Relaxation  of  the  ligaments,  anterior  fixation  of  the  cervix, 
tumors,  inflammatory  adhesions  are  also  of  importance.  Retroflexion 
is  frequently  combined  with  total  prolapse. 

Anteflexion  is  present  when  the  uterus  does  not  assume  its  ordinary 
elevated  position  during  the  filling  of  the  bladder  and  remains  in  part 
of  its  length  tilted  forward,  or  when  the  angle  between  the  axes  of 
corpus  and  cervix  is  135°  or  less.  When  the  angle  is  from  135°  to  90° 
it  constitutes  the  first  degree  of  anteflexion;  when  from  90°  to  45°,  the 
second  degree;  45°  or  less,  the  third  degree.  The  chief  causes  are 
traction  on  the  cervix  by  adhesions  in  the  Douglas'  pouch  in  the  neigh- 
borhood of  the  os,  or  by  bands  between  the  fundus  and  the  bladder. 
In  the  infantile  form,  shortness  of  the  anterior  vaginal  wall  is  of  chief 
importance.  In  adults  the  weight  of  the  abdominal  contents,  where 
the  abdominal  wall  is  relaxed,  plays  the  main  role. 


ABNORMALITIES  OF  THE  UTERINE  CAVITY  AND  OF 
ITS  CONTINUITY. 

Stenosis. — Narrowing  of  the  uterine  cavity  may  be  congenital  or 
acquired.  Partial  narrowing  (stenosis)  usually  occurs  at  either  the  ex- 
ternal or  internal  os,  rarely  at  both.  Stenosis  of  the  internal  os  is  common 
in  elderly  people.  Complete  obliteration,  atresia,  also  occurs.  The 
chief  causes  in  the  acquired  forms  are  mucus  or  tumors  blocking  the 
cavity;  inflammation  and  oedema  of  the  endometrium;  flexions  or  elonga- 
tion of  the  uterus;  and  traumatism,  such  as  arises  from  cauterization  or 
curetting. 

Dilatation. — Dilatation  of  the  cavity  is  produced  by  intra-uterine 
growths  or  collections  of  fluid.  In  young  and  vigorous  individuals  the 


826  THE  UTERUS 

muscular  wall  hypertrophies  as  well,  but  in  elderly  persons,  in  whom  the 
uterus  lacks  tone,  dilatation  is  often  unaccompanied  by  hypertrophy. 

According  to  the  nature  of  the  contained  substances,  we  speak  of 
hydrometra,  hematometra,  pyometra,  lochiometra,  and  physometra. 

Hematometra  is  the  condition  in  which  menstrual  blood  collects 
within  the  uterus  owing  to  congenital  or  acquired  atresia  of  the  genital 
canal.  When  the  occlusion  is  in  the  vagina,  hematometra  is  combined 
with  hematocolpos.  In  advanced  cases  the  Fallopian  tubes  are  dilated 
as  well  (hematosalpinx).  Perhaps  the  commonest  cause  is  imperf orate 
hymen.  In  these  instances  the  uterus  may  attain  the  size  of  the  head. 
Rupture  rarely  takes  place.  The  contents  are  thick,  brownish-black 
blood,  with  cholesterin. 

Hydrometra  comes  on  after  the  menopause.  Atresia  is  again  the 
cause,  or  sometimes  a  tumor  obstructing  the  cervical  canal.  The  amount 
of  fluid  is  rarely  large.  In  appearance  it  is  clear  and  colorless,  grayish 
and  cloudy,  or  possibly  mixed  with  blood  or  mucus.  The  mucous 
membrane  becomes  atrophied  from  pressure  and  the  epithelial  cells  are 
flattened.  Pyometra  is  usually  due  to  the  suppuration  of  a  tumor  in 
the  cervical  canal. 

Physometra,  or  gas  in  the  uterine  cavity,  occurs  most  frequently  in 
the  puerperal  uterus  from  the  decomposition  of  blood,  membranes, 
or  placental  remains.  It  may  also  be  due  to  the  decomposition  of  a 
malignant  growth.  Anaerobic  bacilli  of  the  malignant  oedema  class, 
B.  Welchii,  etc.,  may  occasionally  lead  to  the  condition.1 

Diverticula. — Diverticula  may  form  in  the  uterine  wall  owing  to 
irregular  involution  (Klebs2),  or  to  the  scarring  of  the  wall  following 
parturition  or  Cesarean  section. 


WOUNDS  AND  OTHER  INJURIES. 

Rupture. — Rupture  of  the  uterus  almost  invariably  occurs  in  connec- 
tion with  the  pregnant  or  parturient  state.  The  tearing  of  the  cervix 
is  one  of  the  commonest  events  during  delivery.  All  grades  exist,  from 
a  slight  fissure  of  the  mucosa  to  a  rent  that  extends  into  the  muscle  or 
even  into  the  abdominal  cavity.  The  laceration  is  unilateral,  bilateral, 
or  stellate,  and  usually  runs  in  the  long  axis  of  the  uterus.  In  rare  cases 
the  cervix  is  torn  in  its  transverse  axis.  One  or  other  lip,  or  even  the 
whole  cervix,  may  be  torn  off.  Rupture  may  also  take  place  in  the  later 
months  of  pregnancy,  in  cases  where  the  uterus  is  abnormally  soft  from 
inflammation,  thin-walled,  or  scarred  from  previous  operations.  It  has 
been  met  with  where  pregnancy  has  occurred  in  a  rudimentary  horn  or 
in  an  incarcerated  uterus,  or,  again,  where  there  has  been  obstruction 
to  the  progress  of  labor. 

Bruising,  laceration,  and  perforation  of  the  uterus  sometimes  occur 
during  operative  measures  (curetting)  and  in  attempts  at  criminal 

1  See  Lindenthal,  Monatsschr.  f.  Geb.  u.  Gyn.,  7:  1898:  269. 
2Partielle  Erweiterung.  Handb.,  1:2:  1876:  900. 


METRORRHAG1A  §27 

abortion.  As  a  rule,  the  rupture  is  at  or  near  the  fundus.  Besides 
this,  perforation  may  be  due  to  ulceration,  pressure  necrosis,  and 
tumors.  Fistulous  communications  may  be  opened  up  with  the  vagina 
(cervicovaginal  fistula),  the  bladder  (uterovesical  fistula),  or  the  rectum 
(rectovaginal  fistula). 


CIRCULATORY  DISTURBANCES. 

Anemia. — Anemia  of  the  uterus  is  found  in  cases  of  generalized 
anemia,  and,  according  to  Rokitansky,  in  hypoplasia  of  the  organ. 

Hyperemia. — Hyperemia  is  found  physiologically  during  menstrua- 
tion, pregnancy,  and  for  some  time  after  full-term  delivery  and  abortion. 
It  is  said  to  be  caused  also  by  sexual  excitement. 

Pathological  active  hyperemia  is  found  in  many  of  the  infective  fevers, 
as  typhoid,  influenza,  and  the  exanthemata.  As  a  rule,  the  mucosa  of 
the  corpus  is  affected,  being  reddened  and  swollen.  Collateral  hyperemia 
may  occur  when  one  part  of  the  uterus  is  compressed  from  any  cause. 

Passive  hyperemia  is  very  common.  It  occurs  in  systemic  venous 
stasis  or  from  local  causes,  such  as  pressure  of  the  distended  rectum  or 
bladder,  prolapse,  anteversion,  retroversion,  and  inversion.  Hyperplasia 
is  not  infrequently  combined  as  well. 

Hemorrhage. — Hemorrhage  is  one  of  the  commonest  occurrences  in 
the  uterus.  This  is  not  surprising  when  we  bear  in  mind  the  physio- 
logical tendencies  in  this  direction.  The  extravasation  of  blood  takes 
place  into  the  uterine  cavity  or  into  the  endometrium.  When  retained 
within  the  uterus  hematometra  results.  Hemorrhage  occurs  most  fre- 
quently during  or  subsequent  to  parturition.  It  may  also  be  due  to  trau- 
matism,  ulcerating  new-growths,  or  to  retrograde  changes  in  advanced 
life. 

Metrorrhagia. — Metrorrhagia  is  hemorrhage  from  the  uterus  at  times 
other  than  those  of  the  usual  menstrual  discharge.  Apart  from  preg- 
nancy and  the  parturient  state,  it  occurs  in  hemophilia,  scurvy,  the 
hemorrhagic  diatheses,  typhoid,  sepsis,  the  acute  exanthemata,  acute 
yellow  atrophy  of  the  liver,  and  phosphorus  poisoning.  Local  disease 
of  the  uterus,  such  as  endometritis,  myomas,  ulcerating  cancers,  also 
accounts  for  many  cases.  After  abortion,  and  even  full-term  delivery, 
small  portions  of  placenta  may  be  retained  and  lead  to  hemorrhage. 
In  some  cases  the  blood  clots  upon  the  adherent  tissue  and  forms  a  fibrin- 
ous  polypoid  mass  (hematoma  polyposum,  Virchow).  In  extra-uterine 
gestation  a  special  form  of  metrorrhagia  with  exfoliation  of  the  decidua 
may  occur.  Hemorrhage  into  the  mucosa  may  lead  to  the  formation 
of  large  coagula  (hematomata).  A  special  form  is  the  so-called  apoplexy 
(Cruveilhier)  of  the  uterus  that  occurs  in  old  women.  The  uterus  is 
atrophic  and  brittle,  the  vessels  stand  out  as  rigid,  tortuous  tubes,  and 
the  mucous  membrane  is  swollen,  friable,  and  infiltrated  with  blood. 
Hemorrhage  may  also  occur  in  the  muscular  wall.  The  cervix  and  the 
portio  vaginalis  escape. 


828  THE  UTERUS 

Menorrhagia. — Menorrhagia  is  an  excessive  menstrual  discharge.  It 
is  due  to  a  variety  of  causes,  among  which  may  be  mentioned  general- 
ized passive  congestion,  endometritis,  tumors  of  the  uterus,  and  polyps. 
In  some  cases,  in  addition  to  menstrual  blood,  shreds  of  membrane 
are  cast  off  (dysmenorrhcea  membranacea).  The  entire  lining  of  the 
uterus  may  be  exfoliated  in  this  way. 

Microscopically,  the  membrane  consists  of  a  cellular  mucosa  with 
infiltrated  connective  tissue  and  remnants  of  glands.  The  epithelium 
may  be  unaltered  or  fattily  degenerated  and  desquamating.  In  other 
cases  the  membrane  consists  simply  of  a  fibrinous  cast  of  the  uterine 
cavity.  A  peculiar  form  of  membrane  is  one  consisting  of  squamous 
cells  and  containing  the  orifices  of  glands.  As  the  cavity  of  the  uterus 
does  not  normally  possess  such  cells,  it  is  supposed  that  in  these  cases 
the  epithelial  lining  of  the  vagina  has  extended  farther  than  usual  into 
the  cervix  and  that  the  membrane  is  derived  from  the  anomalous  cervix. 

(Edema. — This  is  due  to  passive  congestion  and  inflammation. 
The  first  type  is  well  seen  in  cases  of  suddenly  acquired  malposition,  as 
retroversion. 

INFLAMMATIONS. 

Inflammation  may  affect  the  serous  covering  of  the  uterus  (peri- 
metritis),  the  broad  ligaments  (parametritis),  the  myometrium  (metritis), 
or  the  endometrium  (endometritis). 

Perimetritis. — Perimetritis  is  merely  a  local  peritonitis,  and  will  be 
dealt  with  under  that  head. 

Endometritis. — Endometritis  is  due  usually  to  an  extension  of  in- 
flammation from  the  vagina,  but  rarely  is  hematogenic.  It  may  be 
confined  to  the  cervix  (cervical  endometritis)  or  to  the  corpus  (corporeal 
endometritis),  or  may  involve  the  whole  of  the  lining  membrane.  The 
affection  arises  most  frequently  during  menstruation,  and  the  puerperium. 
According  to  the  mode  of  development  we  can  recognize  acute  and 
chronic  forms,  or  according  to  the  morbid  changes,  exudative  and  pro- 
ductive. 

Acute  Catarrhal  Endometritis. — In  acute  catarrhal  endometritis  the 
mucous  membrane  is  reddened,  swollen,  and  infiltrated  with  inflam- 
matory products.  The  secretion  from  the  cervix,  which  normally  is 
scanty,  viscid,  and  mucoid,  becomes  more  abundant,  more  mucoid,  or 
mucopurulent.  That  from  the  corpus  is  thinner,  serous,  or  seropurulent. 
A  purulent  exudate  (fluor  albus;  leucorrhoea)  is  more  common  in 
cervical  endometritis  than  in  corporeal.  In  very  severe  cases  the  dis- 
charge may  be  mixed  with  blood.  This  is  particularly  the  case  in 
the  form  arising  during  the  infective  fevers,  such  as  typhoid,  cholera, 
and  the  exanthemata.  Here  we  may  perhaps  speak  of  a  hemorrhagic 
endometritis.  This  usually  affects  the  corpus.  Should  the  cervical 
canal  become  obstructed,  the  pus  accumulates  within  the  uterine  cavity 
(pyometra).  In  some  cases  the  retained  material  becomes  inspissated, 
forming  a  granular,  pulpy  detritus  resembling  caseous  matter.  The 


ENDOMETRITIS  829 

disease  is  usually  traceable  to  irritation  and  infection  of  the  endometrium, 
the  extension  of  inflammation  from  the  myometrium  or  uterine  adnexa, 
gonorrhoea,  constitutional  diseases,  disorders  of  the  circulation  in  the 
uterus,  or  the  presence  of  neoplasms. 

Microscopically,  changes  are  found  both  in  the  glandular  structures 
and  in  the  stroma.  The  mucous  membrane  is  infiltrated  with  inflam- 
matory products,  and  the  cells  of  the  interstitial  substance  are  more  or 
less  dissociated.  The  bloodvessels  and  lymphatics  are  dilated  and  there 
may  be  minute  hemorrhages.  The  epithelial  cells  are  swollen,  granular, 
and  desquamating,  while  the  ducts  may  be  blocked  with  secretion.  In 
some  cases  the  most  marked  changes  occur  in  the  stroma  (interstitial 
endometritis),  which  is  cedematous  and  infiltrated  with  round  cells,  so 
that  the  gland-tubules  are  dislocated.  The  epithelium  here  also  shows 
degenerative  changes. 

Membranous  Endometritis. — Another  form  of  acute  endometritis  is 
the  membranous.  It  occurs  by  far  the  most  frequently  in  the  puerperal 
uterus,  but  is  occasionally  met  with  in  the  infectious  diseases,  such  as 
typhoid,  cholera,  and  the  exanthemata.  True  diphtheria  of  the  endo- 
metrium has  been  observed,  secondary  to  infection  of  the  vagina.  Mem- 
branous endometritis  may  also  be  found  associated  with  ulcerating 
cancers  or  other  tumors  of  the  uterus.  The  process  resembles  that 
observed  in  other  mucous  membranes.  A  fibrinous  exudate  is  thrown 
out  which  coagulates  upon  the  surface  and  forms  a  membrane  that  may 
be  exfoliated.  Microscopically,  this  consists  of  interlacing  threads  of 
fibrin,  including  leukocytes,  showing  hyaline  degeneration. 

Chronic  Endometritis. — Chronic  endometritis  assumes  the  guise  of  a 
catarrhal  inflammation,  inasmuch  as  it  is  accompanied  by  an  abundant 
excretion,  but  the  most  important  feature  is  the  proliferation  of  tissue 
(productive  endometritis).  Chronic  productive  or  proliferating  endo- 
metritis, as  it  affects  the  corpus  uteri,  takes  the  form  of  a  new-growth 
of  tissue  (endom.  hyperplastica),  which  later  on  gives  rise  to  a  form  of 
atrophy  of  the  mucosa  (endom.  atrophied).  In  the  earlier  stages  the 
mucous  membrane  is  thickened,  its  surface  smooth  or  irregular,  warty 
or  villous  (fungous  endometritis).  All  grades  exist  from  simple  nodular 
elevations  to  polypoid  or  pedunculated  outgrowths  (endom.  polyposa). 
The  mucosa  is  also  reddened  and  may  show  hemorrhages.  At  the 
menstrual  period  the  lining  of  the  uterus  may  be  exfoliated  in  shreds  or 
as  a  perfect  cast  of  the  cavity  (dysmenorrhcea  membranacea;  endom. 
exfoliativa).  The  tissue  is  soft,  loose,  and  friable,  often  porous. 

Microscopically,  in  ordinary  proliferating  endometritis,  the  gland- 
tubules  are  enlarged,  often  lengthened  and  tortuous,  and  present 
numerous  irregular  dilatations  even  to  the  extent  of  cyst-formation. 
The  overgrowth  may  be  so  great  that  papillary  masses  project  from 
the  surface.  The  epithelial  cells  have  in  great  part  lost  their  cilia, 
and  are  clear,  swollen,  and  mucoid.  Active  mitosis  is  also  going  on. 
The  lumen  of  the  ducts  is  filled  with  mucus,  desquamated  epithelium, 
and  leukocytes.  The  interstitial  stroma  is  infiltrated  with  leukocytes, 
and  shows  proliferation  both  of  the  cellular  elements  and  fibrous  tissue. 


830  THE  UTERUS 

This  may  lead  to  increased  vascularity  of  the  membrane  with  permanent 
induration  and  contraction.  The  enlarged  tubules  commonly  penetrate 
between  the  muscle  bundles  of  the  uterine  wall  (glandular  heterotopia, 
Cornil).  This  must  not  be  mistaken  for  malignancy,  for  it  should  be 
remembered  that  the  uterus  possesses  no  submucosa,  and  the  glands 
normally  abut  upon  and  occasionally  penetrate  the  muscular  layers.  It 
is,  however,  doubtless  true  that  such  proliferation  forms  a  ready  starting 
point  for  malignant  transformation.  As  distinguished  from  the  above 
type,  which  is  also  termed  chronic  glandular  endometritis,  we  have  to 
recognize  an  interstitial  form  in  which  the  morbid  changes  predominate 
in  the  interstitial  substance. 

In  the  later  stages  of  the  disease  a  form  of  atrophy  may  set  in  not 
unlike  senile  involution.  The  mucosa  becomes  smooth  and  thin,  often 
pigmented,  and  is  firmer  and  more  fibrous  than  normal.  Not  infre- 
quently, it  contains  cysts  about  the  size  of  a  pin-head,  containing  a  clear 
or  slightly  turbid  fluid  (endom.  chronica  cystica).  Microscopically,  there 
is  proliferation  of  the  stroma  with  the  production  of  dense  fibrous  or 
scar  tissue.  This  leads  to  atrophy  of  the  gland-tubules  with  contraction 
of  certain  sections  of  them,  so  that  they  become  dilated. 

As  a  result  of  the  irritation,  or  perhaps  to  some  extent  from  pressure, 
the  cylindrical  cells  of  the  mucosa  may  become  converted  into  squamous 
cells  '(ichthyosis  or  psoriasis  uteri).  This  has  an  important  bearing  in 
view  of  the  fact  that  squamous-celled  carcinoma  may  occur  in  the  corpus. 
It  should  be  remarked  in  this  connection,  however,  that  while  endome- 
tritis plays  a  most  important  part  in  this  metaplasia,  it  is  possibly  not 
the  only  factor,  for  islets  of  squamous  cells  have  been  found  in  the  decidua 
(Gottschalk  and  Winckler;  Opitz  and  Gebbard),  and  in  the  uteri  of 
foetuses  and  infants  (Meier  and  Friedlander). 

The  causes  of  chronic  endometritis  vary  considerably.  An  important 
role  is  played  by  local  disturbances  of  the  circulation,  such  as  are  brought 
about  by  retroflexion  and  retroversion.  Intra-uterine  tumors  and  dis- 
ease of  the  ovaries  may  lead  to  irritation  of  the  endometrium.  Endo- 
metritis may  also  follow  the  puerperium  when  portions  of  the  products 
of  conception  are  retained  or  when  infection  has  taken  place.  Gonor- 
rhoea is  also  another  important  factor. 

Chronic  Cervical  Endometritis. — Chronic  cervical  endometritis  is  almost 
invariably  accompanied  by  vaginitis.  The  mucosa  is  reddened  and 
swollen,  and  polypoid  outgrowths  are  not  uncommon.  It  is  not  un- 
common for  small  cysts  to  be  produced,  the  so-called  ovula  Nabothi, 
which  vary  in  size  from  that  of  a  hemp-seed  to  a  pea.  They  may 
project  into  the  lumen  of  the  cervix  or  may  be  concealed  in  the 
deeper  layers.  The  contents  are  usually  a  clear  viscid  mucus,  or  may  be 
cloudy  from  the  presence  of  degenerating  cells.  The  cysts  may  become 
infected  and  produce  follicular  abscesses.  They  are  of  the  nature  of 
retention  cysts. 

Microscopically,  there  is  inflammatory  infiltration  with  some  enlarge- 
ment of  the  glands.  The  epithelial  cells  are  in  many  cases  converted 
into  goblet  cells. 


METRITIS  831 

As  a  rule,  there  is  an  abundant  mucoid  or  mucopurulent  secretion 
(leucorrhoea).  In  women  who  have  borne  children,  and  in  whom  the 
external  os  is  therefore  large  and  fissured,  the  reddened  and  swollen 
membrane  may  be  everted  (ectropion).  As  a  result  of  this  ectropion 
erosions  are  frequently  found  in  the  portio  vaginalis,  which  present  as 
red,  glistening  patches  that  are  moist  and  bleed  at  the  slightest  touch. 
The  surface  may  be  smooth  and  velvety  (simple  erosion),  papillomatous 
(papillary  erosioii),  or  may  present  numerous  cysts  (cystic  erosion). 
From  suppuration  and  rupture  of  the  cysts,  follicular  ulcers  are  pro- 
duced. 

Microscopically,  there  is  superficial  loss  of  substance,  with  congestion 
and  the  formation  of  granulation  tissue.  In  old  erosions  one  sees  fatty 
degeneration  or  even  calcification.  A  point  of  some  importance  in  con- 
nection with  the  etiology  and  forms  of  carcinoma  is  the  replacement 
of  the  eroded  squamous  epithelium  of  the  portio  vaginalis  by  a  layer 
of  cylindrical  cells  as  well  as  the  formation  of  glands  similar  to  those  of 
the  cervical  cavity. 

Chronic  cervical  catarrh  frequently  gives  rise  to  hyperplasia  of  the 
muscular  structures,  and  occasionally  to  adhesion  or  obstruction  of  the 
canal  near  the  internal  os. 

The  most  important  causes  of  the  condition  are,  traumatism  during 
childbirth,  and  vaginitis  extending  to  the  cervix.  Gonorrhoea  is  the 
most  frequent  infective  cause.  The  erosion  is  brought  about  not  only 
from  the  irritation  of  the  inflammatory  agents  but  also  by  the  macerating 
action  of  the  secretions  that  escape  from  the  canal. 

Metritis. — Inflammation  of  the  muscular  wall  of  the  uterus  is  called 
metritis. 

Acute  Metritis. — Apart  from  the  puerperium,  acute  metritis  is  rare  and 
due  generally  to  trauma  or  to  hematogenic  infection.  The  uterus  is 
congested  and  cedematous,  soft  and  doughy,  while  on  section  small 
hemorrhages  may  be  observed  in  its  substance  and  beneath  the  serosa. 

Microscopically,  one  sees  inflammatory  leukocytes  about  the  vessels, 
the  interstitial  tissue  is  cedematous,  and  the  muscle  fibers  swollen  and 
cloudy.  In  rare  instances,  abscesses  are  formed  that  may  attain  a 
large  size.  In  such  cases  the  pus  may  be  discharged  into  the  uterine  or 
peritoneal  cavities,  vagina,  rectum,  bladder,  intestine,  or  even  externally. 
Septic  peritonitis  can  be  set  up  by  the  extension  of  a  metritis. 

Chronic  Metritis. — Chronic  metritis  is  usually  attributable  to  chronic 
endometritis,  traumatic  insults,  and  subinvolution.  Potent  predisposing 
causes  are  repeated  or  chronic  congestion,  such  as  is  brought  about  by 
dislocations.  The  disease  occasionally  follows  the  acute  form.  The 
uterus  is  more  or  less  elongated,  and  the  anteroposterior  transverse 
diameter  is  increased.  Both  the  overlying  peritoneum  and  the  endo- 
metrium  are  thickened.  In  the  earlier  stages  the  uterus  is  soft,  congested, 
but  later  becomes  firm  and  indurated.  On  section,  in  long-standing 
cases,  the  tissue  is  grayish  in  color,  tough  and  fibroid. 

Microscopically,  there  is  an  accumulation  of  leukocytes  about  the 
vessels,  although  this  is  not  a  marked  feature.  The  interstitial  connec- 


832  THE  UTERUS 

tive  tissue  is  increased.  Numerous  "Mast-zellen"  may  be  seen  in  the 
interstices  of  the  stroma.  The  condition  of  the  muscle  varies  according 
to  circumstances.  It  may  be  normal,  or  hypertrophied,  where  excessive 
uterine  contraction  has  taken  place,  or,  again,  may  be  atrophic. 

Tuberculosis. — Genital  tuberculosis  is  rarer  in  women  than  in  men. 
Tuberculosis  of  the  uterus  is  almost  invariably  a  descending  infection 
originating  in  the  Fallopian  tubes.  Not  infrequently,  the  lungs,  kidneys, 
and  peritoneum  are  involved  at  the  same  time.  Hematogenic  infection 
is  met  with  in  the  disseminated  miliary  form  of  tuberculosis.  It  is 
questionable  whether  primary  tuberculosis  ever  takes  place  under 
ordinary  circumstances.  The  affection  has  been  found  at  all  ages, 
from  infancy  to  old  age,  but  is  most  common  during  the  period  of 
greatest  vitality. 

The  body  of  the  uterus  is  the  site  of  election.  The  disease  begins  at 
the  orifices  of  the  tubes  and  thence  spreads  throughout  the  endometrium, 
generally  stopping  abruptly  at  the  internal  os.  It  may,  however, 
extend  to  the  cervix  and  even  to  the  vagina. 

Several  forms  may  be  differentiated:  (1)  Acute  miliary  tubercu- 
losis; (2)  chronic  local  tuberculosis;  and  (3)  diffuse  fibroid  tuberculosis. 
In  the  earlier  stages  the  lesions  produced  are  not  unlike  those  of  chronic 
productive  endometritis.  The  mucosa  is  soft,  swollen,  and  reddened, 
often  nodular.  Microscopically,  the  resemblance  to  productive  endo- 
metritis is  also  close,  with  the  addition,  however,  of  giant  cells  in  the 
neighborhood  of  the  areas  of  cellular  infiltration. 

Sooner  or  later  caseation  sets  in  and  the  tubercles  are  recognizable  as 
grayish  elevations.  The  epithelium  of  the  glands  shows  evidence  of 
cloudiness  and  degeneration,  and  the  tubules  tend  to  disappear  in  the 
course  of  the  formation  of  what  amounts  to  tuberculous  granulation 
tissue.  Caseation  rarely  remains  local,  and  the  rule  is  for  neighboring 
tubercles  to  coalesce  until  the  whole  endometrium  is  converted  into  a 
caseous  mass.  The  surface  of  the  uterine  wall  becomes  uneven,  nodular 
and  eroded,  and  the  uterine  cavity  is  more  or  less  completely  filled  with 
a  caseopurulent  detritus.  The  walls  of  the  uterus  are  often  distended. 
The  destructive  process  gradually  extends  into  the  muscularis,  and 
small,  caseous  foci  may  be  found  along  the  margin  of  the  destroyed 
zone,  or  simply  areas  of  cellular  infiltration  with  giant  cells. 

Tuberculosis  of  the  neck  of  the  womb  as  a  primary  disease  is  exces- 
sively rare.  The  cervix  may  be  enlarged  and  the  lesions  produced  are 
similar  to  those  in  the  corpus. 

Syphilis. — The  indurated  primary  sore  may  be  found  on  the  portio 
vaginalis  and  within  the  cervical  canal.  The  cervix  may  be  swollen 
and  hypertrophied,  and  there  is  frequently  a  complicating  endometritis. 
The  ulcer  does  not  differ  materially  from  those  found  on  other  mucous 
surfaces.  Secondary  lesions  are  also  met  with. 

Parasites  and  Foreign  Bodies. — Bacteria  and  yeasts  of  various 
kinds  are  found  in  the  cervical  secretion.  The  most  important  parasite 
is  the  Echinococcus.  The  cysts  are  usually  submucous,  but  have  been 
found  also  in  other  situations.  The  disease  may  form  a  hindrance  tq 


HYPERTROPHY  833 

childbearing,  as  in  a  case  recorded  by  Birch-Hirschfeld.1    A  calcified 
round-worm  has  been  found  on  the  posterior  wall  of  the  uterus. 

Among  foreign  bodies  may  be  mentioned  tents,  catheters,  needles, 
foetal  remains,  clots,  bits  of  tumors,  and  free  myofibromas. 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Simple  atrophy  of  the  uterus  is  of  common  occurrence, 
and  is  found  more  especially  in  women  past  the  climacterium.  It  may 
also  be  found  in  what  has  been  called  premature  senility,  where,  either 
from  operative  interference  (castration),  tumors,  or  inflammation,  the 
normal  function  of  the  ovaries  is  markedly  inhibited.  The  process 
affects  first  and  chiefly  the  portio  vaginalis,  in  contradistinction  to  what 
occurs  in  congenital  hypoplasia,  where  the  corpus  is  the  part  mainly 
involved.  The  uterus  is  small,  thin-walled,  the  muscle  fibers  wasted, 
so  that  the  connective  tissue  appears  to  be  increased.  The  vessels 
are  atheromatous.  The  mucous  membrane  is  thin,  flattened,  and  in- 
filtrated, while  the  lining  cells  have  in  great  part  lost  their  cilia.  A 
form  of  atrophy  is  also  met  with  in  Addison's  disease  and  exophthalmic 
goitre.  Retained  secretion  leads  to  atrophy  of  the  wall  through  pressure. 
Occasionally  other  local  causes  are  at  work,  as,  for  instance,  the  pressure 
of  a  tumor  within  the  cavity. 


PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Apart  from  that  form  which  occurs  during  the 
puerperal  period,  pathological  hypertrophy  affects  the  uterus  either  in 
whole  or  in  part.  In  partial  hypertrophy,  the  corpus,  cervix,  or  the 
endometrium  may  be  involved.  Many  cases  are  associated  with  in- 
flammation, and  it  is  not  always  easy  to  draw  the  line  between  what  is 
inflammatory  and  what  is  not.  Not  only  the  muscle  but  the  connective 
tissue  may  be  affected.  One  important  type  is  hypertrophy  from  over- 
work, found  in  cases  of  retained  secretion  and  tumors  within  the  cavity. 
Muscular  hypertrophy  may  also  be  seen  in  many  cases  of  chronic  endo- 
metritis. 

A  remarkable  form  of  partial  hypertrophy,  said  to  be  due  to  chronic 
inflammation,  leads  to  a  proboscis-like  elongation  of  the  whole  cervix. 
An  exceedingly  common  form  of  hypertrophy  of  the  endometrium  is 
seen  in  the  formation  of  polyps  as  a  result  of  inflammation.  Some  of 
these  are  cystic.  A  form  known  as  follicular  hypertrophy  or  cystic 
glandular  hypertrophy  is  found  at  the  os.  A  special  form  of  hypertrophy 
of  the  endometrium  should  also  be  mentioned,  namely,  the  formation  of 
a  decidua  in  cases  of  extra-uterine  gestation. 

'Lehrbuch,   1887:  789. 
53 


834  THE  UTERUS 

Tumors. — Among  the  benign  tumors  we  have  fibroma,  leiomyoma, 
fibromyoma,  myxoma,  lipoma,  adenoma,  adenomyoma,  and  chondroma. 

By  far  the  most  common  tumor  of  the  uterus  is  the  fibroma  (see  also 
vol.  i,  p.  687).  This  is  almost  invariably  a  mixed  tumor,  containing  a 
variable  quantity  of  muscular  elements  (myofibroma,  uterine  fibroid). 
The  appearance  of  the  growth  varies  according  to  the  amount  of  muscle 
it  contains.  The  purer  myomas  are  usually  submucous  and  start  from 
the  fundus.  They  are  soft,  vascular,  and  of  a  reddish,  flesh-like  appear- 
ance. They  are  apt  to  be  indefinitely  bounded.  The  more  fibrous 
the  tumor  is,  however,  the  firmer  and  paler  it  becomes.  Myofibromas 

FIG.  219 


Submucous  polyp  of  the  uterus.      (From  the  Pathological  Museum  of  McGill  University.) 

are  generally  multiple  and  may  vary  in  size  from  that  of  a  pin-head  to 
that  of  an  adult  man's  head,  or  even  larger.  Most  of  them  originate 
in  the  posterior  wall  of  the  corpus;  next  in  frequency,  in  the  anterior 
wall  and  the  fundus.  From  5  to  8  per  cent,  begin  in  the  cervix. 

In  a  well-marked  example  of  a  myofibroma  the  mass  is  hard,  possibly 
more  or  less  nodular,  and  well-defined.  On  section,  it  is  hard,  grating 
somewhat  under  the  knife,  and  of  a  pale,  grayish  color.  On  closer 
inspection,  the  cut  surface  is  seen  to  be  glistening,  the  substance  being 
formed  of  interlacing  fibrillse,  and  has  a  sheen  like  watered  silk.  Very 
often  nodules  or  whorls  of  fibrous  tissue  can  be  made  out.  In  the  middle 
of  the  smaller  nodules  a  bloodvessel  can  often  be  seen.  In  some  forms 
(teleangiectatic  and  cavernous  myofibromas)  the  vessels  are  abundant  and 
form  large  sinuses. 

Microscopically,  both  muscular  and  fibrous  elements  are  to  be  made 
out,  the  proportion  varying  in  different  cases.  The  connective  tissue 
tends  to  be  grouped  about  the  bloodvessels.  In  many  cases  the  tumor 
consists  of  little  else  but  interlacing  fibrillee  of  connective  tissue,  forming 


UTERINE  FIBROIDS  835 

strands,  whorls,  and  nodules.  Epithelial  remains  and  nerve-fibers  have 
been  demonstrated. 

According  to  the  site  of  the  tumor  we  can  recognize  four  types: 
(1)  the  intramural  or  interstitial;  (2)  the  subserous;  (3)  the  submucous; 
and  (4)  the  intraligamentous. 

Intramural  myofibromas,  on  account  of  their  favorable  position, 
whereby  they  receive  an  abundant  supply  of  blood,  grow  rapidly,  and 
may  attain  a  relatively  large  size.  They  are  often  encapsulated  and 
surrounded  by  a  plexus  of  large  venous  sinuses.  In  other  cases,  they  are 
directly,  though  loosely,  attached  to  the  uterine  musculature  and  may 
form  diffuse  growths.  They  occur  in  simple  nodules  or  aggregations  of 
nodules,  which  are  often  more  or  less  compressed. 


FIG.  220 


• 


Fibromyoma  of  the  uterus.  The  dark  area  to  the  left  is  composed  of  muscle  bundles;  the 
lighter,  to  the  right,  is  dense  fibrous  tissue.  Winckel  obj.  No.  3,  without  ocular.  (From  the 
collection  of  Dr.  A.  G.  Nicholls.) 

Subserous  myofibromas  form  either  sessile  nodules,  appearing  beneath 
the  peritoneal  investment  of  the  uterus,  or  pedunculated  growths. 
Owing  to  torsion  of  the  pedicle,  grave  circulatory  disturbances  are  liable 
to  supervene,  such  as  infarction,  necrosis,  and  gangrene,  unless  the 
tumor  receives  an  adequate  blood  supply  through  the  formation  of 
secondary  adhesions.  Occasionally,  the  mass  becomes  separated  from 
the  uterus  and  forms  a  free  body  in  the  peritoneal  cavity.  When  situated 
low  down  and  posteriorly,  compression  of  the  cervix  and  elongation  of 
the  uterus  may  take  place.  The  tumor  may  also  grow  out  between  the 
layers  of  the  broad  ligament  (intraligamentous  myofibroma). 

Submucous  myofibromas  are  found  most  frequently  at  the  fundus, 


836  THE  UTERUS 

but  occasionally  arise  from  the  internal  os  and  cervical  canal.  They 
are  not  tabulated,  but  form  sessile  nodes  or  pedunculated  outgrowths. 
They  are  usually  small,  but  may  attain  the  size  of  a  child's  head.  Con- 
siderable dilatation  of  the  uterus  may  take  place. 

Histologically,  these  growths  consist  of  a  core  of  fibrous  and  muscular 
tissue,  enveloped  in  mucous  membrane.  Owing  to  contraction  of  the 
uterus,  together  with  retrogressive  changes,  submucous  tumors  may 
become  entirely  detached  or  in  some  cases  shelled  out  from  their 
mucous  investment  and  be  discharged  through  the  genital  passages. 
Occasionally  such  growths  are  calcified. 

The  etiology  of  uterine  myofibromas  is  still  obscure.  Certain  points 
are,  however,  fairly  well  established.  Fibroids  do  not  occur  before  the 
age  of  puberty,  and  are  found  chiefly  in  elderly  women.  The  part  that 
the  sexual  activities  play  is  doubtful.  A  relatively  high  percentage  of 
unmarried  persons  are  said  to  have  these  growths.  Cohnheim1  has 
advanced  the  view  that  the  uterus  contains  "germ  centres"  that  remain 
more  or  less  in  abeyance  while  the  sexual  functions  are  in  operation,  and 
that  some  irritation,  apart  from  the  physiological  one,  leads  to  the  atypical 
and  excessive  development  of  these  centres.  Virchow  also  attributed 
fibroids  to  an  irritative  cause.  Race  plays  a  part,  for  it  is  said  that  the 
condition  is  more  common  in  black  peoples.  With  regard  to  the  site 
of  origin  opinions  also  differ.  Virchow  held  that  myomas  originate  in 
the  muscle  fibers  of  the  myometrium.  Other  views  are  that  they  grow 
from  the  walls  of  the  bloodvessels,  or  from  certain  round  cells  that  are 
said  to  exist  about  capillaries  that  are  undergoing  involution.  Some 
have  also  held  that  certain  cases  are  the  result  of  endometritis.  A  para- 
sitic theory  has  also  been  advanced. 

Myofibromas,  particularly  the  intramural  and  submucous  forms,  but 
also  to  some  extent  the  subserous,  lead  to  generalized  hypertrophy  of 
the  myometrium  with  dilatation  of  the  cavity.  Where  multiple  tumors 
exist,  atrophy  from  pressure  in  some  instances  takes  place.  It  is  not 
uncommon  for  the  endometrium  covering  a  large  submucous  growth 
to  be  thinned  and  atrophic.  Secondary  inflammatory  changes  are 
frequent.  The  Fallopian  tubes  often  show  changes,  the  mucosa  being 
oedematous,  hemorrhagic,  and  infiltrated  with  inflammatory  products, 
while  productive  manifestations  are  not  uncommon.  The  ovaries  are 
said  always  to  show  some  alteration.  They  are  enlarged  from  hyper- 
trophy and  hyperplasia  of  the  follicles  and  proliferation  of  the  connective 
tissue.  The  interstitial  stroma  is  infiltrated  with  round  cells  and  the 
vessels  present  signs  of  endarteritis. 

Myofibromas  are  subject  to  secondary  changes  that  should  be  men- 
tioned. Fatty  degeneration  occurs  usually  in  patches  but  may  sometimes 
be  so  extensive  as  to  convert  the  tumor  into  a  soft,  yellowish  material 
resembling  pus.  It  is,  however,  more  common  to  find  areas  of  softening 
and  degeneration  cysts  in  these  tumors.  In  rare  instances  the  growth 

'Allg.  Path.,  1:1882:744. 


UTERINE  FIBROIDS  837 

may  entirely  disappear.  Pregnancy  and  the  puerperal  state  are  potent 
influences  in  bringing  about  these  retrogressive  changes.  The  tumor 
may  participate  in  the  involution  process  of  the  puerperal  uterus. 

Hyaline  degeneration  and  necrosis  may  affect  the  muscular  elements  to 
such  a  degree  that  the  muscle  tumor  is  gradually  converted  into  a  fibrous 
one  The  fibrous  tissue,  in  turn,  may  undergo  hyaline  and  myxo- 
matous  transformation.  In  the  latter  event,  the  tumor  increases  rapidly 
in  size,  and  may  contain  numerous  cystic  areas  filled  with  mucin  (myxo- 
myomd).  Amyloid  infiltration  has  also  been  observed.  Calcification  is 
relatively  more  common  in  the  subserous  variety.  The  lime  salts  form 
a  porous  network  that  may  be  compared  to  the  siliceous  skeleton  of 
a  sponge,  or  else  form  an  external  hard  covering.  In  rare  cases  the 
entire  tumor  becomes  calcified.  Transformation  into  cartilage  (chondro- 
myomci)  and  into  bone  (osteomyoma)  has  been  recorded. 

Inflammation  of  the  growth,  both  acute  and  chronic,  is  met  with. 
Suppuration  and  gangrene  may  lead  to  complete  disintegration  of  the 
tumor,  or,  if  the  inflammation  be  more  chronic,  to  fibroid  induration. 
In  the  large  growths  oedema  is  of  common  occurrence,  giving  the  struc- 
ture a  soft,  gelatinous  appearance.  This  may  lead  to  the  formation  of 
cystic  cavities  filled  with  clear  fluid.  These  can,  however,  be  readily 
distinguished  from  true  cysts  in  that  they  are  not  lined  with  epithelium, 
and  are  often  traversed  by  shreds  or  bands  of  tissue.  Cysts  may  also 
be  due  to  dilatation  of  the  lymphatics  (myofibroma  lymphangiectaticwn) 
or  bloodvessels  (m.  cavernosum).  Rarely,  cysts  are  found  lined  with 
cylindrical  epithelium.  Some  of  these  are  believed  to  be  due  to  pinching 
off  of  portions  of  the  uterine  glands  that  thus  become  included  in  the 
myomatous  overgrowth,  but  others,  notably  in  the  subserous  forms,  are 
more  probably  derived  from  embryonic  epithelial  "rests."  According 
to  Cullen,  the  forms  in  which  there  are  epithelial  inclusions  are  apt  to 
be  diffused  throughout  the  uterus.  The  "rests"  have  been  definitely 
shown  to  be  derived  from  the  mucosa. 

An  important  modification,  that  should  be  referred  to,  is  sarcomatous 
transformation  of  uterine  myomas,1  of  which  five  or  six  cases  are  now 
on  record.  The  condition  gives  rise  to  metastatic  myomatous  deposits 
in  the  various  organs.  The  tumor  grows  rapidly  and  on  section  presents 
a  more  homogeneous  appearance  than  the  ordinary  myofibroma.  It  is 
liable  to  undergo  degenerative  changes,  necrosis  and  extravasation  of 
blood.  Microscopically,  the  cells  are  large,  spindle-shaped,  or  irregular, 
containing  large  nuclei  rich  in  chromatin.  Giant  cells  may  also  be  seen. 
Two  forms  are  to  be  differentiated,  the  first  in  which  the  sarcomatous 
elements  arise  from  more  or  less  undifferentiated  and  embryonic  muscle 
cells  (myosarcoma) ,  and  the  second,  in  which  there  is  transformation  of 
the  interstitial  fibrous  tissue  into  sarcoma  (myoma  sarcomatodes). 

1  See  Mastuy,  Zur  Kenntniss  der  malignen  Myome  des  Uterus,  Zeit.  f.  Heilk., 
22;  Abth.  f.  Path.  Anat:  also  Schlagenhaufer,  Myoma  teleangiectodes  Uteri, 
Wien.  klin.  Woch.,  15: 1902: 523. 


838  THE  UTERUS 

The  combination  of  adenoma  with  myoma  is  rare.  Here,  in  addition 
to  the  muscular  or  fibromuscular  ground  substance,  there  are  more  or 
less  numerous  glands  lined  with  epithelium,  in  some  cases  dilated  into 
cysts.  In  the  tumors  originating  in  the  centre  of  the  myometrium 
the  glandular  elements  have  been  shown  to  be  derived  from  the  endo- 
metrium  (Cullen),  while  in  those  cases  where  the  tumors  start  in  the 
cornu  of  the  uterus  or  in  a  tube,  they  are  probably  derived  from  the  re- 
mains of  the  Wolffian  body  (v.  Recklinghausen).  The  growth  is  generally 
devoid  of  a  capsule,  and  is  adherent  to  the  tissue  in  which  it  is  found. 
Several  subvarieties  are  described:  (1)  One  in  which  the  muscular 
elements  are  in  excess;  (2)  one  in  which  the  glandular  elements  predomin- 
ate; (3)  a  tumor  containing  numerous  dilated  bloodvessels  (adenomyoma 
teleangiectaticum);  and  (4)  a  cystic  adenomyoma  (adenomyoma  cysticum}. 

Microscopically,  the  growth  consists  in  a  varying  amount  of  muscular 
and  fibrous  tissue,  either  well-formed  or  of  cellular  appearance,  in  which 
are  several  glandular  tubules  lined  with  epithelium,  from  which  branch 
off  in  a  pectinate  fashion  subsidiary  tubules  ending  in  a  blind  ampulla. 
The  cysts  may  contain  blood-stained  fluid  or  pigment. 

In  a  few  cases  striated  muscle  tumors,  rhabdomyomas,  have  been 
described.  They  form  polypoid  excrescences  in  the  cervical  canal,  and 
are  very  malignant,  owing  to  the  fact  that  the  component  cells  are 
intrinsically  immature  and  endowed  with  great  vegetative  force.  In 
one  such  tumor  glycogen  and  amyloid  material  have  been  found. 

Myocarcinoma  probably  only  occurs  in  the  form  of  a  carcinomatous 
transformation  of  the  glandular  elements  in  an  adenomyoma.  The  con- 
dition may,  however,  be  simulated  closely  by  the  secondary  invasion  of 
a  myofibroma  with  carcinoma,  either  by  metastasis,  which  is  rare,  or  by 
direct  extension. 

Lipoma. — Lipomas  have  been  found  growing  as  polypoid  excrescences 
from  the  cervical  canal.  They  are  excessively  rare. 

Ghondroma. — Chondromas  occur  generally  as  metaplasias  of  other 
tumors,  notably  rhabdomyoma  and  sarcoma. 

Cystic  Growths. — Besides  cystic  polyps,  cystadenomas,  cystic  myomas, 
and  sarcomas,  the  only  form  that  need  be  mentioned  is  the  dermoid 
cyst,  which  may  form  polypoid  outgrowths. 

The  malignant  tumors  of  the  uterus  are  the  malignant  adenoma, 
carcinoma,  sarcoma,  endothelioma,  and  rbabdomyoma  (above  described). 

Malignant  Adenoma. — Here,  as  elsewhere,  it  is  difficult  to  decide  with 
certainty  what  is  a  neoplastic  overgrowth  of  glandular  tissue  and  what 
is  merely  an  inflammatory  hyperplasia.  Some  would  regard  the  inflam- 
matory polyps  to  be  referred  to  later  as  adenomas.  The  difficulty 
arises  from  the  fact  that  in  inflammation  the  glandular  elements  are  in- 
creased, both  in  numbers  and  in  size,  forming  branching  and  often  com- 
municating tubes,  with,  sometimes,  cystic  dilatation.  The  gland-tubes 
also  tend  to  invade  the  muscular  layers.  The  existence  of  cellular  in- 
filtration, again,  affords  no  clue,  since  it  is  present  alike  in  new-growths 
and  in  inflammation.  There  can  be  no  doubt,  however,  that  the  so- 
called  malignant  adenoma  is  a  true  tumor.  It  consists  almost  entirely 


CARCINOMA  839 

of  glandular  elements  in  the  shape  of  branching  and  intercommunicating 
tubules,  which  may  present  dilatation  or  intraglandular  invagination, 
held  together  by  a  scanty  stroma.  The  tubules  in  question  are  lined 
with  a  single  layer  of  more  or  less  distorted  and  closely  packed,  long, 
cylindrical  cells,  sometimes  ciliated,  and  showing  mitotic  figures.  The 
muscular  wall  of  the  uterus  may  be  extensively  infiltrated.  This  tumor 
is  found  usually  about  the  menopause  or  later.  The  importance  of 
the  growth  lies  in  the  fact  that  it  is  infiltrating  and  may  give  rise  to 
distant  metastases,  also  of  the  simple  adenomatous  type. 

Carcinoma. — The  uterus  is  a  favorite  place  for  carcinoma.  In  about 
30  per  cent,  of  women  suffering  from  this  disease,  the  growth  is  located 
in  the  uterus  (Orth).  With  few  exceptions,  carcinoma  of  the  uterus  is 
primary  in  that  organ.  The  affection  may  be  found  at  any  time  after 
puberty,  but  is  generally  met  with  about  the  menopause  or  later.  In 
nulliparae,  cancer  of  the  uterus  is  rare,  and  when  it  does  occur  is  usually 
in  the  corpus.  In  parous  women  it  is  usually  at  the  cervix.  The 
etiology  is  obscure,  but,  so  far  as  we  can  judge,  the  most  important  factor 
is  chronic  endometritis  leading  to  glandular  hyperplasia.  Whether 
traumatism,  as  laceration  of  the  cervix,  has  much  to  do  with  it  is  per- 
haps debatable,  yet  it  seems  probable.  Tears  or  fissures  in  this 
situation,  if  they  do  not  heal  in  complete  apposition,  often  become 
covered  with  epithelium,  either  squamous  from  the  vagina,  or  columnar 
from  the  cervix.  Such  epithelium,  being  in  an  abnormal  situation,  is 
likely  to  be  unstable  and  more  susceptible  to  irritation.  Granular 
erosion  of  the  portio  vaginal  is  is  possibly  a  factor  also  in  some  cases. 
Race  plays  some  part,  for  the  disease  is  said  to  be  more  frequent  in  the 
white  peoples. 

Carcinoma  may  affect  any  part  of  the  uterus,  but  in  the  vast  majority 
of  cases  is  met  with  in  the  cervix.  We  have  to  recognize  three  points  of 
origin,  the  portio  vaginalis,  the  cervical  endometrium,  and  the  mucosa 
of  the  corpus.  With  regard  to  the  forms  occurring  in  the  cervix,  it  is 
only  possible  to  differentiate  between  them  macroscopically  in  the 
earlier  stages. 

Carcinoma  of  the  cervix  presents  at  first  a  smooth,  slightly  reddened 
surface,  quickly  becoming  uneven,  granular,  warty,  and  eroded.  The 
outgrowth  is  not  always  so  marked  as  the  infiltration,  but  very  commonly 
polypoid  or  papillomatous  excrescences  are  formed  giving  to  the  tumor 
the  well-known  "  cauliflower"  appearance.  In  cancer  of  the  corpus  and 
cervical  cavity  such  outgrowths  are  not  so  common.  The  tumor  takes 
the  form  of  squamous  epithelioma,  adenocarcinoma,  scirrhous,  and  colloid1 
carcinoma.  The  last  two  varieties  are  excessively  rare. 

A  frequent  type  is  the  squamous-celled  carcinoma,  which  arises  almost 
invariably  from  the  portio  vaginalis.  It  forms  flat  or  papillomatous 
outgrowths  originating  in  the  superficial  layers  of  the  mucosa.  It  is, 
moreover,  liable  to  spread  to  the  vagina,  and  may  extend  into  the  para- 
metrium  with  extensive  destruction  of  tissue.  In  very  rare  instances,  as 

1  Waldeyer,  Virch.  Archiv,  55: 1872: 110. 


840  THE  UTERUS 

v.  Rosthorn1  and  Zeller2  have  pointed  out,  a  metaplasia  of  the  cylindrical 
epithelium  of  the  uterine  cavity  into  squamous  cells  may  take  place, 
and  three  undoubted  cases  (Gebbard,3  Kaufmann,4  Fleischlen5)  are  on 
record  where  squamous-celled  carcinoma  has  developed  in  the  body  of 
the  uterus.  This  form  may  also  originate  in  the  cervical  canal  in  cases 
of  erosion  where  the  pavement  epithelium  of  the  portio  has  invaded  the 
cavity. 

Histologically,  the  squamous-celled  carcinoma  presents  an  overgrowth 
of  epithelial  processes,  more  or  less  branching,  which  invade  the  deeper 
layers.  The  stroma  is  infiltrated  with  round  cells,  and  cell-nests  are 
occasionally  to  be  seen.  In  some  cases  the  overgrowth  of  epithelium 
and  stroma  is  so  great  that  papillomatous  excrescences  are  produced. 
Erosion  often  takes  place,  and  the  surface  presents  granulation  tissue 
together  with  masses  of  fibrin.  Rarely,  the  growth  assumes  the  type 
of  a  rodent  ulcer,  being  of  slow  growth,  with  merely  superficial  loss  of 
substance,  and  separated  from  the  underlying  structures  by  a  zone  of 
round  cells. 

The  most  common  form  of  cancer  is  the  adenocarcinoma,  which  is 
found  in  the  cervix  and  occasionally  in  the  corpus.  The  growth  originates 
in  an  atypical  proliferation  of  the  glandular  elements  of  the  endometrium. 
The  explanation  probably  is  that  it  arises  from  portions  of  glands  or 
cysts  (ovula  Nabothi)  that  have  become  pinched  off  from  the  superficial 
mucous  membrane.  The  tumor  is  definitely  of  the  glandular  type  and 
penetrates  deeply  into  the  muscle.  The  cells  are  cylindrical  and  may  be 
grouped  like  glands  about  a  central  lumen,  or  heaped  up  into  several 
layers,  forming  solid  masses  or  strands.  Cavities  may  be  formed  through 
softening  and  necrosis  of  the  central  portion  of  the  growth.  According 
to  the  amount  of  stroma  present  we  can  differentiate  carcinoma  simplex 
and  c.  medullare.  The  tissues  in  the  neighborhood  of  the  growth  show 
an  abundant  round-celled  infiltration.  Myxomatous  degeneration  of  the 
stroma  is  occasionally  observed. 

Palmer  Findley6  has  recorded  a  case  of  cancer  of  the  body  of  the  uterus 
which  was  both  adenocarcinomatous  and  squamous  in  type.  A  peculiar 
and  apparently  unique  form  of  adenocarcinoma  is  one  recorded  by 
Cullen,7  where  the  tumor  formed  dome-like  elevations  affecting  both 
corpus  and  cervix  alike,  in  which  the  epithelium  of  the  glandular  elements 
resembled  closely  that  of  the  normal  glands. 

1  Ueber  Schleimhautverhornung  der   Gebarmutter,    Zeit.  zur  Feier  des  funfzig- 
jahrigen  Jubilaums  der  Gesselsch.  f.  Gyn.  Wien,  1894:  319. 

2  Plattenepithel  im  Uterus,  Zeit.  f.  Geb.  u.  Gyn.,  11:  1884-85:  56. 

3  Zeit.  f.  Geb.  u.  Gyn.,  24:  1892:  1. 

4  Jahresbericht   der  Schlesischen  Gesselsch  f.  vaterlandische  Cultur.  Jahrg.,  72: 
1894:  52. 

5  Ueber  den  primaren  Hornkrebs  des  Corpus  Uteri,  Zeit.  f.  Geb.  u.  Gyn.,  32: 
1895:  347. 

6  Squamous  Cell  Carcinoma  of  the  Body  of  the  Uterus,    Trans.  Chicago  Path. 
Soc.,  5:  No.  6:  1902. 

7  Cullen,  Tumors  of  the  Uterus,  1900:  588,  D.  Appleton  &  Co.,  N.  Y. 


SARCOMA  841 

Primary  melanocarcinoma  is  described.1 

Numerous  secondary  changes  are  associated  with  carcinoma.  The 
surface  may  become  ulcerated  and  almost  gangrenous,  with  consequent 
enlargement  of  the  uterine  cavity.  Obstruction  in  the  cervical  canal 
often  leads  to  retention  of  secretion  and  necrotic  material  and  dilata- 
tion of  the  uterus.  Chronic  endometritis,  either  of  simple  or  membranous 
type,  may  complicate  the  condition  also.  Hypertrophy  of  the  uterine 
wall  may  occur. 

Extension  of  the  disease  to  neighboring  parts  is  common.  Cancers 
of  the  portio  tend  to  invade  the  vagina,  while  those  of  the  cervix  extend 
to  the  parametrium.  Both  the  bladder  and  the  rectum  may  be  involved 
and  fistulous  communications  established.  In  the  former  case,  cystitis 
may  be  set  up  with  obstruction  of  the  ureters,  leading  to  hydro-  and  pyo- 
nephrosis.  In  exceptional  cases  the  bony  pelvis  may  be  attacked.  The 
peritoneal  membrane  is  often  involved,  especially  in  cases  of  carcinoma 
of  the  corpus,  but  also  of  the  cervix.  Local  peritonitis  with  adhesion 
may  occur.  Metastases  develop  relatively  late  and  are  never  extensive. 
Most  frequently  the  lumbar,  retroperitoneal,  and  inguinal  lymphatic 
glands  are  first  and  chiefly  involved,  and  also  the  ovaries.  Secondary 
carcinoma  is  rare  in  the  uterus.  Orth  has  observed  a  metastatic  polypoid 
melanocarcinoma  in  a  case  of  generalized  melanocarcinosis. 

Sarcoma. — Sarcomas  originate  either  in  the  connective  tissue  of  the 
endometrium,  possibly  as  a  sequel  of  chronic  productive  endometritis, 
or  in  the  myometrium.  In  the  latter  case  the  tumor  is  frequently  asso- 
ciated with  myofibroma,  forming  a  mixed  growth. 

Sarcomas  developing  in  the  endometrium  are  found  relatively  early 
in  life  as  compared  with  carcinomas,  even  before  puberty.  They  are 
common  relatively  in  nulliparse. 

Microscopically,  sarcomas  are  large  or  small  round-celled,  spindle-celled, 
oat-shaped,  giant-celled,  or  mixed.  In  some  cases  they  are  very  vascular 
(angiosarcoma) ,  or  the  vessels  may  show  hyaline  thickening  (cylindromd). 
Sarcomas  originating  in  the  mucosa  are  found  in  the  corpus,  rarely  in 
the  cervix.  They  form  local  or  more  or  less  diffuse  growths  having 
a  lobulated,  warty,  or  papillomatous  appearance.  Ulceration  is  apt 
to  occur  early.  In  the  cervix  they  form  polypoid  or  cauliflower-like 
growths  in  the  canal  or  on  the  lips  of  the  os.  In  consequence  of  con- 
gestion and  cedema,  they  may  present  an  appearance  not  unlike  an 
hydatidiform  mole.  The  substance  of  the  tumor  is  whitish,  soft  and 
brain-like,  friable,  and  shows  evidences  of  degeneration. 

The  sarcomas  of  the  myometrium  form  usually  single  or  multiple 
nodules  of  varying  size,  more  rarely  a  diffuse  infiltration  leading  to 
marked  enlargement  of  the  uterus.  As  a  rule,  they  are  met  with  in  the 
corpus,  but  may  occur  also  in  the  cervix.  Pure  sarcomas  of  this  type 
have  a  pale  homogeneous  appearance  and  are  of  soft  consistence. 

As  before  mentioned  sarcoma  may  be  associated  or  combined  with 
myofibroma  and  adenoma. 

1  Haeckel,  Arch.  f.  Gyn.,  32:  1888:  400. 


842  THE  FALLOPIAN  TUBES 

Sarcoma  of  the  uterus  may  for  a  long  time  remain  latent  and  then 
suddenly  take  on  rapid  action,  infiltrating  and  penetrating  the  uterine 
wall,  and  extending  to  the  peritoneum,  broad  ligaments,  tubes,  ovaries, 
intestines,  and  abdominal  parietes.  Implantation  metastases  are  rarer 
than  with  carcinoma.  Distant  metastases  are  also  rare.  When  they 
occur,  they  are  found  first  and  chiefly  in  the  lungs.  The  retroperitoneal 
lymphatic  glands  are  commonly  implicated. 

Degenerative  changes,  particularly  hemorrhagic  extravasation,  necrosis, 
fatty  degeneration,  and  liquefaction,  are  apt  to  be  present  in  sarcomas. 

Secondary  sarcoma  of  the  uterus  has  been  met  with.  There  is  in  the 
pathological  institute  at  Prague  an  interesting  specimen  of  a  uterus 
with  multiple  fibroids  in  which  there  are  secondary  nodules  of  melano- 
sarcoma. 

Endothelioma. — Endotheliomas  originating  in  the  lining  cells  of  blood- 
vessels and  lymphatics  have  been  met  with  in  rare  instances.1 

The  uterus,  it  should  moreover  be  mentioned,  is  a  favorite  site  for 
multiple  and  independent  primary  growths.  The  association  of  uterine 
fibroids  with  carcinoma  is,  of  course,  so  common  as  scarcely  to  excite 
remark,  but  instances  are  on  record  of  associated  malignant  growths. 
A  case,  for  instance,  is  described2  of  carcinoma  and  sarcoma  of  the  body 
of  the  uterus. 

THE  FALLOPIAN  TUBES. 

CONGENITAL    ANOMALIES. 

Congenital  defects  of  the  tubes  are  usually  associated  with  anomalies 
of  the  uterus,  although  exceptions  occur.  The  tube  may  be  almost 
completely  absent,  being  represented  by  a  mere  tag.  Not  infrequently 
the  fimbriae  are  imperfectly  developed,  a  persistence  of  the  infantile  con- 
dition. Another  anomaly  is  an  unusual  position,  as  prolapse  or  a  vertical 
course.  In  about  one-fifth  of  the  autopsies  on  women  a  small  cyst 
(hydatid  of  Morgagni),  filled  with  clear  fluid,  is  found  at  the  end  of  the 
fimbrise  or  attached  by  a  long  pedicle. 

Diverticula  occasionally  are  met  with  caused  by  a  hernial  protrusion 
of  the  mucosa  through  the  muscular  wall.  Accessory  openings  may  be 
found  near  the  fimbriated  extremity  of  the  tubes. 

ALTERATIONS  IN  POSITION  AND  CONTINUITY. 

Apart  from  the  congenital  anomalies  of  position,  above  referred  to, 
the  position  of  the  tubes  depends  mainly  on  pathological  changes  in  the 
neighboring  structures,  uterus,  ovaries,  and  peritoneum.  Displacements 
of  the  uterus,  ovarian  tumors,  and  inflammatory  adhesions  frequently 

1  See  Elizabeth  Hurdon,  Johns  Hopkins  Hosp.  Bull.,  9: 1898: 187. 

2  Emmanuel,  Zeitsch.  f.  Geb.  u.  Gyniik.,  34: 1896: 1. 


STENOSIS  AND  ATRESIA  843 

drag  the  tubes  out  of  their  normal  position.  When  prolonged  tension 
is  put  upon  a  tube  it  atrophies  at  some  point,  usually  near  the  uterus, 
and  may  even  be  separated  from  the  uterus.  The  tube  may  also  be 
twisted  spirally  upon  its  axis.  The  tube  has  been  found  forming  part 
of  the  contents  of  a  hernial  sac  (salpingocele). 

Stenosis  and  Atresia. — Stenosis  and  atresia  of  the  lumen  are  com- 
paratively common.  The  abdominal  and  uterine  ostia  are  the  parts  most 
likely  to  be  involved.  When  both  tubes  are  affected  sterility  results. 
A  slight  grade  of  the  affection  is  said  to  be  one  of  the  causes  of  extra- 
uterine  gestation.  The  cause  is  usually  salpingitis  or  pelvic  peritonitis. 
In  such  cases  secretions  and  fluids  of  various  kinds,  blood,  pus,  or 
serum,  may  distend  the'  tube  into  a  form  of  cyst  (hemato-,  pyo-,  and 

FIG.  221 


Double  hydrosalpinx.     The  specimen  shows  also  an  intramural  "  fibroid"  of  the  uterus. 
(From  the  Pathological  Museum  of  McGill  University.) 

hydrosalpinx).  In  cases  where  the  abdominal  end  only  of  the  tube  is 
closed,  there  may  be  a  periodical  discharge  of  clear  or  bloody  fluid  into 
the  uterus,  which  may  simulate  menstruation  (hydrops  profluens). 
WThen  secretions  are  retained  the  mucous  membrane  is  flattened,  the 
cells  have  lost  their  cilia,  while  the  muscle  bands  are  compressed,  atrophic, 
and  more  or  less  dissociated.  The  tube  in  this  condition  may  be  con- 
verted into  a  semitransparent,  thin-walled  sac.  No  fimbrise  can  be 
seen,  as  in  some  curious  fashion  they  become  inverted. 

Tuboovarian  Cyst. — A  brief  reference  should  be  made  here  to  a 
special  form  of  tubal  hydrops,  the  so-called  tuboovarian  cyst.  The  wall 
of  the  cyst  is  composed  in  part  of  tube,  in  part  of  ovary.  It  is  usually 
brought  about  by  the  bursting  of  an  ovarian  cyst  into  the  tube,  and  is 
of  the  nature  either  of  a  true  cystoma,  a  hydropic  Graafian  follicle, 


844  THE  FALLOPIAN  TUBES 

or  a  cystic  corpus  luteum.  According  to  Bland  Sutton,  there  is  in  some 
cases  an  accessory  fold  of  peritoneum  forming  a  sort  of  covering  about 
the  ovary,  analogous  to  the  tunica  of  the  testis,  in  which  fluid  collects 
(ovarian  hydrocele). 

Rupture  of  the  tube  is  rare,  except  in  cases  of  tubal  gestation. 

Ulceration  is  also  rare.     It  may  occur  in  tuberculosis  and  carcinoma. 


CIRCULATORY  DISTURBANCES. 

These  are  similar  to  those  occurring  in  the  uterus.  Hyperemia  is 
found  in  infective  diseases  and  in  obstruction  of  the  inferior  vena  cava. 
Small  hemorrhages  into  the  mucosa  are  met  with  not  infrequently  in 
cases  of  burns,  phosphorus  poisoning,  and  in  the  hemorrhagic  diatheses. 

An  accumulation  of  blood  within  the  tube  (hematosalpinx)  may  be 
due  to  retention  of  menstrual  or  extravasated  blood,  owing  to  atresia 
in  some  part  of  the  genital  canal.  It  may  be  associated  with  chronic 
inflammation  and  hypertrophy  of  the  muscular  wall  of  the  tube. 

INFLAMMATIONS. 

Salpingitis. — Inflammations  of  the  tubes  (salpingitis)  are,  for  the 
most  part,  comparable  to  those  of  the  uterus.  The  mucous  membrane 
may  be  chiefly  affected,  or  all  the  coats  may  be  involved. 

Acute  Catarrhal  Salpingitis. — In  simple  acute  catarrhal  salpingitis 
the  mucous  membrane  is  reddened,  swollen,  and  infiltrated.  The 
secretion  is  scanty,  grayish  or  grayish-white  in  color,  and  contains  small 
masses  of  desquamated  and  degenerated  epithelium. 

Chronic  Catarrhal  Salpingitis. — More  common  is  chronic  catarrh, 
where  the  most  important  feature  is  productive  change  in  the  mucous 
membrane.  The  folds  of  the  mucosa  are  thickened  and  infiltrated, 
and  owing  to  the  loss  of  the  epithelium,  become  adherent  or  connected 
by  fibrous  bands.  In  this  way  small,  gland-like  structures  are  pinched 
off  from  the  general  cavity,  which  may,  in  time,  be  converted  into  cysts. 
In  this  feature  there  is  a  striking  similarity  to  glandular  endometritis. 
Under  the  designation  of  salpingitis  productiva  glandularis,  Chiari1  has 
described  a  special  form  of  chronic  catarrh,  in  which  small  nodular 
outgrowths  are  found  at  the  uterine  end  of  the  tube,  composed  of  hyper- 
plastic  muscle  and  gland-like  structures  from  the  mucous  membrane, 
often  cystic  in  appearance,  which  contain  a  clear  serous  fluid  and  are 
lined  with  non-ciliated  epithelium. 

In  cases  of  chronic  catarrh  the  muscular  coat  may  be  relatively 
unaffected,  showing  either  hypertrophy  or  atrophy,  but  at  times  it  is 
also  infiltrated  with  inflammatory  products.  Productive  hyperplasia 
of  the  connective-tissue  elements,  chiefly  along  the  bloodvessels,  often 
supervenes  (interstitial  salpingitis). 

1  Zeit.  f .  Heilk.,  8:1887:457. 


SALPINGITIS  845 

Apart  from  the  simple  forms  of  salpingitis,  we  have  to  recognize, 
according  to  the  character  of  the  exudate,  suppurative  and  membranous 
varieties. 

Suppurative  Salpingitis. — There  is  no  marked  difference,  etiologically  or 
anatomically,  between  simple  and  suppurative  salpingitis.  In  both 
there  is  the  same  infiltration  and  adhesion  of  the  mucous  folds.  In  the 
suppurative  form,  however,  the  secretion  is  more  abundant,  seropurulent 
or  purulent,  and  tends  to  collect  in  the  ampulla.  The  amount  of  de- 
generation of  the  epithelium  is  also  greater  and  sets  in  earlier.  In  this 
way  most  of  the  mucous  membrane  may  be  destroyed.  In  some  cases 
the  mucosa  is  greatly  infiltrated  with  cells,  and  becomes  fibrous  and 
indurated.  The  condition  of  the  muscular  coat  varies.  Cellular  infiltra- 
tion, the  formation  of  multiple  small  abscesses,  and  productive  changes 
are  common.  In  many  cases,  owing  to  the  inflammation,  both  the 
uterine  and  the  abdominal  openings  of  the  tube  become  blocked,  and 
the  pus  accumulates  until  the  tube  assumes  a  sac-like  appearance 
(pyosalpinx).  The  tube  may  thus  become  greatly  distorted  and  present 
irregular  swellings,  owing  to  the  sacculation  of  the  contents  through 
adhesions  (pyosalpinx  saccata).  Mauclaire1  has  recorded  a  curious  case 
of  purulent  salpingitis  where  gas  was  produced  v  (physopyosalpinx),  the 
exact  cause  of  which  was  not  determined. 

Membranous  Salpingitis. — Membranous  salpingitis  is  of  relatively  little 
importance;  it  is  characterized  by  necrosis  with  the  formation  of  an 
adherent  fibrinous  or  fibrinohyaline  exudation. 

The  causes  of  salpingitis  are  various.  Among  ordinary  sources  may 
be  mentioned  "catching  cold,"  or  traumatism  during  the  menstrual 
period,  inflammation  of  the  uterus  or  ovaries,  tumors  of  the  uterus, 
dislocations,  and  general  systemic  infection.  The  majority  of  cases  are 
bacterial  in  origin.  Some  cases  are  extensions  of  septic  endometritis 
and  metritis.  The  most  frequent  cause,  however,  is  the  Gonococcus, 
which  may  not  only  produce  simple  acute  salpingitis  but  also  chronic 
and  suppurative  inflammation.  It  is  not  always  possible  to  determine 
the  microorganism  at  fault,  for  it  often  happens  that  the  bacteria  die 
out  in  long-standing  cases.  Gonococci  especially  tend  to  disappear 
early. 

A  number  of  serious  results  may  follow  salpingitis.  A  frequent 
event  is  the  extension  of  the  inflammation  to  the  serous  membrane 
(perisalpingitis)  and  to  the  pelvic  peritoneum  (pelvic  peritonitis),  leading 
to  dislocations  of  position  and  distortion  of  the  tubes  from  the  formation 
of  adhesions.  When  pyosalpinx  is  present  the  pus  may  escape  into  the 
peritoneal  cavity,  setting  up  a  general  peritonitis,  or  it  may  be  walled 
off  by  adhesions  so  as  to  form  a  pelvic  abscess.  Abscesses  of  this  kind 
may  discharge  into  the  vagina  or  rectum.  In  the  cases  that  undergo 
involution,  the  pus  is  often  absorbed,  becomes  inspissated  into  a  thick, 
whitish,  putty-like  material  that  may  be  mistaken  for  caseation,  or  the 
contents  may  become  calcified. 

1  Bull.  et.  mem.  de  la  Soc.  anat.  de  Paris,  April,  1901. 


846  THE  FALLOPIAN  TUBES 

Syphilis. — But  little  is  known  of  syphilitic  lesions  of  the  tubes. 
So  far  as  we  know,  only  one  case,  that  of  Bouchard  and  Lepine,1  is 
recorded,  where  the  tubes  were  thickened  and  dilated  to  the  size  of  the 
finger  and  contained  gummas. 

Tuberculosis. — This  is  much  more  common,  and  is  primary  or 
secondary.  It  is  said  (Orthmann)  that  primary  tuberculosis  occurs  in 
about  18  per  cent,  of  all  cases  of  genital  tuberculosis  in  the  female.  The 
infection  is  almost  always  hematogenic,  although  it  is  conceivable  that 
some  cases  may  arise  from  the  presence  of  sperm  containing  tubercle 
bacilli.  Secondary  tuberculosis  may  arise  by  the  extension  of  disease 
from  the  peritoneum,  the  ovaries,  or  the  uterus.  In  both  types  the  peri- 
toneum and  the  rest  of  the  genitalia  are  apt  to  be  involved  as  well.  As 
a  rule,  both  tubes  are  affected,  although  not  always  to  the  same  degree. 

Judging  from  the  extent  of  the  lesions  usually  found,  the  Fallopian 
tubes  form  a  particularly  good  soil  for  the  development  of  the  tubercle 
bacillus.  What  constitutes  this  special  predisposition  is  not  exactly 
known,  but  it  would  seem  that  previously  existing  inflammatory  or 
circulatory  disturbances,  and  disorders  arising  during  menstruation  and 
the  puerperium,  play  an  important  part.  Rokitansky  pointed  out  that 
the  disease  was  particularly  common  after  the  puerperium.  While, 
however,  it  is  true  that  the  disease  is  commonly  met  with  during  the 
period  of  greatest  sexual  activity,  it  is  nevertheless  found  in  old  women 
and  children. 

Tuberculosis  generally  begins  in  the  mucous  membrane  of  the  ampulla 
and  spreads  rapidly  to  the  adjacent  parts.  The  affected  tube  is  greatly 
thickened,  firm,  more  or  less  tortuous,  and  the  muscular  wall  is  hyper- 
trophic.  The  fimbrise  are  short,  thick,  and  firm.  As  a  rule,  the  tube 
is  bound  down  by  inflammatory  adhesions.  On  opening  the  tube,  in 
the  early  stages  the  mucosa  is  swollen,  reddened,  and  the  folds  are 
adherent,  while  the  lumen  contains  a  small  amount  of  grayish  or  yellowish 
secretion.  The  appearance  is  similar  to  that  in  simple  chronic  productive 
salpingitis.  In  more  advanced  cases  grayish  points  can  be  seen  in  the 
mucosa,  or,  again,  caseous  nodules  or  streaks.  Later,  the  mucosa  may 
be  converted  into  a  dense  caseous  mass.  The  lumen  may  be  obliterated, 
or  enlarged  when  the  necrotic  material  has  been  evacuated. 

Microscopically,  the  mucosa  is  swollen,  infiltrated  with  round  and 
epithelioid  cells,  while  here  and  there  can  be  seen  remains  of  the  gland- 
follicles,  frequently  showing  cystic  dilatation.  Definite  tubercles  are  to 
be  seen  near  the  lumen  with  central  caseation.  In  the  more  advanced 
cases  the  mucosa  is  largely  caseous,  and  the  process  can  be  seen  advancing 
into  the  muscular  and  serous  coats.  In  the  more  chronic  forms  giant 
cells  can  be  made  out. 

The  caseous  detritus  in  some  cases  becomes  liquefied  and  puriform, 
and  may  be  retained  and  sacculated  (tuberculous  pyosalpinx).  Some 
of  these  cases  are  examples  of  mixed  infection.  Ulceration  and  perfora- 
tion of  the  tube  is  rare.  Usually  the  abscess  produced  is  walled  off 

^az.  med.  de  Paris,  1866;  726. 


TUMORS  847 

by  adhesions.  The  tube  may  become  adherent  to  the  uterus,  the  ap- 
pendix, or  to  other  portions  of  the  intestinal  tract,  or  may  be  bound 
down  in  Douglas'  sac. 

Actinomycosis.1 — This  is  rare  and  generally  is  due  to  extension  of 
the  disease  from  the  peritoneum.  The  tube  is  thickened,  studded  with 
granulomata,  and  the  puriform  exudate  and  detritus  contain  the  actino- 
myces  "grains." 

Foreign  Bodies  and  Parasites. — These  are  of  little  importance, 
apart  from  bacteria.  Orth  records  a  curiosity  in  the  form  of  a  round- 
worm  that  had  made  its  way  into  a  tube  from  a  ruptured  intestine. 

RETROGRESSIVE  METAMORPHOSES. 

Simple  atrophy  affecting  the  muscular  wall  and  mucosa  is  met  with 
after  the  menopause.  It  may  also  be  due  to  the  pressure  of  retained 
secretions,  and  the  pressure  or  traction  of  tumors.  In  some  cases  the 
tube  may  be  actually  separated  from  the  uterus. 

PROGRESSIVE  METAMORPHOSES. 

Hypertrophy. — Hypertrophy  of  all  the  tissues  of  the  tube  is  not 
uncommon.  Overgrowths  of  the  mucosa  are  met  with  as  a  result  of 
inflammation  or  possibly  as  a  true  hyperplasia,  as,  for  instance,  in  asso- 
ciation with  myofibromas  of  the  uterus.  Polyps  of  the  mucosa  are  very 
rare.  Hypertrophy  of  the  muscle  results  from  overwork,  such  as  is  met 
with  in  stenosis  of  the  ostium  and  in  retention  of  blood  or  secretion. 

The  fimbrise  are  occasionally  thickened,  fibrous,  or  club-like. 

Tumors. — Tumors  of  the  tube,  at  least  the  primary  ones,  are  rare. 
Fibromas  and  myxomas,  often  multiple,  are  met  with. 

Warty  or  papillomatous  outgrowths  of  fibrous  nature  are  described, 
at  times  containing  a  clear  fluid  or  dilated  into  cysts.  Subserous  lipomas 
have  been  met  with.  Benign  papillomas  have  also  been  reported,  and 
a  cystoma  papilliferum  (Eberth). 

Sarcoma  is  excessively  rare.  Carcinoma  is  usually  secondary  to  carci- 
noma of  the  uterus  or  ovary.  As  a  rule,  it  takes  the  form  of  a  diffuse 
growth  in  the  mucosa,  or  forms  nodules  in  the  muscle  and  serosa.  It 
is  usually  of  the  soft  type,  but  may  be  scirrhous.  Primary  carcinoma  of 
the  tube,  as  a  rule,  takes  the  papillary  form.  Le  Count2  has  discussed 
the  nature  of  these  growths. 

THE  OVARIES. 

The  ovaries  are  ovate  glands  situated  on  the  posterior  aspect  of  the 
broad  ligaments.  They  are  attached  by  a  reduplication  of  the  peri- 

1  Zemann,  Wien.  med.  Jahrb.,   1883:  477. 

2  The  Genesis  of  Carcinoma  of  the  Fallopian  Tubes  in  Hyperplastic  Salpingitis, 
Johns  Hopkins  Hosp.  Bull,  12: 1901:  120, 


848  THE  OVARIES 

toneal  membrane — the  mesovarium,  which,  however,  ends  abruptly 
without  forming  a  complete  covering  for  the  organs.  In  the  adult 
the  ovaries  measure  2.5  to  5  cm.  in  length,  2  to  3  cm.  in  breadth,  and 
1  to  2  cm.  in  thickness.  Their  weight  ranges  from  5  to  7  grams.  From 
the  inner  end  within  the  layers  of  the  broad  ligament  runs  a  fibrous 
cord  containing  unstriped  muscle — the  ligamentum  ovarii. 

Microscopically,  the  ovary  consists  of  two  parts :  a  medullary  portion 
composed  of  strands  of  connective  tissue,  unstriped  muscle,  abundant 
bloodvessels,  and  in  some  cases  rows  of  cells  of  embryonic  type  derived 
from  the  Wolffian  body,  which  are  in  relationship  with  the  epoophoron 
(Markstrange-Kolliker) ;  and  a  cortical  part  composed  of  rather  cellular 
connective  tissue,  containing  the  Graafian  follicles.  The  outermost 
layer  of  the  cortex,  called  the  tunica  albuginea,  'is  more  condensed  and 
contains  fewer  follicles,  but  does  not  form  a  definite  membrane. 

The  Graafian  follicles  measure  0.04  to  0.15  mm.  in  diameter.  They 
are  formed  externally  of  a  connective-tissue  membrane — the  theca 
folliculi — which  is  composed  of  an  outer  layer  of  fibrous  character  (tunica 
fibrosa),  an  inner  softer,  more  cellular,  and  vascular  layer  (tunica  propria), 
and  a  stratified  layer  of  epithelial  cells  (membrana  granulosa).  At  a 
certain  point,  about  the  ovum,  these  cells  are  heaped  up  into  the  discus 
proligerus. 

The  cavity  of  the  follicle  is  filled  with  fluid,  the  liquor  folliculi.  The 
ovum  possesses  an  outer  layer  of  hyaline  appearance,  the  zona  pellucida, 
presenting  radiating  strise;  a  nucleus,  and  a  germinal  spot. 

The  ovary  is  developed  from  the  Wolffian  body  by  the  ingrowth  of 
connective  tissue  into  a  mass  of  epithelial  cells  derived  from  the  ccelom. 
The  follicles  are  by  most  authorities  believed  to  be  derived  from  the 
downgrowth  and  subsequent  separation  of  the  superficial  mesothelial 
cells  of  the  body  cavity.  The  ovary  is  thus  entirely  mesoblastic.  There- 
fore, tumors  arising  from  it,  even  if  histologically  of  epithelial  or  carcino- 
matous  type,  are  from  the  point  of  view  of  development  to  be  classed 
with  the  sarcomas  (mesotheliomas). 

The  ovary  is  particularly  liable  to  circulatory  disturbances;  indeed, 
these  are  largely  physiological,  for  congestion  accompanies  the  functions 
of  ovulation  and  menstruation,  and  hemorrhage  takes  place  into  the 
follicles  after  the  ovum  is  discharged.  Neither  circulatory  disturbances 
nor  inflammations,  however,  are  of  so  much  importance,  either  clinically 
or  anatomically,  as  are  cysts  and  tumors,  which  form  a  large  proportion 
of  the  pathological  conditions  found  in  these  organs. 


CONGENITAL  ANOMALIES. 

Complete  absence  of  the  ovaries  is  rare  and  generally  associated  with 
absence  of  rudimentary  development  of  the  uterus.  Unilateral  defect, 
associated  with  the  condition  of  uterus  unicornis,  is  more  frequent.  In 
such  cases  the  kidney  on  the  same  side  is  sometimes  absent  or  dis- 
located, In  exceptional  instances  a  uterus  of  normal  type  may  be 


HYPEREMIA  849 

present.  The  condition  is  not  due  to  aplasia  then,  but  rather  to  some 
condition  that  exerts  traction  or  torsion  on  the  Fallopian  tube.  In  this 
way,  from  atrophy,  a  portion  of  the  tube  with  its  attendant  ovary  is 
completely  separated  from  the  uterus  and  in  time  disappears.  It  may, 
however,  become  attached  in  some  other  situation  or  form  a  free  body 
in  the  abdominal  cavity. 

Unilateral  and  bilateral  hypoplasia  is  described.  The  ovaries  may 
either  be  small,  with,  however,  normally  developed  follicles,  or  the 
follicles  may  be  rudimentary  or  absent.  In  bilateral  hypoplasia  the 
individual  often  presents  the  secondary  male  characteristics  of  develop- 
ment. 

Accessory  ovaries  have  been  observed.  They  are  usually  of  small 
size,  multiple,  and  situated  at  the  hilus  of  the  normal  ovary  at  the 
free  margin  of  the  peritoneum.  It  is  possible  that  they  are  not 
always  true  accessories,  for  some  hold  that  they  are  simply  portions  of 
the  main  ovary  that  have  been  pinched  off  through  peritoneal  adhesions. 
Winckel  reports  a  case  where  a  third  ovary  was  found  in  front  of  the 
uterus. 

The  ovary  may  occupy  an  abnormal  situation,  for  instance,  in  the 
canal  of  Nuck. 

DISLOCATIONS. 

Hernia. — Malpositions  of  the  ovaries  are  congenital  or  acquired. 
Hernia  of  the  ovary  (ovariocele)  is  usually  congenital  and  due  to  patency 
of  the  processus  vaginalis.  The  condition  is  often  bilateral.  The  ovary 
may  be  found  in  any  part  of  the  inguinal  canal  (hernia  ovarica  inyuinalis), 
even  to  the  labium  (hernia  ovarica  labialis).  The  acquired  form  is  usually 
met  with  during  confinement.  Hernia  ovarica  cruralis  is  also  acquired. 
Rare  varieties  are  the  hernia  ischiadica,  abdominalis  (into  the  scar  of  a 
Cesarean  section),  umbilicalis,  and  that  into  the  obturator  foramen. 
The  dislocated  ovary  is  often  congested  and  inflamed,  rarely,  cystic, 
carcinomatous,  or  sarcomatous. 

Prolapse. — The  ovary  may  also  be  prolapsed  when  for  any  reason 
the  uterus  is  dislocated.  Other  causes  are  increase  in  the  weight  of  the 
ovary,  as  from  congestion,  oedema,  cysts,  and  tumors,  in  some  instances 
combined  with  diminished  elasticity  of  the  ligaments  or  the  traction  of 
fibrous  bands.  The  prolapsed  organ  is  generally  swollen  and  congested. 


CIRCULATORY  DISTURBANCES. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia,  apart  from  the 
physiological  congestion  that  occurs  in  the  course  of  the  various  sexual 
functions,  coitus,  menstruation,  ovulation,  and  pregnancy,  is  of  little 
importance.  It  occurs  in  the  early  stages  of  inflammation. 

1  For  literature  see  Falk,  Berl.  klin.  Woch.,  44: 1891 : 1069. 
54 


850  THE  OVARIES 

Passive  Hyperemia. — Passive  hyperemia  may  be  a  part  of  a  general 
systemic  condition,  or  localized  to  the  ovary.  In  the  latter  case  it  is 
due  to  any  cause  that  interferes  with  the  proper  discharge  of  blood  through 
the  veins.  Among  these  may  be  mentioned,  torsion  of  the  pedicle, 
prolapse  of  the  ovary,  the  pressure  of  fibrous  adhesions,  and  the  presence 
of  cysts  or  tumors.  The  ovary  is  enlarged,  reddened,  and  oedematous, 
and  in  long-standing  cases  may  be  fibroid.  Phlebectasia  of  the  veins  of 
the  medulla  is  described  by  Kaufmann. 

Hemorrhage. — Hemorrhage  into  the  ovary  may  occur  in  any  of  the 
foregoing  forms  of  hyperemia,  and  in  the  infectious  fevers,  such  as 
typhoid,  diphtheria,  and  cholera,  in  the  hemorrhagic  diatheses,  in  phos- 
phorus poisoning,  and  in  severe  burns.  The  effusion  of  blood  may  be 
local  or  diffuse,  and  may  involve  the  stroma  (interstitial  hemorrhage) 
or  the  follicles  (follicular  hemorrhage). 

When  extravasation  takes  place  into  a  follicle,  the  follicle  is  enlarged, 
sometimes  to  the  size  of  the  fist.  The  blood  may  be  normal  in  appear- 
ance, clotted,  or  resembling  tar.  Frequently,  from  degeneration  and 
reactive  inflammation,  a  yellowish  zone  is  formed  at  the  periphery. 
The  distended  follicle  may  give  way  and  lead  to  fatal  bleeding  or  to 
retro-uterine  hematocele.  Hemorrhage  into  a  follicle  is  generally  due 
to  the  rupture  of  a  distended  vessel,  but  the  possibility  of  ovarian 
pregnancy  must  also  be  borne  in  mind.  Should  rupture  not  take 
place,  the  theca  becomes  thickened,  the  blood  is  gradually  absorbed, 
and  a  pigmented,  fibrous  scar  is  the  result.  Small  hemorrhages  are 
sometimes  seen  in  the  stroma,  usually  about  the  follicles.  These  may 
coalesce,  forming  large  extravasations,  or  the  hemorrhage  may  be  exten- 
sive from  the  first,  leading  to  a  diffuse  infiltration  of  the  organ.  In 
such  cases  the  ovary  is  greatly  enlarged  and  the  tissue  more  or  less 
destroyed,  so  that  it  resembles  a  sponge  filled  with  blood  (hematoma 
ovarii).  This  is  not  infrequent  in  children. 

Leukemia. — In  leukemia  the  vessels  of  the  ovaries  are  filled  with 
leukocytes  and  there  is  also  infiltration  of  the  stroma  with  white  cells, 
which  are  found  along  the  course  of  the  vessels  or  else  form  definite 
nodules. 

INFLAMMATIONS. 

Obphoritis. — Inflammation  of  the  ovary — oophoritis — is  usually 
secondary,  being  caused  by  the  extension  of  inflammation  from  the 
uterus,  tubes,  broad  ligaments,  or  peritoneum.  The  infection  may  be 
immediate  or  through  the  bloodvessels  and  lymphatics.  The  majority 
of  cases  arise  from  the  uterus  during  the  puerperium,  or  from  gonorrhoea. 
In  some  cases,  as  in  typhoid,  measles,  septicemia,  pneumonia,  influenza, 
diphtheria,  and  cholera,  the  infection  is  hematogenic.  In  rare  instances 
oophoritis  is  primary,  due  to  reactive  inflammation  about  areas  of 
degeneration  or  hemorrhage  in  the  ovary.  Among  the  germs  regarded 
as  exciting  causes  may  be  mentioned  the  Gonococcus,  Streptococcus, 
B.  coli,  Diplococcus  lanceolatus,  and  B.  typhi. 


OOPHORITIS  851 

According  to  the  portion  of  the  ovary  chiefly  affected,  we  can  divide 
cases  into  follicular  or  parenchymatous  and  interstitial.  Frequently, 
however,  the  whole  structure  is  involved.  Slavjansky1  has  described  a 
degenerative  form  of  follicular  oophoritis  occurring  in  the  acute  infec- 
tive diseases.  The  cells  of  the  membrana  granulosa  are  swollen,  cloudy, 
and  later  undergo  fatty  degeneration.  The  ovum  may  be  affected  in  a 
similar  way,  and  the  whole  follicle  may  be  destroyed,  so  that  the  cavity 
becomes  filled  with  a  whitish,  granular  detritus.  The  follicle  in  some 
cases  undergoes  cystic  change.  Some  extravasation  of  blood  may  be 
found  about  the  follicles,  especially  in  cholera.  In  more  severe  cases 
the  follicles  or  the  corpora  lutea  may  become  filled  with  pus  (suppura- 
tive  foil,  oophoritis).  Occasionally  the  infection  travels  along  the  lymph- 
channels  of  the  broad  ligament  to  the  ovary  (obph.  lymphangitica)  or 
takes  the  form  of  thrombophlebitis  (obph.  thrombophlebitica).  The 
ovary  is  enlarged,  softened,  and  infiltrated  with  inflammatory  products. 
When  suppuration  occurs,  which  frequently  happens,  one  can  make  out 
yellowish  streaks  running  from  the  hilus  to  the  cortex  along  the 
lymphatics  or  veins. 

It  is  by  no  means  always  possible  to  draw  a  hard  and  fast  line  between 
the  follicular  and  interstitial  forms  of  oophoritis,  for  in  many  cases  the 
inflammation  is  diffuse.  In  the  milder  grades  the  ovary  is  enlarged, 
reddened,  and  cedematous,  being  infiltrated  mainly  with  serum  (ooph. 
serosa).  In  other  cases  suppuration  occurs  (ooph  purulenta)  or  extra- 
vasation of  blood  (ooph.  hemorrhagica).  In  the  most  severe  forms  the 
entire  ovary  may  become  purulent  and  necrotic. 

The  suppurative  form  of  oophoritis  is  usually  due  to  the  Gonococcus 
or  to  septic  microorganisms  that  gain  an  entrance  after  parturition. 
In  such  cases,  as  a  rule,  the  infective  agents  spread  from  the  broad 
ligaments  or  from  the  peritoneum,  less  commonly  from  the  tube. 

The  results  of  suppurative  oophoritis  are  various.  Frequently  the 
inflammation  extends  outward  to  the  surface  of  the  ovary,  to  the  peri- 
toneum, or  tubes  (periobphoritis),  or  a  tuboovarian  abscess  may  form. 
A  local  pus  collection  may  burst  into  the  peritoneal  cavity,  setting  up 
a  serious  and  often  fatal  peritonitis,  or  may  burst  into  the  rectum, 
bladder,  more  rarely  into  the  vagina,  or  even  externally.  Should  the 
patient  survive,  the  abscess  may  become  encapsulated  and  partly 
absorbed.  The  ovary  in  such  cases  is  usually  tied  down  by  firm  ad- 
hesions. As  a  result  of  secretion  from  the  walls  of  the  abscess  the  cavity 
may  attain  a  considerable  size.  The  largest  abscesses  occur  where  a 
previously  existing  cyst  has  become  infected.  The  commonly  resulting 
perioophoritis  may  result  in  the  attachment  of  the  ovary  to  the  tube, 
the  uterus,  or  to  the  Douglas'  pouch.  Sterility  is  a  common  result  of 
oophoritis  and  salpingitis. 

Chronic  Oophoritis. — Chronic  oophoritis  is,  in  the  main,  due  to  the  same 
causes  as  the  acute  form.  Thus,  it  may  supervene  upon  the  subsidence 
of  an  acute  attack  or  after  repeated  relapses.  It  may  also  result  from 

1  Arch.  f.  Gyn.,  3:  1872:  183,  and  23: 1890. 


852  THE  OVARIES 

prolonged  or  repeated  congestion,  as  from  excessive  sexual  excitement 
or  venous  stasis.  The  main  characteristics  of  this  form  are  those  of  a 
productive  inflammation  affecting  the  interstitial  tissue,  though  the 
follicles  usually  show  degenerative  changes. 

The  ovary  is  possibly  at  first  somewhat  enlarged,  but  diminishes  in 
size  as  the  process  becomes  established.  The  surface  is  often  nodular 
but  may  be  even,  and  is  covered  with  bands  or  tags  of  adhesion.  The 
tunica  albuginea  is  thickened  and  of  a  pearly-white  or  grayish  appear- 
ance. On  section,  the  organ  is  cirrhotic  and  contains  numerous  small 
cysts,  due  to  the  dilatation  of  the  follicles. 

Microscopically,  in  the  early  stages,  there  is  a  small-celled  infiltration 
in  the  stroma,  principally  about  the  vessels.  Later  this  is  less  marked, 
and  fibrous  hyperplasia  predominates.  The  vessels  usually  show  hyaline 
thickening.  The  follicles  in  some  parts  may  be  normal,  but  many  of 
them  show  degeneration.  The  membrana  granulosa  is  cloudy  or  fattily 
degenerated  and  often  stripped  off  from  the  theca,  while  the  ovum  is 
destroyed.  In  other  cases  the  follicles  are  atrophic  and  represented  only 
by  corpora  fibrosa,  or  are  converted  into  cysts. 

Syphilis. — Gummas  analogous  to  those  found  in  syphilitic  orchitis 
have  been  observed  (Lancereaux1). 

Tuberculosis. — Tuberculosis  is  more  common  and  takes  the  form 
of  milia,  large  caseous  foci,  or  areas  of  colliquative  necrosis.  As  a  rule, 
the  disease  is  bilateral.  It  is  rarely  primary  in  the  ovary,  but  originates 
in  the  uterus,  tubes,  or  peritoneum.  Sometimes,  however,  the  ovaries 
are  alone  affected.  Occasionally  the  follicles  (Heiberg,  Schetlander) 
or  cysts  of  the  ovary  are  secondarily  infected.  A  tuboovarian  abscess 
may  be  formed  or  the  ovary  may  be  enveloped  in  a  caseofibroid  mass. 

Actinomycosis. — Actinomycosis  is  excessively  rare,  and  is  invariably 
secondary.  Small  abscesses  containing  the  actinomyces  "  grains"  are 
found  in  the  stroma. 

Parasites  and  Foreign  Bodies. — Echinococcus  disease  has  been  found 
in  the  ovary  (Schatz,  Pe*an),  and  in  a  dermoid  cyst  (Freund2). 

Needles  have  been  discovered  in  the  ovary,  having  reached  it  from 
the  uterus  or  bowel. 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Atrophy  of  the  ovary  occurs  as  a  senile  change,  or  as  the 
result  of  chronic  oophoritis.  The  senile  ovary  is  smaller  than  normal, 
firm,  nodular,  and  of  a  grayish  or  pearly  white  color.  The  albuginea 
is  hard  and  may  be  several  millimeters  thick.  The  follicles  are  in  all 
stages  of  atrophy  and  degeneration  and  for  the  most  part  are  converted 
into  minute  fibrous  nodules  (corpora  fibrosa)  with  marked  thickening 
of  the  theca.  The  arteries  show  hyaline  change  and  often  calcification. 

1  TraitS  hist,  et-prat.  de  la  syph.,  1874:  228. 
2Gyn.  Klin.,  1885. 


PROGRESSIVE  METAMORPHOSES  853 

Cloudy  and  fatty  degeneration  are  found  in  the  ovum  and  the  membrana 
granulosa  in  the  various  forms  of  atrophy,  as  well  as  hyaline  change 
in  the  vessels  and  connective  tissue. 


PROGRESSIVE  METAMORPHOSES. 

As  has  been  frequently  remarked  with  regard  to  other  organs,  so  with 
the  ovaries,  it  is  difficult  to  draw  a  hard  and  fast  line  between  develop- 
mental overgrowths  of  tissue  and  certain  forms  of  inflammation. 

An  increase  in  the  number  of  the  follicles  or  a  precocious  ripening 
of  the  same  is  met  with  in  young  children,  associated  with  precocious 
menstruation  and  puberty,  and  by  many  is  regarded  as  a  form  of  hyper- 
trophy (hyperplasia)  of  the  follicles.  In  a  certain  number  of  these  cases 
the  ovary  presents,  also,  a  number  of  small  cysts.  These,  as  Leo  Loeb 
has  shown,  may  originate  in  follicles  that  have  atrophied  prematurely 
(atresia  of  the  follicles).  This  atrophy  is  common  in  early  life.  In 
some  cases  of  uterine  fibroids  the  ovaries  are  hypertrophic,  showing 
not  only  cystic  dilatation  of  the  follicles,  but  also  round-celled  infiltra- 
tion, proliferative  changes  in  the  stroma,  and  hyaline  thickening  of 
the  vessels. 

Besides,  however,  the  cysts  just  mentioned,  there  are  certain  others 
that  must,  like  them,  be  differentiated  from  the  large,  developmental 
cysts  or  cystadenomas.  Such  are  the  small  cysts  that  are  commonly 
found  in  the  ovaries  in  cases  of  chronic  oophoritis,  perioophoritis,  and 
salpingitis,  originating  in  the  follicles  or  corpora  lutea.  These  are  to  be 
regarded  as  "retention"  cysts  (hydrops  follicularis).  Here  it  is  probable 
that  there  is  a  thickening  or  condensation  of  the  theca  or  tunica  albuginea, 
which  prevents  the  bursting  of  a  follicle  and  the  discharge  of  its  contents. 

Usually  there  are  more  cysts  than  one,  but  eventually  one  or  two 
predominate.  A  thin-walled  sac  is  produced,  reaching  in  size  from  that 
of  a  walnut  to  that  of  the  fist  or  of  a  man's  head.  The  smaller  cysts 
are  lined  by  cylindrical  epithelium,  but  in  the  larger  ones  this  is  more 
or  less  altered  from  pressure.  The  contained  fluid  is  usually  clear, 
transparent,  and  serous,  resembling  the  normal  liquor  folliculi,  but  may 
contain  blood,  degenerated  epithelium,  and  pigment.  In  the  larger 
cysts,  as  a  rule,  the  ovum  degenerates  and  disappears.  The  remaining 
stroma  of  the  ovary  presents  but  little  change,  except  that  in  the  case 
of  the  larger  cysts  it  becomes  fibroid  and  atrophied  from  pressure. 
Occasionally  the  wall  becomes  calcified.  Ovarian  cysts  of  this  type  are 
unilateral  or  bilateral. 

Analogous  to  follicular  hydrops,  are  the  retention  cysts  sometimes 
originating  in  the  corpora  lutea.  They  are  usually  single,  although  two 
or  more  may  be  found.  In  size  they  do  not  often  exceed  a  walnut,  but 
have  been  found  as  large  as  a  child's  head.  The  cyst  wall  is  composed 
of  a  loosely  attached  corrugated  membrane  of  reddish  or  reddish-yellow 
color,  containing  capillaries,  leukocytes,  and  pigmented  round  cells 
(lutein  cells).  The  cavities  contain  a  thin,  ropy  fluid  of  reddish  or 


854  THE  OVARIES 

yellowish  color,  containing  more  or  less  altered  blood.  There  is  no 
epithelial  lining  in  this  type. 

Tumors. — In  the  attempt  to  arrive  at  an  adequate  scientific  classifi- 
cation of  ovarian  tumors  we  are  beset  by  many  difficulties.  The  older 
writers  boldly  cut  the  Gordian  knot  by  dividing  them  into  cystic  and 
solid  growths.  This,  while  a  fairly  good  practical  division  from  a 
clinical  point  of  view,  must,  however,  be  regarded,  pathologically  speak- 
ing, as  unscientific  and  misleading,  inasmuch  as  under  the  term  "  cystic 
tumors"  are  grouped  not  only  the  true  "proliferation"  cysts,  or  cyst- 
adenomas,  but  also  "  retention"  cysts  which  are,  of  course,  not  properly 
neoplasms.  Further,  certain  growths  are  separated  that,  embryologically 
speaking,  should  be  classed  together.  Still,  when  all  is  said,  it  may  be 
questioned  whether  we  have  much  better  to  propose  for  it.  The  difficulty 
is  that  the  etiology  of  many  of  the  ovarian  growths  is  still  in  doubt. 
Thus,  with  the  important  class  of  the  cystomas,  leaving  out  of  the  account 
the  simple  retention-cysts,  and  confining  our  attention  to  the  develop- 
mental cysts  (cystadenomas) ,  we  know  that  certain  of  them  tend  to  be 
unilocular,  others  multilocular,  while  the  fluid  contents  of  cysts  that 
appear  in  the  main  to  be  the  same  vary  considerably,  being  at  one 
time  serous,  at  another  ropy  and  mucinous.  Other  cysts  again  are 
papillomatous.  These  striking  variations  suggest  differences  in  origin, 
but  what  these  are  we  are  not  able  to  say  positively. 

Theoretically,  it  is  possible  to  get  cystadenomas  developing  (1)  from 
the  follicles,  (2)  from  invagination  of  the  original  germinal  epithelium 
(Keimepithel),  (3)  from  remains  of  the  Wolffian  body,  and  (4)  from 
extra-ovarian  tissues.  The  ideal  classification  would  be  along  em- 
bryological  and  developmental  lines,  but  in  the  state  of  our  present 
knowledge,  or,  rather,  ignorance,  it  is  perhaps  better  to  fall  back  on 
morphological  differences,  at  least  in  the  main.  This  is  the  basis  of 
Pfannenstiel's  classification,  in  which  three  groups  are  recognized: 
(1)  The  parenchymatous  growths,  divided  into  (a)  those  derived  from 
the  epithelium  and  (6)  those  derived  from  the  ovum;  (2)  growths  arising 
from  the  stroma ;  and  (3)  mixed  types.  This  is  not  entirely  satisfactory, 
as  it  brings  dermoids  and  teratomas  into  the  same  class  as  the  cyst- 
adenomas,  with  which  they  have  little  in  common,  either  in  structure  or 
in  origin. 

K  We  would  suggest  the  following  classification,  not  as  having  finality 
or  being  scientifically  accurate,  but  as  having  at  least  the  advantage  of 
grouping  like  things  together  and  separating  unlike  ones. 


f  Benign. 


I.  New-growths  of  epithelial 

type 


f  Cystadenoma : 

1.  Arising  from  the  follicles. 

2.  From  the  corpus  luteum. 

3.  From  the  germinal  epithelium. 

4.  From  Wolffian  "rests." 

5.  From  remains  of  paroophoron. 
[      6.  From  tubal  "  rests." 


TVT  r  fl.  Cystic  carcinoma. 

Malignant. 


SoHd  carcinoma. 


CYSTADENOMA 


855 


II.  New-growths  of  connec- 
tive  tissue  type   .      . 


III.  Teratomas   . 


f  Benign. 


Malignant. 


IV.  Mixed  tumors. 


{1.  Fibroma. 
2.  Papilloma. 
3.  Hemangioma. 
4.  Lymphangioma. 

{1.  Endothelioma 
2.  Perithelioma. 
3.  Sarcoma. 

;1.  Epidermoid  cysts. 
2.  Pure  dermoid  cysts. 
3.  Compound  dermoids. 
[  4.  Compound  teratomas. 


Combined   and   cognate   forms: 
(I.  Myofibroma. 
Benign.          ->  2.  Adenofibroma. 
(3.  Cystic  fibroma. 


Malignant. 


fl.  Myosarcoma. 

i2.  Adenosarcoma. 
3.  Sarcocarcinoma. 
4.  Cystic  carcinoma. 
5.  Cystic  sarcoma. 


Teratogenous  blastemas: 

Benign.  1.  Cystadenoma  with  teratoma. 


Malignant. 


J  1.  Sarcoma  with  teratoma. 
I  2.  Carcinoma  with  teratoma. 


Cystadenoma. — Of  the  epithelial  growths,  the  most  important  are  the 
cystadenomas,  which,  indeed,  are  the  commonest  neoplasms  found  in 
the  ovaries.  They  may  be  unilateral  or  bilateral.  Nowadays  they 
rarely  reach  a  large  size,  inasmuch  as  they  are  usually  operated  upon 
somewhat  early,  but  in  former  times  cases  used  to  be  met  with  in  which 
the  weight  of  the  cyst  actually  exceeded  that  of  the  patient  affected. 
Several  varieties  are  recognized,  according  to  the  number  of  cysts,  the 
character  of  the  lining  epithelium,  and  the  nature  of  the  contents.  As 
the  main  type,  we  may  take  the  common  ovarian  cyst  or  simple  cystoma. 
This  is  generally  unilateral,  and  consists  of  one  main  cyst  of  proportion- 
ately large  size,  with  several  subsidiary  or  daughter  cysts.  The 
smaller  cysts  may  exist  more  or  less  independently  in  the  fibrous 
stroma  or  may  encroach  upon  the  cavity  of  the  major  cyst.  On  examin- 
ing the  inner  surface  of  the  wall,  one  can  generally  make  out  ridges  repre- 
senting the  remains  of  former  divisions  between  the  cysts.  Hence,  the 
major  cyst  is  evidently  developed  from  the  confluence  of  smaller  cysts. 
A  multilocular  cyst  may  thus  be  converted  into  a  unilocular  one.  The 
cyst-wall  is  often  tough,  thin,  and  translucent,  but  in  some  cases  thick. 
The  blood  supply  is  by  means  of  large  vessels  that  enter  in  the  pedicle 
and  ramify  over  the  surface.  The  fluid  found  within  the  various  cysts 
differs  somewhat  in  character,  being  thinner  in  the  larger  cavities. 
The  specific  gravity  varies  from  1010  to  1030.  It  is  often  viscid, 
mucinous,  or  stiff  like  honey.  In  color  it  may  be  clear  and  glassy,  in 
other  cases  turbid,  brownish,  or,  rarely,  tinged  with  blood.  The  char- 
acter of  the  fluid  is  due  to  the  presence  of  certain  bodies,  regarded  by 


856 


THE  OVARIES 


FIG.  222 


Scherer  and  Eichwald  as  paralbumin  and  metalbumin,  but  which, 
according  to  Hammerstein  and  Pfannenstiel,  are  more  nearly  related 
to  mucin.  They  term  them  pseudomucin. 

The  cyst-wall  is  composed  of  two  layers  of  fibrous  tissue,  an  outer 
firmer  and  more  fibrous,  an  inner  cellular  and  vascular.  The  lining 
membrane  of  the  cyst  is  usually  composed  of  a  single  layer  of  high 
cylindrical  cells.  In  the  larger  cysts  the  lining  cells  are  short,  columnar, 
cuboidal,  or  even  occasionally  flattened.  The  lining  epithelium  forms 

downward  evaginations,  so  that 
simple  or  compound  gland-tubules 
are  produced  in  the  wall  of  the 
cyst.  Some  of  these  may  become 
pinched  off  and  form  minute, 
intramural  cysts.  It  is  rare  for 
the  epithelium  to  be  stratified. 
Some  cysts  are  lined  with  ciliated 
cells,  either  wholly  or  in  part. 
In  many  cases  the  cells  of  the 
lining  epithelium  present  colloid 
change  and  discharge  their  con- 
tents into  the  cavity.  The  cells, 
also,  from  pressure,  frequently 
show  fatty  degeneration,  atrophy, 
and  necrosis. 

Microscopically,  the  fluid  con- 
tent contains  fat  globules,  leuko- 
cytes, degenerating  cells,  detritus, 
blood,  and  cholesterin.  In  many 
cases  the  typical  ovarian  struc- 
ture has  completely  disappeared, 
but  occasionally  some  more  or  less 
flattened  and  atrophied  remains 
can  still  be  made  out  containing 
active  follicles. 

A  second  but  rare  form  of  cyst- 
adenoma  is  a  pedunculated,  multi- 
locular  cyst  of  moderate  size, 
usually  unilateral,  lined  with 
ciliated  cylindrical  epithelium. 
The  cyst-contents  are  thin,  more  serous  than  in  the  last  form,  and 
light  yellow  or  greenish  in  color.  The  fluid  is  rich  in  albumin  and  con- 
tains no  pseudomucin.  The  cyst-wall  usually  contains  gland-tubules 
in  considerable  numbers,  especially  near  the  pedicle. 

A  third  and  important  type  is  the  papillary  cystoma  (cystadenoma 
papilliferuwi).  This  is  a  multilocular,  or  occasionally  unilocular,  cyst, 
and  is  liable  to  be  bilateral.  The  growth  may  extend  between  the 
layers  of  the  broad  ligament  or  form  a  pedunculated  mass  springing 
from  the  surface  of  the  ovary.  The  cysts  are  usually  smaller  than  in 


Ovarian  cyst.     (From  the  Gynecological  Clinic 
of  the  Montreal  General  Hospital.) 


CYSTADENOMA 


857 


the  case  of  the  simple  cystadenoma.  In  this  variety,  the  cavities 
are  more  or  less  completely  filled  with  warty,  villous,  or  tree-like 
excrescences  derived  from  the  proliferation  of  the  connective-tissue 
stroma  of  the  cyst-wall,  which  are  covered  with  ciliated  cylindrical  epi- 
thelium. In  some  few  cases,  the  cilia  are  absent  or  only  to  be  observed 
on  the  papillae.  The  stroma  is  composed  of  fibrous  tissue  containing 
numerous  bloodvessels.  It  may  show  mucinous  degeneration.  Excep- 
tionally, papillary  outgrowths  are  found  on  the  external  aspect  of  the 
tumor.  This  is  due,  for  the  most  part,  to  the  fact  that  the  cyst-wall, 


FIG.  223 


Cystadenoma.     Multilocular  ovarian  cyst.     (Dudley.) 

through  atrophy,  has  given  way  and  the  originally  intracystic  outgrowths 
appear  on  the  surface.  More  rarely,  there  is  a  true  invasion  or  infiltra- 
tion of  the  cyst-wall  by  the  papillae.  It  is  not  unusual  to  find  granules 
of  lime  (sand  bodies,  psammoma)  in  the  wall  and  in  the  papillae.  The 
fluid  contained  is  thin,  watery,  and  more  serous  than  in  the  simple  cyst- 
adenomas.  It  contains  little  or  no  pseudomucin.  The  color  is  often 
dark  like  coffee.  The  tumor  is  clinically  of  great  importance,  since 
in  time  it  invariably  develops  malignancy,  and  secondary  nodules  are 
found  scattered  over  the  peritoneal  membrane.  According  to  Pfannen- 


858 


THE  OVARIES 


stiel,  one-half  of  the  ovarian  papillary  cystadenomas   are  in   reality 
carcinomatous  from  the  start. 

With  regard  to  the  origin  of  these  cystic  growths  much  has  been  written 
and  much  remains  to  be  learned.  It  is  not  likely  that  they  are  all  derived 
from  the  same  elements.  Theoretically,  cystadenomas  may  arise  from 
the  epithelium  of  the  follicles,  from  the  corpus  luteum,  from  the  super- 
ficial germinal  epithelium,  from  certain  tubules  of  the  paroophoron 
(Waldeyer),  from  displaced  "rests"  of  the  ciliated  tubal  epithelium 
(Kassmann),from  remains  of  the  Wolffian  body(Kolliker's  Markstrange). 
Attempts  have  been  made  to  assign  a  particular  origin  to  the  cysts  accord- 
ing to  the  character  of  the  contained  fluid,  but  this  is  a  small  point  to 

FIG.  224 


Cystoma  papilliferum  of  the  ovary.     Zeiss  obj.  A,  without  ocular.     (From  the  collection  of. 

Dr.  A.  G.  Nicholls.) 

decide  upon,  since,  as  is  well  known,  the  fluid  varies  considerably  in 
different  parts  even  of  the  same  growth.  As  much  depends  upon  absorp- 
tion as  upon  secretion.  Again,  differentiation  has  been  made  on  the 
ground  of  the  presence  or  absence  of  papillary  outgrowths  and  of  ciliated 
epithelium.  As  Orth  points  out,  however,  it  is  difficult  to  draw  a 
hard  and  fast  line  between  the  simple  and  the  papillary  cystadenomas, 
inasmuch  as  all  sorts  of  transitional  forms  have  been  met  with.  It  has 
been  shown,  moreover,  that  under  certain  circumstances  non-ciliated  epi- 
thelium may  acquire  cilia,  and  in  man  the  existence  of  ciliated  germinal 
epithelium  has  been  proved.  This  being  the  case,  it  will  readily  be  seen 
how  difficult  it  is  to  come  to  any  satisfactory  conclusion  as  to  the  etiology 
of  these  cysts.  A  developmental  origin  for  many  of  them  is  supported 


CARCINOMA  859 

by  several  facts.  Cystadenomas  are  usually  met  with  during  the  period 
of  sexual  activity  and  not  infrequently  in  both  ovaries.  Again,  cases 
have  been  recorded  where  sisters,  or  mother  and  daughter,  have  been 
similarly  affected,  suggesting  an  hereditary  vitium.  Perhaps  even  more 
suggestive  is  the  not  uncommon  event  of  the  combination  of  a  cystadenoma 
with  a  dermoid.  In  some  multilocular  cysts  certain  of  the  cysts  are  lined 
with  cylindrical  epithelium  and  present  a  glandular  character,  while 
others  resemble  dermoids. 

The  origin  of  the  papilliferous  cystadenomas  has  been  variously 
referred  to  the  follicles,  the  germinal  epithelium,  Wolffian  "rests,"  or, 
bearing  in  mind  their  frequent  situation  between  the  layers  of  the  broad 
ligament,  the  parovarium.  Authorities  are  divided  whether  to  assign 
the  same  etiology  to  the  simple  cystadenomas  and  the  papillary  forms. 
Orth  is  inclined  to  attribute  the  majority  of  them  to  the  same  origin — 
the  germinal  epithelium. 

From  the  cystadenoma  there  is  a  natural  transition  to  the  carcinoma, 
for  the  epithelial  benign  tumors  of  the  ovary,  especially  the  cystic  forms, 
are  particularly  liable  to  undergo  malignant  metamorphosis.  This  is 
borne  out  by  the  fact  that,  like  the  cystadenoma,  carcinoma  of  the 
ovary  has  been  found  in  early  life,  even  before  the  age  of  puberty. 

Carcinoma. — Carcinomas  may  be  conveniently  divided  into  cystic  and 
solid  growths.  The  former  arise  commonly  in  the  simple  cystaden- 
omas, but  still  more  frequently  in  the  papillomatous  variety.  The  cystic 
carcinomas  in  general  resemble  their  non-malignant  prototypes,  but 
seldom  attain  such  a  large  size.  In  the  malignant  cystadenomas,  the 
walls  and  septa  are  found  to  be  infiltrated  with  nodules  that  histologically 
are  composed  of  masses  of  epithelial  cells.  In  many  places  the  lining 
epithelium  has  proliferated,  so  that  a  stratified  layer  of  cells  has  taken 
the  place  of  the  original  single  row.  The  fluid  contents  of  the  cysts 
are  clear,  or  cloudy  from  the  admixture  of  cells  and  blood.  The  material 
may  also  be  viscid  or  even  colloid.  In  the  latter  case  the  metastases  are 
also  colloid,  as  in  a  case  recently  under  our  observation. 

In  the  papillary  type  (cystadenoma  papilliferum  malignum)  there  is 
an  exuberant  growth  of  the  excrescences,  which  are  more  cellular  than 
usual,  and  the  septa  are  infiltrated  with  secondary  cancerous  nodules 
of  papillomatous  appearance.  In  many  cases  the  papillae  extend  through 
the  septa  and  appear  externally,  giving  rise  to  peritoneal  and  other 
metastases.  The  stroma  frequently  presents  mucinous  degeneration, 
and  occasionally  sarcomatous  transformation.  As  in  the  benign  form 
psammoma  bodies  may  be  found.  In  some  few  cases  it  has  been  thought 
that  simple  cystadenoma  and  cystic  carcinoma  have  arisen  independently 
in  the  same  ovary. 

The  solid  carcinomas  of  the  ovary  are  also  unilateral  or  bilateral. 
They  form  smooth  or  nodular  growths,  sometimes  attaining  the  size  of 
a  child's  head.  The  ovarian  tissue  is  frequently  diffusely  infiltrated 
and  destroyed,  or  the  main  mass  of  the  organ  may  be  pushed  to  one  side 
in  the  course  of  growth.  As  a  rule,  the  tumor  is  of  the  medullary  or 
scirrhous  type,  but  may  be  colloid.  A  curious  form  is  the  so-called 


860  THE  OVARIES 

superficial  papillary  carcinoma,  in  which  papillomatous  outgrowths 
develop  on  the  surface  of  an  otherwise  fairly  normal  non-cystic  ovary. 

Histologically,  carcinoma  is  made  up  of  cylindrical,  cuboidal,  or 
polymorphous  cells,  either  arranged  in  more  or  less  perfect  alveoli, 
or  forming  a  diffuse  infiltration,  and  not  infrequently  showing  mucinous 
change. 

The  mode  of  origin  of  the  solid  carcinomas  is  obscure.  They  are 
supposed  to  arise  from  any  of  the  following  sources:  the  superficial 
germinal  epithelium,  Pfluger's  tubules,  the  follicles,  the  corpus  luteum 
(Rokitansky),  or  remains  of  the  Wolffian  body. 

Ovarian  carcinomas  of  all  kinds  spread  readily  over  the  peritoneum, 
owing  to  grafting  in  the  Douglas'  pouch.  They  also  spread  to  the 
broad  ligaments  and  produce  distant  metastases  by  invasion  of  the 
lymphatics  and  bloodvessels.  Metastases  in  the  opposite  ovary  are 
recorded.  Secondary  growths  are  formed  in  the  tubes  and  ovaries 
either  by  local  implantation  or,  in  the  case  of  the  tubes,  by  direct 
infiltration  or  by  a  method  similar  to  the  transportation  of  the  normal 
ovum. 

Secondary  cancer  may  arise  by  the  extension  of  cancer  of  the  neigh- 
boring parts,  as  the  uterus  or  rectum.  The  primary  growth  may  also  be 
in  the  breast  or  stomach.  A  curious  fact  is,  that  in  some  cases  the  ovaries 
are  the  only  seat  of  secondary  growths,  and  this,  together  with  the  addi- 
tional fact  that  a  considerable  length  of  time  (some  years)  may  elapse 
between  the  occurrence  of  the  primary  tumor  and  the  appearance  of 
the  secondary  growths,  has  led  to  the  suspicion  that  some,  at  least,  of 
these  cases  may  not  be  examples  of  metastasis,  but  rather  of  multiple 
independent  neoplasms.  This  possibly  has  also  to  be  thought  of  in  those 
cases  where  in  primary  carcinoma  of  one  ovary  cancerous  nodules  have 
been  found  in  the  other.  It  is  not  always  easy  to  decide  whether  they 
are  independent  growths  or  not,  but  it  is  quite  probable  that  many  are 
so,  when  we  remember  the  decided  tendency  of  ovarian  growths,  benign 
as  well  as  malignant,  to  be  bilateral.1  Metastatic  deposits  originating 
in  distant  organs  develop  through  the  blood  stream,  or  in  cases  of 
abdominal  growths,  by  means  of  the  peritoneum. 

Of  the  connective-tissue  tumors,  the  only  ones  worthy  of  note  are  the 
fibroma  and  the  sarcoma. 

Fibromas  form  usually  diffuse  growths  leading  to  uniform  enlarge- 
ment of  the  ovary,  but  circumscribed  nodules  are  occasionally  formed. 
They  may  be  unilateral  or  bilateral,  and  may  attain  a  considerable  size. 
Multiple,  warty,  nodular,  or  papillary  fibromas  are  occasionally  met 
with,  arising  from  the  surface  of  the  ovary.  Occasionally  fibromas 
arise  from  the  corpora  lutea  from  overgrowth  of  the  theca  and  form 
tumors  the  size  of  a  walnut.  The  theca  is  thickened  and  thrown  into 
deep  folds,  while  the  centre  is  composed  of  loose  connective  tissue  of 
soft  consistence  and  grayish  or  grayish-brown  color.  Occasionally, 

^ee  Woolley,  Boston  Jour.  Med.  and  Surg.,  January  1,  1903:  1;  and  Nicholls 
Montreal  Med.  Jour.,  32;  1903:  326. 


SARCOMA  861 

these  growths  contain  cavities  filled  with  serous  fluid  and  altered  blood 
pigment.  Leo  Loeb1  has  recently  described  a  curious  fibrocystic  tumor, 
containing  numerous  cells  derived  from  the  lutein  tissue  occurring  in 
the  ovary  of  a  calf. 

Several  modifications  of  fibromatous  tumors  are  described,  such  as 
myofibroma,  fibrocystoma,  adenofibroma,  and  fibro-adenocystoma.  In  some 
cases  osteoid  tissue  is  formed  (fibroma  osteoides)  or  true  bone  (fibroma 
osseum).  It  is  doubtful,  however,  whether  true  osteoma  or  chondroma 
are  ever  found  in  the  ovaries.  The  bloodvessels  and  lymphatics  may 
be  abundant  and  greatly  dilated.  Suppuration,  gangrene,  and  calcifica- 
tion may  occur  in  fibromas. 

A  simple  hemangioma  has  been  met  with  by  Orth2  in  both  the  ovaries 
of  a  child,  associated  with  angiomas  in  the  skin  and  internal  organs. 

Lymphangioma  has  been  described  by  Leopold3  arid  others. 

The  malignant  growths  arising  from  the  stroma  are  endothelioma, 
perithelioma,  and  sarcoma.  Marchand4  was  the  first  to  describe  endo- 
theliomas,  or  tumors  arising  from  the  lining  membrane  of  bloodvessels 
and  lymphatics,  in  the  ovary.  Some  originate  in  the  lining  cells  of  the 
perivascular  lymphatics,  one  form  of  perithelioma.  These  tumors  vary 
considerably  in  appearance.  They  attain  a  considerable  size,  are  uni- 
lateral or  bilateral,  and  are  often  soft,  spongy,  and  friable.  Occasionally 
they  are  cystic. 

Histologically,  they  resemble  carcinoma  or  sarcoma,  or  one  of  the  many 
forms  of  mixed  growth.  One  may  see  masses  of  cuboidal,  cylindrical, 
or  polyhedral  cells,  arranged  in  bands  or  alveoli,  or  often  more  or  less 
definitely  enclosing  a  lumen,  which  may  contain  blood  or  lymph.  In 
these  masses  giant  cells  may  sometimes  be  seen.  Hyaline  degeneration 
often  occurs.  The  nests  of  cells  are  separated  by  fibrous  tissue,  occa- 
sionally presenting  a  myxomatous  appearance.  In  some  cases  the  fibrous 
tissue  penetrates  the  lumina  so  as  to  form  intracanalicular  papillomas. 
Amann  has  also  described  a  form  of  sarcoma  (perithelioma)  arising  from 
the  adventitia  of  the  vessels.  The  most  recent  writer,  Ribbert,  however, 
it  may  be  said,  doubts  the  existence  of  tumors  arising  from  the  endo- 
thelium,  and  sees  no  reason  for  separating  peritheliomas  from  other 
sarcomas.  It  should  be  remarked  in  this  connection  that  the  so-called 
endotheliomas  may  in  some  cases  arise  from  "rests"  derived  from  the 
mesothelial  lining  of  the  coelom,  a  possibility  that  does  not  seem  to 
have  suggested  itself  to  the  systematic  writers. 

Sarcoma. — Sarcomas  are  relatively  rare,  constituting,  according  to 
Schroder,  only  1.5  per  cent,  of  all  ovarian  tumors.  They  are  frequently 
bilateral,  but  also  unilateral,  and  may  be  found  in  quite  young  children. 
The  affected  ovary  may  be  uniformly  enlarged  or  present  a  nodular 
surface.  The  tumor  is  moderately  hard,  and  is  usually  covered  with  a 

1  Virchow's  Archiv,  166:  1901 :  157. 

2Lehrb.,  2:  1893:  572. 

3  Die  soliden  Geschwiilste  der  Ovarien.,  Arch.  f.  Gyn.,  6. 

<Beitr.  z.  Kenntn.  d.  Ovarialtumoren,  1879:  47. 


862  THE  OVARIES 

serosa-like  membrane.  The  growth  originates  in  a  single  focus  that 
gradually  enlarges  until  the  whole  ovary  is  involved.  Nodular  forms 
are,  however,  met  with. 

Histologically,  ovarian  sarcomas  are  spindle-celled  and  round-celled. 
Mixed  forms,  however,  occur,  with  or  without  giant  cells,  and  sometimes 
containing  much  fibrous  tissue — fibrosarcoma.  Myxomatous  change 
may  often  be  observed.  The  growth  is  usually  isolated,  although  peri- 
toneal adhesions  and  metastases  may  occur.  Metastasis  in  distant 
organs  is  rare,  and  is  found  chiefly  in  the  round-celled  variety.  In  all 
forms,  hyaline  and  fatty  degeneration,  necrosis,  hemorrhage,  and  throm- 
bosis may  be  seen.  Martin  and  Hamilton1  have  recorded  an  apparently 
unique  case  in  which  there  was  bilateral  mixed-celled  sarcoma  of  the 
ovaries,  with  fairly  generalized  sarcomatosis  and  purpura.  The  blood 
showed  a  marked  leukocytosis  of  lymphocytic  type.  The  metastases 
seem  in  the  main  to  have  formed  along  the  perivascular  lymphatics, 
and  spindle-celled  emboli  were  found  in  the  vessels  of  the  skin,  thus 
accounting  for  the  purpura. 

In  some  cases  the  sarcoma  cells  are  seen  to  be  grouped  about  the 
small  and  middle-sized  bloodvessels.  Von  Rosthorn  would  classify  this 
form  with  endothelial  peritheliomas  above  mentioned.  It  should  be 
noted  that  sarcomatous  change  may  be  found  secondarily  in  adenoma, 
cystadenoma,  myoma,  and  carcinoma.2 

Teratomas  (Embryo mata). — Under  the  term  "embryomata,"  Wilms 
includes  that  class  of  tumors  that  we  generally  call  dermoids  and  tera- 
tomas,  on  the  ground  that  they  contain,  more  or  less  abundantly, 
structures  similar  to  those  found  in  the  embryo.  These  tumors  are 
generally  cystic,  and  may  be  simple,  composed  of  structures  resembling 
skin  (hence  the  term  "dermoid"),  or  compound,  where  in  addition  to 
epidermal  tissues  there  are  others  derived  from  the  mesoblast  and  hypo- 
blast,  teeth,  bone,  cartilage,  muscle,  glands,  nerve  tissue,  and  mucous 
membrane. 

It  should  be  remarked,  however,  that  the  term  "dermoid,"  which  has 
had  such  extensive  vogue,  in  the  light  of  recent  researches  has  lost  much 
of  its  significance,  if,  indeed,  it  is  not  now  actually  misleading.  It  is 
commonly  held,  for  example,  that  the  dermoids  are  cysts  composed  of 
more  or  less  modified  skin  with  other  structures  of  epidermal  origin. 
Careful  study  has  shown,  however,  that  even  the  simplest  of  them 
contain  structures  from  the  other  primitive  germ  layers.  Consequently, 
the  distinction  between  "  dermoid  and  teratoma"  is  an  artificial  one, 
and  had  better  be  discontinued.  It  is  simpler  and  more  correct  to  class 
all  the  growths  of  this  kind  under  the  one  generic  term  "teratoma." 
If  the  word  "dermoid"  be  retained  at  all,  it  should  be  employed  simply 
for  convenience  of  description. 

Teratomas  are  rather  frequent  in  the  ovaries.  They  are  gener- 
ally unilateral,  sometimes  bilateral.  More  rarely,  multiple  separate 

1  Jour.  Exper.  Med.,  1:  1896:  595. 

2  Wilms,  Die  Mischgeschwiilste,  Leipzig,  1899  and  1902. 


DERMOIDS  863 

teratomas  are  found  in  one  or  both  ovaries.  These  tumors  are  of  slow 
growth,  remain  latent  for  a  long  time,  and  usually  give  rise  to  symptoms 
first  during  the  period  of  sexual  activity.  The  growths  are  in  the  vast 
majority  of  cases  cystic,  and  range  in  size  from  that  of  an  apple  to  a 
man's  head  or  larger.  They  are  usually  pedunculated  and  project  into 
the  peritoneal  cavity.  More  rarely,  they  extend  partially  between  the 
layers  of  the  broad  ligament.  We  may,  perhaps,  recognize,  with  Orth : 
1.  Epidermoid  cysts,  in  which  there  is  a  layer  of  stratified  and  keratinous 
squamous  epithelium,  lined  with  connective  tissue.  The  usual  structure 
of  the  skin  is  only  in  a  general  way  preserved.  The  surface  is  fissured 
and  scaly,  and  of  a  grayish-white,  often  mother-of-pearl,  appearance. 
The  cavity  contains  debris  of  keratin  of  a  firm,  friable,  soapy,  and 
cholesteatomatous  character. 

FIG.  225 


Interior  view  of  an  ovarian  teratoma  ("  dermoid  cyst  "),  showing  Rokitansky's  island 
bearing  c,  hairs  with  d,  teeth  surrounding.     (Schwalbe.) 

2.  Pure  dermoid  cysts.    These  are  the  most  frequent  type.     Here,  there 
is  an  epidermis  and  cutis  in  which  rudimentary  papillae  can  be  made  out. 
Hair-follicles,  sudoriparous  and  sebaceous  glands  are  very  numerous  in 
certain  parts.     Hair  is  present,  usually  in  considerable  quantities,  form- 
ing felted  masses  or  long  tresses  lying  free  in  the  cavity,  or  growing  from 
the  wall  in  tufts.     Besides  hair,  the  cavity  is  filled  with  a  yellowish, 
rather  granular,  fat  (sebum),  which  during  life  is  fluid,  together  with 
scales  of  epithelium,  debris,  and  cholesterin  crystals. 

3.  Compound  dermoids  (simple  teratomas).    In  addition  to  the  structures 
found  in  the  pure  dermoids,  there  is  the  formation  of  teeth  and  bone. 
The  bone  usually  forms  irregular  p  ates  in  the  wall  of  the  cyst,  but 
occasionally  from  its  shape  suggests  a  maxilla.     The  teeth  have  the  char- 
acteristics of  the  permanent  and  milk  teeth,  and  are  of  all  types.     At 


864  THE  OVARIES 

times  they  are  only  rudimentary.  In  number  they  may  greatly  exceed  the 
normal  complement  of  teeth.  From  one  to  three  hundred  have  been 
counted.  The  teeth  may  project  into  the  cavity  or  lie  embedded  in  the 
wall. 

4.  Compound  teratomas.  Here,  in  addition  to  hair,  bone,  cartilage, 
and  teeth,  are  found  more  complicated  structures,  such  as  mucous 
membrane,  mucous  and  salivary  glands,  smooth  and  striated  muscle, 
nerve-fibers,  brain-substance,  rudimentary  eyes,  fingers  with  nails, 
mammae,  ribs,  and  extremities. 

Teratomas  are  particularly  liable  to  complications.  The  pedicle  may 
be  twisted,  causing  anemia  and  necrosis.  Hemorrhages  may  occur  into 
the  substance  or  into  the  cavity.  The  cyst  often  becomes  inflamed  and 
suppurates.  It  is  common  to  find  it  more  or  less  bound  down  by  inflam- 
matory adhesions.  Peritonitis  may  result,  or  the  dermoid  perforate  into 
the  bladder,  rectum,  vagina,  or  through  the  abdominal  wall. 

Mixed  tumors  of  the  ovary  usually  constitute  merely  a  variation  of 
the  forms  hitherto  described.  They  may  be  divided  into  two  classes: 
(1)  those  in  which  two  distinct  types  of  tissue  develop  independently 
and  simultaneously  (mixed  tumors  proper) ,  and  (2)  those  in  which  we 
have  a  modification  or  transformation  occurring  secondarily  in  a  pre- 
viously existing  new-growth. 

In  the  first  class  of  the  benign  growths  may  be  mentioned  myofibroma, 
adeno fibroma,  cysto fibroma;  of  the  malignant,  myosarcoma  (?),  cystosar- 
coma,  cystadenosarcoma,  and  sarcocarcinoma.  In  the  second  group  we 
may  recognize  myxosarcoma,  cystadenoma  carcinomatodes,  cystadenoma 
sarcomatodes,  cystadenomatous,  carcinomatous,  or  sarcomatous  transforma- 
tion of  teratomas.  In  the  last  connection  it  may  be  mentioned  that 
Amann1  has  reported  a  melanotic  sarcoma  developing  in  an  ovarian 
dermoid. 

Owing  to  their  structure  and  position,  ovarian  tumors,  particularly 
the  cystadenomas,  are  liable  to  undergo  important  secondary  changes. 

Carcinomas  show  frequently  oedema,  fatty  or  mucinous  degeneration, 
hemorrhagic  infiltration,  or  necrosis.  Many  of  these  retrogressive 
changes  result  in  the  formation  of  pseudo-  or  degenerative  cysts.  When 
the  tumor  is  pedunculated,  torsion  leads  not  infrequently  to  strangula- 
tion of  the  bloodvessels  and  its  well-known  consequences.  The  growth 
becomes  swollen  and  cedematous,  the  veins  are  overdistended  with  blood, 
and  the  tissue  is  often  infarcted  and  necrotic.  In  such  cases  infection 
and  suppuration  readily  take  place.  Sometimes  the  pedicle  is  com- 
pletely severed  and  the  growth  lies  free  in  the  abdominal  cavity,  where 
it  will  necrose  unless  it  becomes  vascularized  through  new  adhesions 
forming -about  it. 

Peritonitis  is  also  not  uncommon.  Should  the  cyst- wall  rupture,  the 
contents,  if  unirritating,  may  be  absorbed,  but  if  infected,  set  up  septic 
peritonitis.  In  the  case  of  rupture  of  a  cystadenoma,  the  pseudo- 
mucinous  material  is  only  imperfectly  absorbed,  and  part  of  it  may 

1  Monatssch.  f.  Geb.  u.  Gyn.,  January,  1903. 


HEMORRHAGE  865 

become  encysted  through  the  formation  of  inflammatory  adhesions, 
giving  rise  to  a  structure  resembling  at  first  sight  a  tumor  (pseudo- 
myxoma). 

Hyaline  and  calcareous  degeneration  in  the  wall  have  also  been 
observed.  Secondary  tuberculous  infection  of  an  ovarian  cyst  has  been 
recorded. 


THE  UTERINE  LIGAMENTS:  THE  PELVIC  PERITONEUM 
AND  CONNECTIVE  TISSUE. 

CONGENITAL  ANOMALIES. 

In  cases  of  complete  defect  of  the  uterus  the  ligaments  are  also  absent. 
When,  however,  the  tubes  and  ovaries  are  present  the  round  ligament 
is  often  well-formed.  One  round  ligament  may  be  shorter  than  the 
other,  leading  to  dislocation  of  the  uterus.  Reduplication  of  the  round 
ligaments  has  been  observed.  A  comparatively  common  anomaly  is 
the  persistence,  either  partial  or  complete,  of  the  canal  of  Nuck,  causing 
hernia  and  hydrocele.  Remains  of  the  parovarium  and  its  attendant 
ducts  may  give  rise  to  disturbance. 

CIRCULATORY  DISTURBANCES. 

The  veins  of  the  round  and  broad  ligaments  and  those  in  the  neighbor- 
hood of  the  ovaries  may  be  dilated  and  tortuous  (varicocele).  Throm- 
bosis frequently  occurs,  with  the  formation  of  phleboliths. 

Hemorrhage. — Of  more  importance  is  hemorrhage,  which  may  be 
intraperitoneal  or  extraperitoneal.  This  usually  takes  place  between 
the  layers  of  the  broad  ligament  or  in  the  round  ligament.  The  most 
common  causes  are,  the  rupture  of  a  vessel  during  parturition,  more 
rarely  during  menstruation;  the  giving  way  of  a  varicose  vein;  and 
tubal  gestation.  The  extravasation  may  be  extensive  and  remain 
more  or  less  localized,  forming  a  tumor-like  mass  (hematoma).  In  other 
cases  it  infiltrates  the  connective  tissue  about  the  uterus,  bladder,  and 
rectum,  and  exceptionally  may  be  discharged  into  the  vagina  or  rectum. 
In  other  instances  the 'blood  is  effused  into  the  abdominal  cavity 
(hematocele).  The  usual  site  for  this  is  the  Douglas'  pouch  (hema- 
tocele  retro-uterina).  Rarely  it  is  into  the  uterovesical  pouch  (hematocele 
ante-uterina).  This  form,  when  present,  is  due  to  an  extensive  effusion 
or  to  the  fact  that  the  Douglas'  pouch  has  been  obliterated  by  inflam- 
matory products.  The  most  frequent  causes  are  ruptured  tubal 
gestation  and  hemorrhagic  peritonitis,  where  there  is  a  formation  of 
new  vessels.  Less  commonly,  it  may  be  due  to  the  rupture  of  a  vein 
in  the  broad  ligament,  a  ruptured  Graafian  follicle,  a  cyst  or  varicose 
vein  of  the  ovary,  hematosalpinx,  the  operative  ablation  of  an  ovary, 
55 


866  THE  PERIUTERINE  STRUCTURES 

and  the  discharge  of  blood  through  the  abdominal  ostia  of  the  tube. 
The  blood  is  not  necessarily  free  in  the  cavity,  for  it  may  happen  that 
it  is  extravasated  into  a  sac  formed  of  previously  existing  adhesions.  In 
many  cases,  from  reactive  inflammation,  a  fibrous  limiting  wall  is  pro- 
duced. Provided  the  patient  survive,  the  blood  may  be  absorbed  or  the 
resulting  clot  may  become  organized  after  the  fashion  of  a  thrombus. 
In  this  way  a  pigmented  mass  of  fibrous  tissue  may  be  produced.  In 
less  favorable  cases,  where  infection  has  occurred,  the  mass  may  be  con- 
verted into  an  abscess.  This  may  discharge  into  the  bowel  or,  rarely, 
into  the  abdominal  cavity,  bladder,  or  vagina.  In  this  way  fecal  fistulse 
are  produced  that  often  lead  to  extensive  disorganization  of  the  pelvic 
connective  tissue.  Some  few  cases,  after  the  discharge  of  the  pus, 
may  heal. 

INFLAMMATIONS. 

Inflammation  about  the  uterus  and  its  appendages,  both  in  the  over- 
lying serous  membrane  and  in  the  loose  connective  tissue  of  the  pelvis, 
is  not  uncommon.  According  to  the  localization  of  the  process  to  the 
serosa  covering  the  uterus,  tubes,  and  ovaries,  we  can  speak  of  a  peri- 
metritis,  perisalpingitis,  and  periobphoritis.  When  the  connective  tissue 
is  involved  the  condition  is  termed  parametritis. 

Parametritis. — In  simple  exudative  parametritis,  the  tissues  are  cede- 
matous  and  sodden.  No  clear  line  can,  however,  be  drawn  between 
this  and  the  suppurative  form,  where  the  tissue  is  diffusely  infiltrated  with 
pus  or  presents  abscess-formation.  In  very  severe  cases  the  structures 
become  gangrenous.  The  pus  may  remain  localized  or  may  burrow 
along  the  ligaments  to  the  abdominal  wall,  into  the  thigh,  into  the 
pelvis,  or  behind  the  rectum.  In  this  way  caries  of  the  bony  parts  may 
be  produced.  Perforation  may  occur  in  the  inguinal  region,  in  the 
ischiadic  fossa,  the  vagina,  rectum,  or  bladder.  Should  healing  take 
place,  the  abscess  becomes  encapsulated  and  more  or  less  absorbed,  or 
the  whole  of  the  affected  area  may  be  converted  into  a  dense  mass 
of  fibrous  tissue. 

The  commonest  causes  of  parametritis  are  injuries  during  parturition 
and  puerperal  sepsis.  The  inflammation  occasionally  spreads  from  the 
rectum.  An  actinomycotic  form  is  also  described. 

A  fibrinous  or  fibrinopurulent  inflammation  is  frequently  observed  in 
the  Douglas7  pouch  (pelvic  peritonitis).  It  is  due  to  the  extension  of 
inflammation  from  any  of  the  pelvic  organs,  and  is  met  with  also  in  carci- 
noma, tuberculosis,  and  ovarian  cysts.  Abundant  adhesions  may  form 
so  that  the  pus  is  completely  walled  off  from  the  abdominal  cavity  (retro 
uterine  pyocele).  As  in  the  case  of  suppurative  parametritis,  the  pus  may 
be  discharged  into  the  rectum,  vagina,  bladder,  the  general  peritoneal 
cavity,  or  externally.  The  causes  are  the  same  as  those  of  parametritis, 
except  that  here  gonorrhoea  plays  an  important  role.  The  process 
results  in  the  formation  of  dense,  fibrous  masses  or  adhesions  between  the 
various  parts  (productive  pelvic  peritonitis).  This  may  lead  to  the  occlu- 


CYSTS  867 

sion  of  the  tubes  and  dislocation  of  the  tubes  and  ovaries.  When  the 
adhesions  are  extensive,  pockets  may  be  formed,  filled  with  clear,  sero- 
purulent,  or  colloid-looking  fluid. 

Tuberculosis. — Tuberculosis  of  the  ligaments  is  always  secondary  to 
tuberculosis  of  the  Fallopian  tubes  or  general  peritoneum. 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy  of  the  round  ligaments  accompanies  atrophy  of  the  uterus, 
or  may  follow  parametritis  and  rapidly  recurring  pregnancies. 

After  the  menopause  the  vessels  of  the  broad  ligaments  frequently 
become  tortuous,  thickened,  and  calcareous. 


PROGRESSIVE  METAMORPHOSES. 

The  round  ligaments  are  often  hypertrophied  in  cases  of  hypertrophy 
of  the  uterus.  Hypertrophy  of  the  muscle  of  the  broad  ligaments 
occurs  in  connection  with  ovarian  and  parovarian  cysts. 

Tumors. — The  majority  of  the  tumors  found  in  the  broad  ligaments 
are  due  to  the  extension  of  growths  of  the  uterus  and  ovaries  between  the 
layers.  Subserous  myofibromas  of  the  uterus  sometimes  grow  out  into 
the  broad  ligament  and  may  be  eventually  detached  from  the  uterus. 
Lipomas,  leiomyomas,  and  myxofibromas  have  been  found  both  in  the 
broad  and  round  ligaments.  Cullen1  has  recorded  an  example  of  ade- 
nomyoma  of  the  round  ligament,  and  three  cases  have  been  described 
since  (Pfannenstiel,  v.  Heuff,  Blumer).  The  growth  is  composed  of 
non-striated  muscle  fibers,  together  with  glandular  elements  strongly 
resembling  the  glands  of  the  uterine  mucosa.  The  tumor  has  been 
found  also  within  the  groin  and  in  the  vagina.  It  is  thought  to  be 
derived  from  remains  of  the  Wolffian  body  (v.  Recklinghausen),  or 
possibly  from  the  Miillerian  duct. 

Cysts. — By  far  the  most  common  tumor-like  masses  in  the  uterine 
ligaments  are  the  cysts.  These  may  be  cysts  of  the  ovaries  that  have 
extended  into  the  broad  ligament,  cysts  derived  from  the  parovarium, 
or  very  rarely  from  the  paroophoron  between  the  parovarium  and  the 
uterus. 

Parovarian  cysts  vary  greatly  in  size,  but  may  be  as  large  as  a  man's 
head.  The  growth  lies  at  first  between  the  abdominal  end  of  the  tube 
and  the  ovary.  The  tube  may  to  some  extent  encircle  it,  but  in  the  case 
of  the  larger  growths  is,  of  course,  elongated,  more  or  less  flattened,  and 
atrophic.  The  ovary  is  also  much  flattened  so  that  it  may  be  difficult 
if  not  impossible  to  find  it.  As  the  cyst  develops  between  the  two  layers 
of  the  peritoneum,  it  is  covered  externally  by  loose  connective  tissue 
and  serosa,  which  can  usually  be  readily  stripped  off.  As  a  rule,  it 

1  Johns  Hopkins  Hosp.  Bull,  62-63:  1896:  112;  Ibid.,  87:  1898:  142, 


868 


THE  PERIUTERINE  STRUCTURES 


possesses  no  pedicle  and  the  wall  is  thin,  being  composed  of  connective 
tissue  and  more  or  less  hypertrophic  muscle  derived  from  the  muscle  of 
the  broad  ligament.  The  wall  internally  sometimes  presents  flattened 
or  papillary  outgrowths,  in  some  part,  at  least,  of  the  surface.  The 
contained  fluid  is  clear  and  watery  with  relatively  little  albumin  and 
few  cells.  Its  specific  gravity  is  1004  and  1005.  In  other  cases  it  is 
more  viscid  and  may  be  mixed  with  blood.  The  wall  is  lined  with  ciliated 
cylindrical  cells,  although  in  some  cases  the  cells  are  somewhat  flattened 
and  the  cilia  lost. 

FIG.  226 


Cyst  of  the  parovarium;  there  is  no  distortion  of  the  ovary;  the  Fallopian  tube  has  been 
much  elongated.     (Beyea.) 

Rarely,  dermoid  cysts  have  been  found  in  the  pelvic  connective  tissue, 
supposed  to  be  derived  from  embryonic  "rests." 

Primary  sarcoma  has  been  recorded  occasionally  in  the  broad  ligament 
and  on  the  surface  of  the  uterus  (Sanger). 

Carcinoma  is  always  secondary  and  generally  arises  by  extension  from 
the  cervix  uteri  or  ovary.  Occasionally  it  is  produced  by  lymphatic 
metastasis. 


CHAPTEE    XXXIX. 

THE  PUERPERAL  UTERUS  AND  THE  PRODUCTS  OF  CONCEPTION 

AFFECTIONS  OF  THE  PUERPERAL  UTERUS. 
CIRCULATORY  DISTURBANCES. 

DURING  the  act  of  parturition,  and  for  some  time  subsequent  to  it, 
a  certain  amount  of  blood  is  lost,  chiefly  from  the  placental  site.  Occa- 
sionally, this  passes  physiological  limits,  and  the  bleeding  may  be  so 
severe  as  to  endanger  the  life  of  the  individual  (postpartum  hemorrhage). 
Bleeding  also  takes  place  from  the  lacerations  of  the  cervix  normally 
produced  during  labor,  or  from  actual  rupture  of  the  corpus  uteri. 

Placental  remains  that  have  been  retained  within  the  uterus  give 
rise  frequently  to  menorrhagia  and  metrorrhagia.  Such  placental  tissue 
may  become  crusted  over  with  fibrin  and  form  large  polypoid  masses 
(fibrinous  placental  polyps).  A  noteworthy  form  of  metrorrhagia  is 
that  where  in  extra-uterine  gestation  the  decidua  is  cast  off  with  discharge 
of  blood. 

INJURIES.     (See  also  p.  826.) 

Lacerations  of  greater  or  less  extent  occur  commonly  in  the  cervix 
during  labor.  Rarely,  the  fundus  of  the  uterus  is  ruptured.  The  uterus 
may  be  perforated  by  instruments  during  operations  and  in  attempts 
at  criminal  abortion.  It  may  also  be  injured  by  contusions  of  the 
abdomen,  bullet  wounds,  and  the  horns  of  animals. 

INFLAMMATIONS. 

Inflammations  of  the  puerperal  uterus  may  be  purely  local,  but  fre- 
quently give  rise  to  systemic  manifestations  (puerperal  fever).  The 
endometrium,  the  muscular  wall,  or  the  serosa  may  be  involved  (endo- 
metritis  puerperalis,  metritis  puer.,  perimetritis  puer.). 

The  process  usually  begins,  as  one  would  expect,  at  the  point  of  primary 
injury,  such  as  the  cervix  and  the  placental  site,  and  may  extend  to  any 
part  of  the  uterus. 

Endometritis. — Two  main  types  of  puerperal  endometritis  are  to 
be  recognized,  the  putrid  and  the  septic.  The  former  is.  characterized  by 
the  decomposition  of  the  lining  membrane  of  the  uterus  with  the  pro- 


870  THE  PUERPERAL   UTERUS 

duction  of  foul  gases ;  the  latter,  by  various  forms  of  necrosis,  coagulation, 
or  suppuration.     Both  are  not  infrequently  combined. 

Putrid  Endometritis. — Putrid  endometritis  originates  in  retained  foetal 
products,  stagnated  blood,  pent-up  lochia,  or  sequestrating  portions  of 
the  uterus,  to  which  putrefactive  microorganisms  have  gained  access, 
either  directly  through  manual  or  instrumental  manipulation,  or  by 
extension  from  the  vagina  and  vulva. 

The  uterus  in  advanced  cases  is  enlarged,  the  wall  thickened  and 
oedematous,  and  in  parts  congested.  The  endometrium  is  converted 
into  a  dirty  green  or  brownish-black,  pulpy  mass  having  a  very 
offensive  odor.  The  process  may  extend  to  the  muscle  which,  in  time, 
becomes  soft  and  rotten  (putrid  metritis).  Physometra  may  be  pro- 
duced. 

Microscopically,  the  affected  tissue,  when  decay  is  advanced,  is 
cloudy  and  the  nuclei  stain  badly,  while  numerous  bacteria  of  many 
kinds  are  present.  At  the  periphery  the  necrotic  portion  is  bounded  by 
a  zone  of  inflammatory  leukocytes. 

The  process  leads  to  the  sequestration  of  uterine  tissue  and  sometimes 
to  the  formation  of  fistulse  between  the  uterus,  or  the  uterus  and  vagina, 
and  the  bladder.  Perforation  into  the  peritoneal  cavity  is  rare.  Masses 
of  clot  at  the  placental  site  may  be  involved  in  the  necrotic  process 
(putrid  thrombosinusitis).  When  a  strong  line  of  demarcation  is  formed, 
portions  of  the  endometrium  or  even  of  the  muscle  may  be  exfoliated 
(metritis  diseccans).  Complete  cure  may  result  with  the  formation  of 
scars  and  contraction. 

Septic  Endometritis. — Septic  endometritis.  in  its  simplest  expression, 
consists  in  superficial  suppuration,  which  is  particularly  liable  to  involve 
the  site  of  the  cervical  lacerations,  converting  them  into  discharging  ulcers. 
More  commonly  there  is  the  formation  of  a  grayish  adherent  membrane, 
perhaps  limited  to  the  eroded  surfaces,  although  at  times  it  may  extend 
over  the  greater  part  of  the  uterine  cavity.  When  affecting  the 
placental  site,  the  membrane  is  most  marked  on  the  top  of  the  promi- 
nences of  the  uneven  uterine  wall  and  may  be  slight  or  absent  in  the 
fissures.  It  can,  in  some  instances,  be  peeled  off,  but,  as  a  rule,  the 
necrotic  process  extends  some  little  distance  into  the  deeper  tissues. 
The  surface  of  the  affected  area  is  usually  dry  and  of  a  dirty  grayish-  or 
brownish-green  color. 

Microscopically,  the  superficial  layers  of  the  placental  site  show 
necrosis,  the  cells  staining  badly,  while  there  is  a  thick  network  of  fibrin 
often  presenting  a  certain  amount  of  hyaline  transformation.  Upon  the 
surface  is  an  exudate  of  similar  appearance.  Small  masses  of  leuko- 
cytes can  also  be  seen  both  in  the  membrane  and  in  the  underlying 
necrotic  tissue.  The  glands  show  more  or  less  erosion,  with  degenerative 
changes,  and  the  lumina  may  contain  fibrin.  Bounding  the  necrosing  area 
is  a  zone  of  inflammatory  leukocytes  with  great  hyperemia.  Small 
clusters  of  micrococci  can  generally  be  demonstrated.  The  process 
often  spreads  to  the  uterine  muscle,  and  cases  occur  where  the  wall  is 
affected  from  the  start  (septic  metritis). 


METRITIS  871 

Endometritis  Decidualis. — A  variety  of  inflammation  affecting  the 
lining  membrane  of  the  uterus,  that  should  be  mentioned,  is  that 
involving  the  decidua  (endometritis  decidualis).  This  rarely  occurs 
except  when  the  patient  is  suffering  from  some  infectious  disease, 
notably  cholera  or  measles.  As  a  rule,  it  is  traceable  to  preexisting 
endometritis.  The  process  may  occur  in  all  parts  of  the  decidua  and 
is  of  the  nature  of  a  productive  fibrosis.  The  decidua  is  thickened  and 
often  presents  nodular  or  polypoid  outgrowths  (endometritis  decidualis 
polyposci).  The  tissue  is  dense  and  firm  and  has  lost  the  normal 
yellowish-white  appearance.  In  some  cases  the  surface  may  be  covered 
with  pus.  Occasionally,  secretion  collects  between  the  deciduse  and 
gives  rise  to  discharge  (hydrorrhoea  gravidarum). 

Microscopically,  the  large  decidual  cells  are  increased  in  size  and 
numbers,  and  present  fatty  degeneration.  The  tissue  is  infiltrated  with 
round  cells  and  shows  marked  productive  change.  The  glands  in  the 
deeper  parts  are  rarely  increased  and  still  more  rarely  dilated.  In  the 
lower  strata,  the  lymph-channels  may  be  considerably  distended,  giving 
to  the  tissue  a  cavernous  structure.  Hemorrhage  readily  occurs  so  that 
abortion  is  frequent,  or  there  is  the  formation  of  a  blood  or  fleshy 
mole.  The  placenta  sometimes  also  becomes  adherent. 

Septic  Metritis. — Septic  metritis  may  be  diffuse  (m.  phlegmonosa), 
or  again  the  infective  process  may  extend  along  the  lymphatics  (m. 
lymphangitica)  or  veins  (m.  thrombophlebitica). 

Phlegmonous  Metritis. — In  phlegmonous  metritis,  the  uterine  muscle 
is  relaxed  soft,  swollen,  and  oedematous,  and  has  a  doughy  feel.  The 
interstices  of  the  muscle  contain  an  abundant  thin,  blood-stained  fluid, 
or  actual  pus.  The  condition  is  generally  best  marked  in  the  outer 
layers  of  the  wall. 

Microscopically,  there  is  a  more  or  less  abundant  accumulation  of 
leukocytes  in  the  interstitial  stroma,  which  is  also  cedematous.  The 
muscle  fibers  are  swollen,  cloudy,  and  vitreous.  Clusters  of  micrococci 
may  be  seen.  This  form  is  frequently  combined  with  lymphangitis. 

Lymphangitic  Metritis. — In  lymphangitic  metritis  one  can  recognize 
in  the  uterine  wall  and  adjacent  parts,  dilated  lymph-channels  con- 
taining detritus  and  pus.  On  cross-section,  the  lymphatics  appear  as 
cavities,  the  size  of  a  pea  or  larger,  filled  with  yellowish  material  com- 
posed of  fibrin,  pus  cells,  and  bacteria.  The  walls  of  the  lymphatics 
show  various  grades  of  degeneration  and  may  give  way,  so  that  irregular 
abscesses  are  formed  by  the  extension  of  the  infection.  These  abscesses 
are  often  very  numerous,  and  may  sometimes  be  seen  projecting  upon 
the  serous  surface.  This  form  of  metritis  is  liable  to  occur  in  cases  of 
infection  that  are  running  a  not  very  acute  course. 

Thrombophlebitic  Metritis. — Thrombophlebitic  metritis  is  somewhat 
rarer,  and  begins  generally  at  the  placental  site.  Atony  of  the  uterus 
predisposes  strongly  to  the  condition,  but  not  infrequently  membranous 
endometritis  is  present  as  well.  Large  thrombi  are  found  in  the  veins, 
presenting  all  grades  of  softening,  necrosis,  and  purulent  infiltration. 
The  appearances  are  analogous  to  those  in  lymphangitis  of  the  uterus. 


872  THE  PUERPERAL   UTERUS 

The  process  may  spread  to  the  para-uterine  veins  and  even  to  the  sper- 
matica  interna. 

In  all  forms  of  septic  metritis  the  process  may  extend  widely  from 
its  original  starting  point.  The  inflammation  may  reach  the  parametrium 
and  the  retroperitoneal  tissues.  Large  abscesses  in  the  connective  tissue 
may  result.  Lymphangitis  sometimes  extends  to  the  ovaries  and 
along  the  vertebral  column  to  the  diaphragm.  Thrombophlebitis  may 
extend  to  the  femoral  veins  and  the  inferior  vena  cava.  Peritonitis 
(perimetritis)  is  not  uncommon,  and  may  spread  to  the  pleura  and  peri- 
cardium. Septicemia  is  a  not  infrequent  sequel.  Ulcerative  endocarditis 
and  abscesses  in  the  various  viscera  have  been  observed. 

Puerperal  Perimetritis. — Puerperal  perimetritis  is  characterized  by 
the  formation  upon  the  serosa  of  the  uterus  of  an  exudate  that  is 
fibrinous,  fibrinopurulent,  or  purulent,  according  to  the  nature  and 
intensity  of  the  infection.  In  the  more  chronic  cases,  the  deposit 
becomes  gradually  organized,  adhesions  form,  and  the  exudation  may 
be  walled  off.  In  acute  cases,  general  peritonitis  may  occur.  When 
healing  takes  place,  bands  of  adhesions  may  dislocate  the  uterus  from 
its  normal  position. 

With  regard  to  the  etiology  of  puerperal  sepsis,  the  exciting  cause  is 
the  presence  of  septic  or  putrefactive  microorganisms.  The  most 
important  offenders,  in  order  of  frequency,  are,  the  Streptococcus 
pyogenes,  the  Staphylococcus  albus  and  aureus,  and  the  Diplococcus 
pneumonise. 

PROGRESSIVE  METAMORPHOSES. 

Tumors. — Pregnancy  may  of  course  occur  in  a  uterus  that  is  already 
the  site  of  tumor  growth.  Among  these  may  be  mentioned  myofibroma 
and  carcinoma,  but  the  only  tumor  that  need  specially  be  discussed 
here  is  a  very  remarkable  one — the  choriq-epithelioma  malignum — first 
described  by  Sanger. 

Many  differing  opinions  have  been  advanced  as  to  its  nature,  as  may 
be  gathered  from  the  variety  of  names  that  have  been  proposed  for 
it — -deciduoma  malignum  (Pfeiffer),  syncytioma  malignum,  deciduosar- 
coma,  chorio-epithelioma,  syncytial  carcinoma,  sarcoma  deciduo-chorio- 
cellulare.  It  is  now,  however,  practically  settled  that  it  is  a  new-growth 
originating  in  the  foetal  epiblast  of  the  chorionic  villi.  Consequently, 
being  a  tumor  of  fcetal  origin,  growing  in  the  tissues  of  another  indi- 
vidual— the  maternal  organism — it  should  be  classed  with  the  teratomas. 
Inasmuch  as  it  grows  rapidly,  infiltrates,  and  forms  metastases  in  distant 
parts,  it  is  of  malignant  character. 

The  tumor  only  develops  after  pregnancy.  It  may  occur  after  normal 
parturition,  after  abortion,  after  the  expulsion  of  a  hydatidiform  mole, 
and  in  extra-uterine  gestation.  The  growth  may  remain  latent  for  a 
considerable  time  after  delivery.  It  usually  begins  in  the  chorium 
frondrosum,  but  may  arise  from  any  part  of  the  uterus  to  which  chorionic 
villi  are  attached.  It  is  said  by  Schmorl  that  chorionic  villi  may  be 


CHORIO-EPITHELIOMA   MALIGNUM 


873 


detached  and  carried  to  distant  parts  where  the  cells  may  proliferate 
and  form  a  primary  chorio-epithelioma  extra-uterine  in  situation. 

Chorio-epithelioma  tends  to  form  polypoid  or  fungating  growths  pro- 
jecting into  the  uterine  cavity,  but  eventually  invades  the  muscle  beneath 
the  endometrium  and  may  infiltrate  more  or  less  deeply.  The  mass  is 
of  reddish  color,  frequently  hemorrhagic,  and  is  of  a  soft,  friable,  and 
spongy  nature. 

Microscopically,  according  to  Webster,1  three  types  are  to  be  differen- 
tiated: (1)  Where  the  primary  growth  and  metastases  are  of  sarco- 
matous  or  carcinomatous  type,  or  both;  (2)  where,  in  addition,  to  the 
appearances  just  mentioned,  syncytial  or  plasmodial  masses  may  be 
recognized;  and  (3)  where  in  addition  to  the  structures  of  the  second 
group,  there  are  cell-masses  resembling  placental  villi. 

FIG.  227 


''wSfr 


V<" 


Chorio-epithelioma  growing  within  uterus:  F,  wall  of  uterine  sinus;  Syn.,  multinucleate 
cells  of  syncytial  type;  L.c.,  cells  of  Langhans'  type.     (Teacher.) 

The  growth  is  seen  to  originate  in  the  proliferation  of  the  syncytium 
and  Langhans7  layer  of  the  chorionic  villi.  The  normal  relationships 
are  disturbed,  the  syncytium  is  thickened,  and  the  cells  of  the  Langhans' 
layer  tend  to  extend  through  to  the  surface.  The  superficial  area  is 
generally  necrotic  and  is  covered  with  a  deposit  of  fibrin.  The  deeper 
parts  present  an  alveolar  structure,  the  spaces  possessing  no  epithelial 
lining  and  containing  blood  and  fibrin.  The  resulting  tumor  has  no 
stroma  and  no  bloodvessels. 

The  so-called  syncytial  elements,  when  present,  are  large,  irregular, 
or  elongated  plasmodial  masses,  containing  numerous  deeply-staining 
nuclei.  The  protoplasm  is  finely  granular  and  may  contain  vacuoles. 
The  plasmodia  are  well  defined  but  their  protoplasm  may,  in  certain 
parts,  pass  imperceptibly  into  that  of  the  surrounding  tissue.  It  must 

1  Canadian  Practitioner,  22  :  1897  :  714. 


874  THE  PUERPERAL    UTERUS 

be  admitted  that  these  plasmodial  cells  are  not  necessarily  of  syncytial 
origin,  for  identical  appearances  are  to  be  found  in  other  malignant 
growths,  notably  sarcomas,  originating  in  other  parts  of  the  body. 

The  cells  derived  from  Langhans'  layer  form  groups  of  varying  size. 
They  are  irregular  in  shape,  long,  spindle,  or  spherical,  with  pale  proto- 
plasm. When  not  pressed  upon,  they  have  an  epithelial  appearance. 
The  nuclei  often  show  mitosis.  In  many  cases  the  cells  of  Langhans 
are  grouped  about  the  plasmodial  masses,  but  in  other  cases  the  latter 
are  in  excess  and  arranged  in  more  or  less  parallel  rows  with  their  long 
axes  at  right  angles  to  the  base  of  the  tumor.  At  the  periphery  of  the 
growth,  the  new-formed  cells  dissect  their  way  between  the  adventitia 


FIG.  228 


/ 


Chorio-epithelionfia  malign um.     Zeiss  obj.  DD,  without  ocular.     (From  the  collection  of 
the  Montreal  General  Hospital.) 

and  the  wall  of  the  bloodvessels,  compressing  the  lumina,  and  often 
invading  the  whole  thickness  of  the  vessel.  As  a  consequence  of  this, 
hemorrhage  into  the  growth  and  necrosis  are  common.  This  infiltration 
of  the  vessels  explains  the  great  tendency  of  the  tumor  to  form  metastases, 
which  may  be  found  in  the  vagina,  lungs,  and  occasionally  in  the  ovaries, 
liver,  and  spleen.  The  most  frequent  site  for  the  secondary  deposits  is 
the  vagina  which  is  involved  in  half  the  cases.  The  lining  of  the  uterus 
in  the  neighborhood  of  the  growth  takes  the  form  of  a  decidual  membrane 
or  a  normal  or  more  or  less  inflamed  endometrium. 

With  regard  to  the  ultimate  causation  of  this  extraordinary  form  of 
tumor,  it  may  be  mentioned  that  Leo  Loeb,  on  the  basis  of  experi- 
mental work,  holds  that  the  stimulus  to  the  formation  of  both  decidua. 


HEMORRHAGE  §75 

and  deciduomata  comes  from  a  certain  internal  secretion  of  the  ovary, 
possibly  in  connection  with  the  corpus  luteum.1 


THE  PRODUCTS  OF  CONCEPTION. 

Apart  from  the  decidua  that  has  just  been  referred  to,  these  are  the 
placenta,  the  cord,  the  amnion,  the  amniotic  fluid,  and  the  foetus. 

The  Placenta. 

An  abnormally  large  placenta  is  found  when  the  child  is  large.  The 
placenta  may  be  unusually  thin,  although  the  villi  may  be  hypertrophied. 
(placenta  membranacea).  Instead  of  one  placenta,  from  one  to  seven 
have  been  observed,  even  in  the  case  of  only  one  child  (p.  duplex, 
triplex,  etc.).  When,  in  addition  to  a  large  placenta,  several  subsidiary 
ones  are  met  with,  the  smaller  are  called  placentae  succenturiatae. 

An  otherwise  normal  placenta  when  in  an  abnormal  position,  that  is 
in  the  lower  uterine  segment,  is  called  placenta  prsevia.  Several  varieties 
of  this  may  be  recognized.  Placenta  prcevia  centralis  is  the  form  in  which 
the  centre  of  the  placenta  lies  over  the  internal  os.  In  partial  placenta 
prsevia  the  internal  os  is  covered,  but  the  margins  of  the  placenta  are  not 
equidistant  from  the  central  axis.  In  lateral  placenta  praevia  the  edge 
of  the  placenta  reaches  nearly  to  the  internal  os.  When  the  placenta 
projects  slightly  over  and  into  the  internal  os  it  is  termed  placenta  prcevia 
marginalis.  The  condition  is  not  common,  and  is  especially  rare  in 
primiparse.  Various  theories  have  been  advanced  to  account  for  the 
condition,  such  as  fructification  of  the  ovum  when  in  a  false  position, 
and  irregular  growth  and  vascularization  of  certain  parts  of  the  decidua, 
with  atrophy  of  others  (Hofmeier,2  Kaltenbach3). 

The  predisposing  cause  seems  to  be  endometritis.  Not  infrequently, 
myofibromas  of  the  uterus  are  present  and  the  placenta  usually  presents 
some  abnormality  also. 

Ischemia. — Ischemia  is  produced  by  the  obstruction  of  the  circu- 
lation in  the  umbilical  arteries,  and  in  those  cases  where  at  birth  the 
umbilical  vessels  have  been  severed  without  tying  them. 

Hyperemia. — Torsion,  looping,  compression  of  the  cord,  and  thick- 
ening of  the  umbilical  vein  lead  to  hyperemia. 

Hemorrhage. — By  far  the  most  important  circulatory  disturbance 
is  hemorrhage,  inasmuch  as  it  is  one  of  the  most  important  causes  of 
abortion.  Hemorrhage  may  occur  in  the  decidua  serotina,  or  in  the 
placenta  and  membranes  and  leads  to  more  or  less  complete  separation 
of  the  maternal  and  foetal  organisms. 

Hemorrhage  into  the  placenta  takes  the  form  of  dark  red  nodules  of 

1  The  Production  of  Deciduomata,  Journ.  Amer.  Med.  Assoc.,  50  :  1908  :  1897, 
p.  1897. 

2  D.  menschl.  Placenta,  1890. 

3  Zeitschr.  f.  Geb.  u.  Gynak.,  18:1890:1. 


876 


THE  PLACENTA 


coagulated  blood  in  the  intervillous  spaces  and  blood  sinuses.  It  comes 
on  suddenly  and  leads  to  compression  of  the  villi,  with  some  destruction 
of  tissue.  The  pigment  is  gradually  absorbed,  leaving  a  pale,  brownish 
or  brownish-yellow  area  which  finally  undergoes  organization,  with  the 
formation  of  a  fibrous  patch  (placental  infarcf). 

(Edema. — (Edema  of  the  placenta 'is  found  in  cases  of  hydramnios 
and  in  hydremia.  The  placenta  is  large,  pale,  juicy,  and  somewhat 
friable. 

Syphilis. — Syphilis  of  the  placenta  usually  takes  the  form  of  an  endo- 
or  peri-arteritis,  or  a  cellular  infiltration  of  the  villi.  Widespread  gum- 
matous  degeneration  is  unknown.  The  endarteritis  leads  to  obstruction 
of  the  circulation  with  infarction  and  fibrin  deposit  in  the  intervillous 
spaces.  The  lesion  resembles  that  of  simple  infarction,  with  the  addition 
that  there  is  thickening  of  the  villi  with  cellular  infiltration. 

Degenerations. — Hyaline  Degeneration  and  Necrosis. — Hyaline  degen- 
eration and  necrosis  occur  in  the  chorionic  villi  as  a  result  of  grave 
circulatory  disturbances,  such  as  infarct. 

Fatty  Degeneration. — Fatty  degeneration  is  also  largely  due  to  circu- 
latory disturbances. 

Calcification. — Calcification  often  follows  fatty  degeneration,  and  is 
found  in  the  villi.  It  is  met  with  early  in  syphilitic  embryos. 

Hypertrophy. — Hypertrophy  of  the  chorionic  villi  may  occur  as  a 
result  of  increased  demands  of  function,  or  in  association  with  inflam- 
mation. It  is  possible  that  in  cases  of  extensive  destruction  of  the 

chorion  certain  villi  undergo  compen- 
satory overgrowth.  A  form  of  hyper- 
trophy, or  rather  hyperplasia,  is  found 
in  cases  of  abortion,  and  also  after 
normal  labor,  where  portions  of  the 
placenta  or  chorion  are  retained  and 
take  on  overgrowth,  leading  to  the 
formation  of  the  so-called  placental 
polyps.  Malignant  transformation 
may  take  place  in  such  cases  (chorio- 
epithelioma  malignum). 

A  peculiar  and  striking  manifesta- 
tion allied  to  hypertrophy  is  the  cystic 
or  hydatidif  orm  mole  (myxoma  chorii— 
Virchow).  Here,  in  addition  to  hyper- 
trophy of  the  villi,  there  is  cystic 
metamorphoses.  The  villi  become  ex- 
traordinarily lengthened  and  branched, 
and  are  converted  into  numbers  of 
round,  oval,  or  elongated  vesicles 
containing  a  clear,  viscid,  or  slightly 
blood-stained  fluid.  The  appearance 
produced  bears  a  general  resemblance 
to  a  bunch  of  grapes.  The  condition 


FIG.  229 


A  small  portion  of  an  hydatidiform  mole 
natural  size. 


PLACENTAL  MOLES  377 

is  due  to  the  infiltration  of  the  enlarged  chorionic  villi  with  abundant 
gelatinous  fluid  not  unlike  the  Wharton's  jelly.  The  extent  of  the  change 
varies.  In  very  young  embryos  the  whole  chorion  may  be  affected,  but 
it  is  more  common  for  the  placenta  alone  to  be  involved.  On  liberating 
the  fluid,  a  delicate  meshwork  or  supporting  stroma  is  disclosed. 

Microscopically,  one  finds  a  delicate,  connective-tissue  framework 
with  but  few  cells,  covered  by  an  epithelial  layer.  Sometimes  the 
growth  is  very  vascular. 

It  is  an  important  practical  point  that  these  moles  occasionally  take  on 
malignant  action  and  invade  the  wall  of  the  uterus.  Neumann1  has 
reported  eight  cases  of  hydatidiform  mole  in  three  of  which  chorio- 
epithelioma  subsequently  developed.  Pestalozza2  has  also  met  with  a 
case  where  one  of  these  moles  eroded  into  the  vessels  and  gave  rise  to 
numerous  metastases. 

Apart  from  the  ordinary  mole  of  the  placenta  just  described,  Virchow 
recognized  a  form  in  which  the  nodules  present  a  connective-tissue 
character,  rather  than  the  usual  myxomatous  appearance  (myxoma 
fibrosum).  Increase  in  the  embryonic  connective  tissue  that  normally 
is  present  between  the  chorion  and  the  amnion  gives  rise  to  the  so- 
called  diffuse  myxoma  of  the  chorion. 

Tumors. — Fibroma,  fibromyoma  (Alin),  and  angioma  have  been 
described.  The  occurrence  of  true  sarcoma  is  not  proved.  The  sar- 
comas of  the  placenta  described  by  Hyrtl  and  Waldeyer  are  probably 
to  be  classified  as  chorio-epitheliomas. 

Small  cysts  of  doubtful  origin  are  occasionally  seen  in  the  placenta. 


The  Cord. 

The  cord  may  be  abnormally  long  or  short.  In  the  first  case  it  may 
be  encircled  about  the  neck  of  the  foetus  and  cause  strangulation;  in  the 
latter  it  may  be  a  hindrance  to  birth  and  be  torn  off  violently. 

The  cord  may  be  inserted  eccentrically  in  the  placenta  (insertio 
marginalis),  or  the  umbilical  vessels  pass  first  to  the  membranes  before 
reaching  the  placenta  (insertio  velamentosa).  The  cord  may  also  divide 
into  several  trunks.  The  omphalomesenteric  duct  at  the  attachment 
of  the  cord  may  be  imperfectly  closed  leading  to  hernial  protrusion  of 
the  intestines  or  other  organs  (hernia  of  the  cord). 

The  cord  may  be  twisted  or  looped  about  the  neck,  trunk,  extremities, 
or  breech  of  the  foetus  (Fig.  230).  In  the  earlier  stages  of  the  develop- 
ment it  may  cause  amputation  of  certain  parts. 

Thrombosis  of  the  umbilical  vein  and  hemorrhage  into  the  cord  has 
been  described. 

Fatty  degeneration  and  calcification  are  occasionally  observed. 

1  Ueber  Blasenmole  u.  malignes  Deciduom.  Verhandl.  d.  deutschen  Gesellsch.  f . 
Gynak.,  1897:304. 

2 II  Morgagni,  October,  1891. 


878 


THE  FCETUS 


FIG.  230 


Septic  infection  of  the  foetal  end  of 
the  cord  is  sometimes  met  with,  lead- 
ing to  arteritis  and  phlebitis. 

Periarteritis  and  endarteritis,  with 
cellular  infiltration  and  fibrous  in- 
duration, may  occur.  The  most  fre- 
quent cause  is  syphilis. 

Chorionic  and  allantoic  cysts  are 
found  at  both  the  foetal  and  placental 
ends  of  the  cord. 

Myxoma  and  angioma  are  met  with, 
but  rarely. 

The  Amnion. 

The  amnion  may  be  imperfectly 
formed  and  fuse  with  the  foetus  in 
certain  regions.  It  may  thus  form 
bands  and  adhesions.  These  are 
important,  inasmuch  as  they  may 
produce  arrest  of  development  and 
malformation  in  the  affected  parts, 
or  again,  by  obstructing  the  circula- 
tion may  produce  giant  growth.1 

Little  is  known  with  regard  to 
the  inflammatory  affections  of  the 
amnion.  Amnionitis  (Ahlfield)  is 
mentioned,  as  well  as  periarteritis 
and  endarteritis. 

The  Amniotic  Fluid. 


Girl,  aged  ten  years,  showing  cicatricial 
grooves  due  to  constriction  of  umbilical 
cord.  At  birth,  according  to  the  mother, 
the  grooves  in  the  abdominal  wall  and 
left  thigh  were  occupied  by  the  cord. 
(Hawthorne.) 


The  amniotic  fluid  is  sometimes 
increased  in  amount  (hydramnios, 
polyhydramnios),  or  it  may  be  di- 
minished (oligohydramnios).  From 
contamination  with  meconium  or 

putrefactive  germs,  the  fluid  may  become  foul,  cloudy,  discolored,  and 
full  of  bubbles  of  gas.  The  amniotic  fluid  also  becomes  altered  in  cases 
of  maceration  of  the  foetus,  although  generally  without  putrefaction. 
Various  drugs  administered  to  the  mother  may  appear  in  the  fluid. 

The  Foetus. 

Death  of  the  foetus  may  arise  from  a  variety  of  causes  and  leads  to 
several  curious  results.     If  it  be  not  discharged  prematurely  (abortion), 


1  Klaussner,  Ueber   Missbildungen  der   menschlichen  Gliedmassen,   Wiesbaden, 
Bergman,  1905, 


ECTOPIC  GESTATION  879 

it  may  be  retained  within  the  uterus  for  weeks  or  months,  leading  to 
atrophy  and  necrotic  changes  in  the  various  structures.  In  some  cases 
the  foetus  becomes  macerated  or  undergoes  a  form  of  mummification. 
Should  pressure  be  exerted  upon  it,  it  may  be  flattened  out  to  the  thin- 
ness of  paper  (foetus  papyraceus).  Rarely,  calcification  takes  place 
(lithopedion).  In  the  event  of  microorganisms  gaining  access  to  the 
uterus,  putrefaction  of  its  contents  and  sepsis  may  occur. 

The  most  potent  cause  of  death  of  the  fcetus  is  syphilis,  either 
by  producing  a  generalized  weakness  of  the  system,  or  by  cutting  off 
the  blood  supply  through  endarteritis  of  the  umbilical  vessels.  In  a 
macerated  fcetus  it  is  often  possible  to  find  evidences  of  syphilis  at  the 
epiphyses  of  the  bones  and  in  the  viscera.  Other  causes  that  should 
be  mentioned  are  pathological  changes  in  the  membranes,  placenta, 
or  cord,  and  constitutional  disease  in  the  mother  (Bright's  disease; 
eclampsia). 

Malformations. — Malformations,  apart  from  developmental  anom- 
alies, may  arise  from  mechanical  causes.  Such  are :  too  small  an  amount 
of  the  amniotic  fluid,  adhesion  of  the  membranes,  and  the  pressure 
of  bands  traversing  the  amniotic  sac.  (See  vol.  i,  p.  193  et  seq.) 

The  developmental  anomalies  are  of  great  interest.  Such  conditions 
as  dwarfism.  gigantism,  chondrodystrophia,  micromelia,  osteopsathy- 
rosis  are  discussed  elsewhere  (p.  1005  et  seq.). 

Ectopic  Gestation. — When  the  embryo  develops  in  an  abnormal 
situation,  the  condition  is  called  ectopic  gestation.  Of  this  there  are 
several  varieties. 

Intra-uterine  Ectopic  Gestation.  —  Gestation  may  occur  within  the 
cavity  of  the  uterus  but  in  an  abnormal  position  (intra-uterine  ectopic 
gestation).  Thus,  the  embryo  may  lie  in  a  rudimentary  horn  of  the 
uterus  or  in  a  diverticulum  from  its  wall.  The  embryo  has  also  been 
found  in  the  lower  uterine  segment,  in  the  cervical  canal,  and  even  in 
the  vagina.  The  latter  cases  are,  however,  more  correctly  to  be  regarded 
as  forms  of  abortion  than  of  ectopic  gestation. 

Interstitial  Ectopic  Gestation. — A  second  form  is  interstitial  ectopic 
gestation.  Here  in  most  cases  pregnancy  occurs  in  the  uterine  portion 
of  a  Fallopian  tube  (interstitial  tubal  gestation). 

Extra-uterine  Gestation. — The  third,  and  most  frequent  form,  is  extra- 
uterine  gestation.  This  takes  place  in  the  free  portion  of  the  tube, 
as  a  rule  about  the  middle.  In  these  cases  it  generally  happens  that 
some  time  between  the  second  and  fifth  month  the  tube  ruptures  and  the 
foetus  is  extruded  into  the  peritoneal  cavity.  Very  rarely,  tubal  preg- 
nancy may  go  on  to  full  term.  This  occurs  most  commonly  when  the 
embryo  grows  out  between  the  layers  of  the  broad  ligament  (intraliga- 
mentou-s  tubal  gestation).  Occasionally,  pregnancy  occurs  in  the  in- 
fundibulum,  and  the  embryo  may  project  into  the  abdominal  cavity 
(tubo-abdominal  gestation).  In  this  case  discharge  of  the  fcetus  into  the 
abdominal  cavity  readily  takes  place  (tubal  abortion).  In  those  cases 
where  the  fimbrise  closely  embrace  the  ovary,  so  that  it  lies  in  close  con- 
tact with  the  abdominal  opening  of  the  tube,  or  where  a  tuboovarian  cyst 


880 


THE  FCETUS 


has  existed  into  which  a  ripe  follicle  has  ruptured,  we  may  speak  of  a 
tuboovarian  gestation. 


FIG.  231 


Tubal  ectopic  gestetion.  The  uterus  and  vagina  are  opened  from  the  front.  The  right 
Fallopian  tube  is  greatly  distended  and  is  ruptured.  The  foetus  and  cord  are  also  shown. 
(From  the  Gynecological  Clinic  of  the  Montreal  General  Hospital.) 

Ovarian  Gestation. — Ovarian  gestation  is  excessively  rare,  but  undoubt- 
edly occurs.  The  first  case  was  reported  by  Katherine  van  Tussenbroeck 
at  the  Third  International  Congress  of  Gynecology  and  Obstetrics  at 
Amsterdam  (1899).  Others  have  been  described  since  by  Thompson 
and  Clarence  Webster,1  True  ovarian  gestation  may  occur,  or  the  ovario- 
abdominal  form. 

Abdominal  Gestation. — The  last  variety  of  extra-uterine  gestation  is  the 
abdominal.  It  is  open  to  question  whether  a  pure  form  of  abdominal 
gestation  exists,  that  is,  where  an  ovum  has  been  discharged  into  the 
abdominal  cavity  and  has  been  fertilized,  either  while  within  the  ovary 
or  after  its  liberation.  Possibly,  a  more  critical  analysis  of  the  cases 
reported  would  show  that  they  were  primarily  tubal  or  ovarian. 
Gutierrez2  has;  however,  recently  reported  a  case  that  is  almost  con- 
vincing. He  found,  in  a  woman  aged  thirty-four  years,  a  mature  fcetus 
in  a  sac  within  the  abdomen  attached  to  the  great  omentum  and 
parietal  peritoneum.  The  placenta  was  inserted  on  the  great  omentum. 

1  Amer.  Journ.  of  Obstetrics,  50: 1904:28. 

2  Re* vista  Ibero-Americana  de  Ciencias  Medicas,  March,  1904. 


EC  TOPIC  GESTATION  881 

Cases  of  what  may  be  termed  secondary  abdominal  pregnancy  occur, 
where  through  rupture  of  the  sac  the  foetus  is  discharged  into  the 
abdominal  cavity.  So  long  as  the  placenta  remains  in  situ  and  com- 
munication is  kept  up  with  the  mother,  development  is  possible. 

In  ectopic  gestation  the  uterus  undergoes  changes  in  kind,  although 
not  in  degree,  strictly  comparable  to  those  occurring  under  normal  condi- 
tions. The  muscle  hypertrophies,  a  decidual  membrane  is  formed,  and 
the  cervix  may  partially  dilate.  The  decidua  in  time  may  be  cast  off. 

Apart  from  those  rare  cases  of  interstitial  pregnancy  where  delivery 
takes  place  per  vias  naturales,  the  foetus  invariably  dies.  When  small, 
the  products  of  conception  may  be  completely  absorbed,  but  usually 
this  is  not  possible,  and  the  foetus,  when  discharged  into  the  abdominal 
cavity,  becomes  encapsulated  and  degenerates.  Mummification,  macer- 
ation, or  calcareous  infiltration  may  occur.  Occasionally,  the  membranes 
alone  become  calcified  (lithokelyphos).  With  regard  to  the  effects 
upon  the  mother,  tubal  gestation  is  the  most  dangerous,  as  rupture  of 
the  tube  almost  invariably  takes  place  after  the  earlier  months,  and  may 
lead  to  sudden  and  fatal  hemorrhage.  Blood  may  be  effused  freely  into 
the  abdominal  cavity  (hematocele)  or  may  become  more  or  less  encapsu- 
lated owing  to  reactive  peritonitis  (hematoma). 

The  causes  of  extra-uterine  gestation  are  various.  The  most  potent 
are  all  conditions  that  interfere  with  the  natural  passage  of  the  ovum 
down  the  Fallopian  tube  to  the  uterus.  Fructification  may  occur  in  a 
tube  and  the  ovum  be  retained  owing  to  salpingitis,  kinks,  tubal  polyps, 
compression,  diverticula,  or  defects  in  the  ciliated  epithelium.  Among 
inflammatory  causes,  gonorrhoeal  salpingitis  plays  an  important  part. 


56 


CHAPTEK    XL. 

THE  MAMMARY  GLAND. 

FROM  the  point  of  view  of  development  and  anatomical  structure 
there  is  no  essential  difference  between  the  male  and  female  breast. 
In  the  male,  however,  with  rare  exceptions,  the  organ  remains  rudimen- 
tary and,  therefore,  functionless  throughout  life.  In  the  female,  with  the 
onset  of  puberty,  certain  developmental  forces  are  set  in  motion,  looking 
toward  the  preparation  of  the  breast  for  its  important  function  of  lacta- 
tion, that  result  in  a  peculiarity  of  type  which,  among  other  things,  dif- 
ferentiates the  one  sex  from  the  other.  The  breast  in  the  case  of  both 
sexes  is  susceptible  to  the  same  diseases,  with  the  qualification  that  owing 
to  the  greater  functional  activity  in  the  female  the  organ  is  much  more 
frequently  involved  than  in  the  male.  Particularly  is  this  true  of  in- 
flammation. 

The  breast  is  formed  by  a  downward  proliferation  of  the  epidermis, 
according  to  Minot,  from  the  sudoriparous  glands;  according  to  others, 
from  the  sebaceous  glands.  At  birth  the  organ  is  at  most  2  cm.  across 
by  1  cm.  thick.  The  glandular  portion  consists  in  from  ten  to  twenty 
ducts,  arranged  radially,  which  discharge  in  a  small  depression  in  the 
nipple.  These  ducts  are  lined  by  cylindrical  epithelium  or  stratified 
pavement  cells,  and  end  in  club-like  enlargements.  During  the  first  few 
weeks  after  birth  the  proliferative  activity  may  be  so  great  that  the 
ducts  become  distended  with  masses  of  epithelial  cells  and  granular 
detritus.  The  breast  becomes  enlarged  and  possibly  somewhat  painful. 
Frequently  a  milk-like  fluid  can  be  expressed,  to  which,  in  some  countries, 
the  popular  name  of  "witch's  milk"  is  given.  The  dilatation  may 
in  some  cases  be  so  extreme  that  a  cavernous  structure  is  produced 
(Kolliker).  In  both  sexes  up  to  the  age  of  puberty  there  is  only  a  slight 
further  development. 

As  puberty  is  approached,  the  various  ducts  produce  a  few  side 
branches,  which  to  some  extent  bifurcate  and  form  in  turn  club-like 
terminations.  In  the  male,  complete  development,  so  far  as  it  goes,  is 
reached  about  the  twentieth  year,  when  the  breast  measures  from  4 
to  5  cm.  in  breadth.  Only  rarely  does  it  develop  further,  as  in  pseudo- 
hermaphroditismus  masculinus.  In  the  female,  however,  the  forma- 
tion of  side  branches  and  end  bulbs  is  more  extensive,  especially  in  the 
deeper  parts  of  the  organ,  although  the  transition  to  a  perfect  acinous 
gland  is  still  incomplete.  The  glandular  elements  are  composed  of  a 
structureless  basement  membrane  lined  by  short  cylindrical  cells,  and 
are  surrounded  by  a  zone  of  firm,  almost  hyaline,  connective  tissue. 
Besides  the  regular  ducts,  in  the  lobules  are  to  be  seen  solid  masses  of 


ANOMALIES  OF  DEVELOPMENT  883 

epithelial  cells  bounded  by  a  basement  membrane.  These  various  parts 
are  held  together  by  a  firm  connective-tissue  stroma  containing  elastic 
fibrils  and  fat;  about  the  larger  ducts  in  the  nipple  fibers  of  unstriped 
muscle  are  present.  The  changes  incident  to  the  onset  of  puberty  may  be 
so  marked,  both  in  boys  and  girls,  that  the  breasts  become  swollen  and 
tender  and  a  milk-like  secretion  is  produced. 

It  is  only,  however,  at  the  onset  of  lactation  that  the  breast  attains  its 
full  development.  Numerous  side  branches  and  end  bulbs  are  pro- 
duced, so  that  regular  acini  are  formed,  which  now  present  a  definite 
lumen.  The  lining  epithelium  is  a  single  layer  of  cylindrical  cells  more 
or  less  flattened  from  the  accumulation  of  secretion.  The  basement 
membrane  is  composed  of  subepithelial  flat  cells,  that  about  the  end 
bulbs  assume  a  stellate  appearance,  but  about  the  ducts  are  more 
spindle-like,  with  their  long  axes  the  way  of  the  ducts.  Outside  the 
membrane  is  the  hyaline  zone,  and  then  a  kind  of  adventitia  composed 
of  a  cellular  and  vascular  connective  tissue.  The  stroma,  as  a  whole,  is 
softer,  more  juicy,  and  congested.  After  the  cessation  of  lactation  the 
gland-tubules  collapse  and  diminish  somewhat  in  size,  while  the  con- 
nective-tissue stroma  is  again  relatively  increased.  It  never  again, 
however,  attains  its  former  firmness  and  consistency. 

After  the  menopause  the  acini  atrophy  and  the  tubules  collapse,  while 
the  epithelium  shows  degenerative  changes.  The  tubules  gradually 
revert  to  the  infantile  condition.  It  is  not  uncommon  to  find  cystic 
dilatation  of  the  ducts  with  accumulation  of  a  brownish  or  grayish,  thin, 
or  mucoid  fluid.  Ultimately,  the  acini  entirely  disappear  and  only  the 
ducts  remain. 

ANOMALIES  OF  DEVELOPMENT. 

Absence  of  the  breasts  (amastia)  is  a  rare  condition  and  only  found  in 
association  with  grave  developmental  defects.  Sometimes  only  one  organ 
is  defective,  usually  the  right.  The  ovary  on  the  same  side  may  also  be 
absent  in  such  cases.  Abnormal  smallness,  either  of  the  gland  as  a  whole 
( micro mastia)  or  of  the  nipple  (microthelia),  is  more  common.  Micro- 
mastia,  like  amastia,  is  more  frequent  on  the  right  side  and  becomes  in 
evidence  first  at  puberty. 

Of  more  importance  is  an  increase  in  the  number  of  nipples  (poly- 
thelia)  or  of  the  glands  themselves  (poly mastia).  The  supernumerary 
organs  may  be  found  on  both  sides  or  only  on  one,  usually  the  left. 
The  redundant  nipples  are  often  defective  in  size  or  abnormally  formed, 
and  may  be  situated  on  one  and  the  same  gland  or  may  be  connected  with 
accessory  glands.  As  a  rule,  in  polymastia  there  are  only  one  or  two 
supernumerary  breasts,  but  as  many  as  ten  have  been  observed.  The 
additional  organ  is  usually  below  and  to  the  inner  side  of  the  normal 
breast,  rarely  above  and  external.  Exceptionally,  breasts  have  been 
found  on  the  acromion,  the  thigh,  or  the  labium.  As  a  rule,  these 
supernumerary  structures  are  imperfectly  developed,  but  occasionally 
have  been  known  to  functionate.  The  condition  is  met  with  in  males 


884  THE  MAMMARY  GLAND 

as  well  as  in  females,  and  has  been  explained  as  a  reversion  to  an  ancestral 
condition.  Many  cases,  however,  are  due  to  the  separation  in  the  course 
of  growth  of  portions  of  the  original  gland  substance,  which  become  dis- 
located to  some  distant  part. 


CIRCULATORY  DISTURBANCES. 

Hyperemia. — Hyperemia  of  a  physiological  character  is  met  with 
during  menstruation,  the  breast  becoming  swollen  and  tender.  This 
condition  may,  however,  go  on  to  hemorrhage,  which  may  take  place  into 
the  skin,  the  interstitial  substance,  or,  rarely,  into  the  ducts.  In  the  last 
event,  which  is  more  liable  to  occur  in  cases  of  dysmenorrhcea  and 
amenorrhcea,  the  blood  may  be  discharged  through  the  nipple  (vicarious 
menstruation).  The  blood  is,  as  a  rule,  absorbed,  but  may  form  tumor- 
like  nodules  of  a  yellowish-red,  fibrinous  substance  or  a  chocolate-like 
debris.  It  is  possible  that  such  areas  may  soften  and  be  converted  into 
cysts.  Hemorrhage  from  the  nipple  may  also  be  due  to  the  development 
of  a  papilloma  within  the  ducts. 

A  remarkable  affection  is  one  sometimes  met  with  in  hysterical  women, 
where  there  are  single  or  multiple  nodules'  the  size  of  a  hen's  egg,  that 
on  palpation  give  the  sensation  of  tumors.  They  are  nothing  more  than 
local  areas  of  oedema  of  neuropathic  origin  (angioneurotic  oedema,  hyster- 
ical or  blue  oedema — Charcot).1 


INFLAMMATIONS 

Inflammation  of  the  breast  may  begin  in  the  skin,  the  superficial 
connective  tissue,  the  nipple  or  areola,  or  in  the  substance  of  the  gland 
itself.  It  is  acute  or  chronic. 

Acute  inflammation  of  the  areola  begins  in  the  sebaceous  glands  and 
may  lead  to  local  abscess-formation  or  a  diffuse  phlegmon  involving  the 
skin  and  subcutaneous  tissue.  The  appearances  do  not  differ  materially 
from  those  of  ordinary  erysipelas  of  the  skin  elsewhere. 

Inflammation  of  the  connective  tissue  behind  the  breast  is  of  more 
importance  (paramastitis).  It  is  rather  a  rare  condition,  and  may  be 
caused  by  extension  of  inflammation  from  the  substance  of  the  breast, 
by  carious  ribs,  by  the  bursting  of  an  empyema  through  the  chest  wall, 
or  by  an  axillary  abscess.  Fever  and  constitutional  disturbance  are 
marked.  The  breast  is  pushed  forward,  but  retains  its  normal  shape,  is 
tense,  and  feels  as  if  it  were  resting  on  an  elastic  cushion.  Pain  is  severe 
and  increased  by  the  movements  of  the  arm  and  chest.  Generally  an 
abscess  forms  (retromammary  abscess),  which  may  burst  into  the  thoracic 
cavity  or  dissect  through  the  breast,  forming  numerous  sinuses  or  external 
fistulse.  Orth2  records  an  extraordinary  case  where  the  entire  breast 

1  See  Fowler,  Medical  Record,  1  :  1890  :  179  and  191.        2  Lehrbuch,  1893:  654. 


MASTITIS  885 

became  sequestrated  owing  to  the  dissection  of  an  abscess  in  the  con- 
nective tissue  surrounding  it. 

Thelitis. — Inflammation  of  the  nipple  (thelitis)  is  not  uncommon  in 
nursing  women,  and  originates  in  small  cracks  or  fissures  caused  by  the 
irritation  of  the  mechanical  act  of  sucking  and  the  macerating  action  of 
the  milk  and  saliva.  In  such  cases  infection  readily  takes  place,  espe- 
cially with  the  staphylococcus,  less  commonly  the  streptococcus,  or  the 
thrush  fungus.  The  process  readily  spreads  to  the  substance  of  the 
gland  by  means  of  the  lymphatic  channels  or  along  the  galactophorous 
ducts. 

Mastitis. — Acute  Mastitis. — Acute  inflammation  of  the  breast  proper 
— acute  mastitis — occurs  at  all  ages.  It  is  found  in  the  first  few  days 
of  infancy,  where,  as  has  been  before  remarked,  swelling  and  inflam- 
mation of  the  breasts  is  not  uncommon.  This  would  rarely,  however, 
cause  much  trouble  were  it  not  for  the  meddlesome  practice  among  nurses 
of  "rubbing  out  the  milk/'  It  is  also  seen  in  girls  about  the  time  of 
the  first  menstruation,  particularly  in  those  of  a  strumous  disposition, 
and  is  met  with  as  a  complication  of  the  infectious  fevers.  These 
forms  rarely  go  on  to  suppuration.  Acute  mastitis  is,  however,  by  far 
the  most  frequently  found  during  the  first  month  after  delivery,  and 
especially  in  primiparse.  Here  the  process  is  in  immediate  relationship 
to  the  function  of  lactation.  Traumatism,  as,  for  instance,  erosions  or 
fissures  of  the  nipple,  is  the  direct  exciting  cause,  as  it  leads  to  the  infec- 
tion of  the  breast  with  microorganisms.  The  old  view  that  retention  or 
oversecretion  of  milk  is  the  cause  is,  of  course,  incorrect,  except  in  so  far 
as  these  conditions  predispose  to  the  occurrence  of  infective  processes. 

Puerperal  Mastitis. — In  puerperal  mastitis  the  inflammation  is  of  the 
exudative  type,  and  may  either  be  uniformly  disseminated  throughout 
the  breast  (diffuse  mastitis)  or,  as  is  most  frequently  the  case,  affects 
a  circumscribed  area,  usually  the  lower  and  outer  portion  of  the  organ. 
Sometimes  multiple  isolated  foci  are  produced.  The  inflammatory 
process  in  most  cases  originates  in  the  connective  tissue  between  the  lobules 
(interlobular  mastitis),  which  is  hyperemic,  infiltrated  with  inflammatory 
products  and  round  cells.  The  epithelium  of  the  acini  shows  merely 
secondary  degenerative  changes.  The  process  may  resolve  or  go  on  to 
abscess-formation.  Multiple  foci  of  suppuration  are  produced,  which 
often  coalesce  to  form  large  pus  cavities.  The  pus  frequently  burrows 
around  and  between  the  lobules,  until  the  breast  is  practically  disorgan- 
ized. Perforation  may  take  place,  usually  through  the  skin,  or  into 
a  milk  duct,  or,  again,  into  the  pleural  cavity.  When  the  abscess  is  large 
we  get  a  cavity  with  irregular  nodular  walls,  unless  in  the  case  where 
the  pus  is  contained  within  a  dilated  milk  duct,  when  the  wall  is  smooth. 
Fistulse  are  often  formed  externally,  which  may,  in  some  cases,  com- 
municate with  the  milk  ducts  (milk  fistulce).  Occasionally  the  abscess 
does  not  perforate,  the  pus  becomes  more  or  less  absorbed,  and  only  a 
fatty,  granular,  and  calcareous  detritus  remains.  In  other  cases  the 
process  assumes  a  chronic  course  with  the  formation  of  contracting 
scar  tissue  and  discharging  sinuses. 


886  THE  MAMMARY  GLAND 

Acute  Galactophoritis. — Acute  galactophoritis,  or  inflammation  of  the 
milk  ducts,  is  found,  as  a  rule,  a  considerable  time  after  the  puerperium, 
usually  in  anemic  patients.  It  apparently  originates  in  a  catarrh  of  the 
larger  ducts,  and  is  again  the  result  of  infection.  Occasionally  it  is 
secondary  to  interstitial  or  interlobular  mastitis.  There  is  spontaneous 
pain  in  the  breast,  increased  during  the  active  function  of  the  gland, 
tenderness  on  pressure  over  the  duct,  and  during  the  quiescent  period 
pus  may  be  expressed  from  the  nipple. 

According  to  Orth,  the  staphylococcus  is  the  most  common  offender  in 
this  affection,  and  the  streptococcus  in  interstitial  mastitis. 

Chronic  Mastitis. — Chronic  mastitis,  as  has  just  been  hinted,  may 
follow  the  acute  form,  and  abscesses  may  remain  more  or  less  latent  for 
a  long  time,  accompanied  by  local  fibrosis.  Besides  this,  however,  we 
have  to  recognize  a  chronic  productive  inflammation  in  which  the  over- 
growth of  fibrous  tissue  is  much  the  most  prominent  feature.  As  in 
the  case  of  the  acute  forms,  here  again,  we  have  a  diffuse  and  a  local 
variety. 

The  diffuse  form  (cirrhosis  mamma,  Wernher)  is  very  rare.  Begin- 
ning with  pain  and  other  signs  of  inflammation,  the  breast  at  first  swells, 
but  later  gradually  shrinks  in  size,  owing  to  the  formation  of  contracting 
scar  tissue.  The  skin  becomes  attached  to  the  deeper  structures,  with 
some  dimpling,  and  the  nipple  is  often  drawn  in  so  that  the  resemblance 
to  certain  forms  of  scirrhus  is  striking.  Only  a  careful  examination  will 
suffice  to  differentiate,  and  not  always  then.  The  axillary  glands  are 
swollen  and  tender,  but  in  mastitis  the  enlargement  is  not  permanent. 
Sometimes  a  compensatory  overgrowth  of  the  surrounding  fatty  tissue 
of  the  breast  occurs,  so  that  the  total  volume  of  the  breast  need  not  be 
diminished. 

Microscopically,  together  with  the  formation  of  scar  tissue,  the  acini 
are  atrophic  and  the  ducts  dilated  into  cysts. 

The  local  form  of  productive  mastitis  is  much  more  common.  This 
gives  rise  to  the  formation  of  multiple  hard  nodules,  sometimes  in  both 
breasts. 

Microscopically,  the  appearances  do  not  differ  from  the  former  type, 
except  that  the  condition  is  circumscribed  and  not  diffuse.  In  spite  of 
the  fact  that  atrophy  of  the  acini  is  the  rule,  carcinomatous  transforma- 
tion has  been  known  to  occur  (mastitis  carcinomatosa  of  some  authors1). 

Tuberculosis. — This  affection  of  the  mammary  gland  occurs  at  all 
ages  and  in  both  sexes.  The  occurrence  of  pregnancy  and  the  puerperal 
state  favor  the  extension  of  the  disease.  The  lesions  may  be  unilateral 
or  bilateral.  The  disease  was  first  described  by  Sir  Astley  Cooper2  in 
1836,  and  since  his  time  several  careful  studies  have  appeared. 

Primary  tuberculosis,  so  far  as  we  know,  does  not  occur,  but  the 

1  See  Reclus,  Maladie  cystique  des  mamelles,  Rev.  d.  Chir.,  1865:  761;  and  Gaz. 
des  Hop.,  1887:  673;  also  Konig,  Centralbl.  f.  Chir.,  3  :  1893  :  49. 

2  Diseases  of  the  Breast,  1836.     See  also  Sabrazes  and  Binaud,  Arch.  gen.  de 
medecine,  1896,  for  the  pathological  anatomy. 


SYPHILIS  887 

affection  originates  in  the  extension  of  infection  from  other  parts,  most 
frequently  by  retrograde  metastases  from  the  axillary  glands  or  thoracic 
cavity,  from  a  carious  rib,  or  occasionally  through  the  blood  stream. 

Three  forms  may  be  differentiated — the  acute  miliary,  the  discrete, 
and  the  confluent. 

The  first  type  is  similar  to  the  miliary  affection  elsewhere  and  does  not 
call  for  an  extended  description.  It  is,  of  course,  hematogenic. 

In  the  discrete  form  one  or  more  nodules  are  to  be  found,  varying  in 
size  from  a  hemp-seed  to  an  almond.  When  a  single  mass  is  present 
it  is  usually  in  the  upper  and  outer  portion  of  the  breast.  The  multiple 
nodules  are  disseminated  throughout  the  organ.  The  breast  is  not,  as 
a  rule,  enlarged,  the  skin  is  intact,  and  the  nodules  are  firm,  distinct, 
and  usually  immovable.  On  section  the  lesions  are  composed  of  a 
central,  grayish  or  whitish  caseous  material,  or  sometimes  contain  a  puri- 
form  fluid,  and  are  surrounded  by  a  more  reddish-gray,  semitranslucent 
zone.  There  is  also  often  a  certain  amount  of  fibrosis  in  the  immediate 
neighborhood. 

In  the  confluent  form,  which  originates  in  the  condition  just  described 
by  the  coalescence  of  neighboring  foci,  the  breast  is  often  considerably 
enlarged,  and  usually  asymmetrically  so.  The  organ  is  firm  and  appears 
to  contain  a  solid  mass.  On  section,  the  affected  area  is  made  up  of 
cavities  irregularly  spherical  with  lateral  pouches.  In  the  adjacent  parts 
may  be  seen  apparently  separate  cavities,  which,  however,  are  found  to 
communicate  with  the  main  abscess  by  small  channels.  The  whole, 
therefore,  frequently  has  an  areolar  appearance.  The  cavities  are  lined 
by  a  soft,  pyogenic  membrane  presenting  yellowish  points.  Radiating 
from  the  central  area  of  softening  are  dense,  fibrous  bands,  and  in  the 
immediate  vicinity  can  be  seen  small  secondary  tubercles.  The  larger 
cavities  frequently  communicate  with  the  exterior  by  fungous-looking 
sinuses.  As  a  rule,  one  breast  only  is  affected.  The  process  begins  in 
the  connective  tissue  surrounding  the  lobules  and  acini,  but  it  may 
spread  into  the  ducts. 

Microscopically,  the  tubercles  have  the  ordinary  composition  of  epi- 
thelioid,  round,  and  giant  cells,  with  central  caseation,  and  peripheral 
fibrosis.  A  point  worthy  of  note,  however,  is  that  tubercle  bacilli  are 
remarkably  scanty. 

A  rare  variety  of  the  confluent  form  is  the  cold  abscess.  It  is  essen- 
tially chronic  and  insidious  in  its  development,  and  is  found  only  in  the 
adult,  and  generally  after  pregnancy.  The  cavity  is  sharply  defined, 
lined  by  a  fungous  pyogenic  membrane  of  a  reddish-purple  color,  and 
contains  thin  pus  and  grumous  material. 

Syphilis. — Secondary  syphilitic  manifestations  in  the  skin  of  the  breast 
are  of  course  common  and  need  only  be  mentioned.  The  most  impor- 
tant lesion  is  the  primary  chancre  or  sclerosis,  which  is  found  starting  in 
the  nipple,  but  may  lead  to  destruction  of  this  organ,  and  extend  to  the 
skin  of  the  breast.  The  lesion  is  ordinarily  due  to  suckling  a  syphilitic 
child.  The  ulcer  is  of  the  characteristic  type,  chronic  and  indurated, 
and  accompanied  by  indolent  bubo  of  the  axillary  glands.  Gummas 


888  THE  MAMMARY  GLAND 

are  rare.     They  have  been  met  with  both  in  the  male  and  the  female 
breast.     More  common  is  said  to  be  diffuse  mastitis. 

Actinomycosis. — Actinomycosis  is  excessively  rare.  It  may  be  due 
to  the  extension  of  pulmonary  actinomycosis  through  the  thoracic 
wall.  One  or  two  cases  have  occurred  superficially  which  were  attributed 
to  the  application  of  poultices. 

RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — It  is  debatable  whether  retrogressive  changes  properly 
so-called  are  ever  met  within  the  mamma.  Simple  atrophy  of  the  gland- 
ular elements  is  found  as  an  involution  process  after  the  menopause,  and 
occasionally,  although  by  no  means  invariably,  after  removal  of  the 
ovaries.  The  atrophy  is  often  masked  by  an  excessive  overgrowth  of 
fat.  Atrophy  is  also  said  to  follow  the  prolonged  use  of  iodine  or  its 
derivatives.  Bollinger  and  others  have  described  a  curious  atrophy  from 
inactivity  among  the  people  of  Upper  Bavaria  that  appears  to  be  a  family 
vitium. 

Cysts. — Cysts,  usually  multiple  and  of  small  size,  filled  with  greenish 
or  brownish  fluid,  are  not  uncommon  in  the  breast  during  the  involution 
period.  They  are  due  to  the  obstruction  of  the  ducts,  and  from  their 
size  and  hardness  may  simulate  scirrhus. 

PROGRESSIVE  METAMORPHOSES. 

It  is  perhaps  a  little  difficult  to  know  exactly  what  conditions  ought 
to  be  discussed  under  this  section.  As  is  well  recognized,  there  is  a 
physiological  relationship  between  the  breasts  and  the  genital  organs, 
and  this  interdependence  is  still  to  be  observed  in  various  pathological 
conditions,  of  which,  indeed,  it  may  be  a  cause.  There  are  also  certain 
states  of  overgrowth  and  functional  overactivity  that  are  perfectly  natural 
in  certain  individuals  at  certain  times,  which  in  other  persons,  at  other 
times  and  under  different  circumstances,  must  be  regarded  as  abnormal. 
Thus,  the  active  and  excessive  growth  associated  with  lactation,  if  found 
in  the  non-pregnant  or  non-parturient  woman,  before  the  age  of  puberty 
or  after  the  menopause,  or,  again,  in  the  male,  must  be  regarded  as  dis- 
tinctly pathological.  We  shall  not  perhaps  greatly  err  if  we  refer  to 
such  conditions  in  this  place. 

Hypertrophy. — An  hypertrophy  of  the  breasts,  simulating  that  found 
in  pregnancy,  is  found  associated  with  tumors  of  the  uterus  or  ovaries. 
Repeated  stimulation,  as  from  the  application  of  a  child  to  the  breast,  has 
sometimes  established  the  function  of  lactation  in  virgins  and  in  old 
women.  Occasionally,  in  males,  the  breasts  assume  the  female  type 
(gynecomastia),  and  milk  may  even  be  secreted.  The  condition  is  often 
associated  with  atrophy  of  the  testicles. 

A  peculiar  form  of  hypertrophy  is  that  not  infrequently  found  in  tuber- 
culous individuals. 


HYPERTROPHY 


889 


Compensatory  Hypertrophy. — Compensatory  hypertrophy  of  one  breast 
after  the  removal  of  the  other  may  be  produced  in  experimental  animals, 
as  Ribbert  has  shown,  but  it  is  doubtful  if  it  occurs  in  the  human  subject 
under  ordinary  conditions. 

A  vicarious  overgrowth  of  the  fatty  tissue  of  the  breast  has  been  ob- 
served in  cases  of  atrophy  and  contraction,  as  in  some  forms  of  scirrhus 
(cancer  atrophicans),  whereby  the  total  volume  of  the  breast  is  not 
altered  materially.  An  overgrowth  of  fat  is  also  common  in  simple 
cases  of  obesity. 

Exceptionally,  after  the  menopause,  and  therefore  after  the  normal 
stimuli  are  removed,  the  breasts  may  not  involute,  but  remain  large  and 
may  even  continue  to  secrete. 


FIG.  232 


Hypertrophy  of  the  breasts.     (James  Bell's  case.) 

Diffuse  Hypertrophy. — Of  more  importance  are  the  cases  of  diffuse 
hypertrophy  of  the  breast,  of  which  a  number  of  instances  are  now  on 
record.  The  affection  is  usually  met  with  in  young  girls  at  or  shortly 
after  the  time  of  the  first  menstruation.  The  growth  may,  for  a  time, 
remain  latent,  being  lighted  up  again  only  with  the  occurrence  of  preg- 
nancy, or  may  be  continuous  from  the  first.  Both  breasts  are  involved 
in  a  uniform  enlargement  of  the  tissues,  in  which,  however,  the  nipple 
does  not  participate,  but  is  gradually  flattened  out  over  the  mass.  Large 


MAMMA&Y  GLAND 

veins  are  generally  to  be  seen  beneath  the  skin,  and  necrosis  from  pressure 
or  interference  with  the  circulation  may  result.  The  breasts  have  a 
soft,  baggy  feel,  or  may  contain  hard,  more  tumor-like  masses,  as  in  a 
case  recorded  by  James  Bell.1  The  enlarged  organ  has  been  known  to 
attain  the  weight  of  from  four  to  seven  kilos  or  more.  Durston2  and 
Williams  have  each  recorded  a  case  in  which  a  breast  reached  the  enor- 
mous size  of  sixty-four  pounds.  Different  pathological  conditions  appear 
to  be  included  under  the  term  "diffuse  hypertrophy."  In  some  cases 
there  is  what  is  practically  a  diffuse  fibromatosis,  in  others  the  glandular 
elements  may  be  increased  as  well,  and  in  still  others  true  adenomatous 
masses  may  be  found  throughout  the  organ.  The  condition  seems  to 
be  associated  with  some  disorder  of  the  genital  functions. 

Care  should  be  taken  not  to  mistake  a  retromammary  lipoma  for  this 
condition. 

Tumors. — Much  difficulty  is  experienced  in  distinguishing  between 
the  various  conditions  that  lead  to  the  formation  of  new  tissue  in  the 
breast.  As  all  clinicians  are  aware,  retention  cysts  and  the  hyperplasia 
incident  to  chronic  mastitis  may  at  times  closely  resemble  the  true 
neoplasms  so  that  diagnosis  is  apt  to  be  difficult.  And  pathologically 
speaking  the  same  difficulty  confronts  us  in  differentiating  between 
what  is  true  tumor-formation  and  that  neoplastic  overgrowth  which  is 
so  often  secondary  to  inflammatory  irritation.  In  the  breast  we  have 
both  fibrous  and  glandular  elements,  and  it  is  usually  easy  to  say  whether 
the  latter  are  in  excess  of  the  normal  amount  for  the  individual  or  not, 
but  the  further  question  whether  this  overgrowth  is  primary,  "  active," 
and  independent,  or  secondary  and  what  might  be  termed  "passive," 
must  sometimes  remain  in  doubt.  This  passive  hyperplasia  is  at  one 
time  the  result  of  inflammatory  or  mechanical  irritation  and  at  another 
a  secondary  manifestation  concomitant  with  the  development  of  a  tumor 
in  the  related  tissues.  The  appearances  are  still  further  complicated 
when  cysts  are  produced.  In  some  cases  the  newly-formed  glandular 
structures  become  dilated,  partly  it  is  probable  from  obstruction,  but 
also  from  excessive  secretion  (cystadenoma),  while  in  others  there  is 
merely  a  retention  of  secretion  within  the  normal  or  approximately 
normal  acini  (retention  or  simple  cyst). 

The  breast  being  in  the  main  composed  of  three  distinct  types  of  tissue, 
the  integument,  the  glandular  elements,  and  the  fibrous  stroma,  we  have, 
corresponding  with  these,  epithelial,  adenomatous,  and  connective- 
tissue  tumors.  With  the  exception  of  the  epithelial  new-growths,  these 
are  not  always  pure  in  type,  for,  as  a  rule,  both  fibrous  and  glandular 
elements  partake  in  the  proliferation,  and  from  modifications  of  their 
structure  give  rise  to  a  considerable  variety  of  forms.  The  breast,  in 
fact,  is  a  common  site  for  the  development  of  mixed  growths.  As  an 
illustration  of  this  we  may  take  the  case  of  the  adenoma,  which  is  rather 
a  rare  tumor,  while  various  combinations  with  fibroma  (adenofibroma; 

1  Montreal  Med.  Jour.,  28: 1899:  772. 

2  Quoted  by  Labarraque,  These  de  Paris,  1875. 


nbroadenoma)  or  modified  connective  tissue  (adenomyxoma;  adeno- 
sarcoma ;  myxoadenoma,  sarcoadenoma)  are  much  more  common. 

The  exact  point  of  origin  for  many  of  the  breast  tumors  is  still  in  doubt. 
With  regard  to  the  fibromas  and  sarcomas,  Dreyfuss1  and  Billroth2 
believed  that  they  took  their  rise  in  the  hyaline  connective  tissue  sur- 
rounding the  acini,  but  this  undoubtedly  does  not  explain  all  forms. 
Again,  in  the  case  of  the  adenomatous  and  carcinomatous  new-growths, 
it  has  usually  been  taught  that  they  develop  from  the  epithelial  cells 
of  the  acini,  but  Creighton3  has  published  an  elaborate  study  in 
which  he  promulgates  the  view,  which,  indeed,  appears  to  be  sup- 
ported by  many  facts,  that  the  majority  of  the  glandular  tumors  originate 
not  in  the  acini  of  the  breast  proper,  but  in  sudoriparous  glands  which 
are  to  be  found  deep  down  in  many  normal  breasts  in  a  more  or  less 
perfect  state.  Some  of  them  are  possibly  to  be  explained  as  originating 
in  a  reversion  to  the  more  embryonic  condition.  This  work  of  Creigh- 
ton's,  while  most  suggestive,  as  yet  lacks  confirmation,  and,  like  other 
theories,  cannot  explain  all  cases.  However,  this  may  be,  the  ultimate 
causes  of  tumor-growth  in  the  breast  are  as  obscure  as  they  are  in  the 
case  of  neoplasms  of  other  regions. 

It  is  a  well-known  fact  that  the  breast  is  one  of  the  most  frequent  sites 
for  tumor-formation.  This  is  perhaps  to  be  explained  in  view  of  the  fact 
that  the  breast,  like  the  uterus,  where  new-growths  are  also  common,  is 
in  the  majority  of  individuals  for  a  prolonged  period  in  a  state  of  both 
physiological  and  anatomical  unrest.  The  truth  of  this  is  obvious 
when  we  consider  the  various  vicissitudes  to  which  it  is  liable  in  the  course 
of  pubescence,  gestation,  lactation,  and  senility.  Normally,  then,  we 
must  conclude  there  is  a  predisposition  to  rapid  proliferation  of  tissue, 
which  a  great  variety  of  apparently  trifling  stimuli  are  competent  to 
bring  about.  Besides  this,  inflammatory  changes  of  all  grades,  with 
their  associated  irritation  and  morphological  changes,  are  particularly 
common  in  the  breast,  so  that  it  is  not  extraordinary  that  the  natural 
balance  of  things  should  frequently  be  upset.  Many  writers  lay  stress 
upon  hereditary  influences,  trauma,  race,  and  sex.  Hereditary  pre- 
disposition is  found  only  in  the  case  of  malignant  growths,  and  has  been 
variously  estimated  as  being  present  in  from  9  to  21  per  cent.  The  in- 
fluence of  trauma  is  still  a  matter  for  debate.  A  history  of  injury  or 
inflammation  is  given  in  from  12  to  40  per  cent,  of  cases  of  carcinoma, 
while  in  sarcoma  the  influence  of  trauma  is  said  to  be  much  greater. 
Whether  this  is  of  etiological  importance  or  is  a  mere  coincidence,  we 
are  not  as  yet  in  a  position  to  say.  With  regard  to  race,  it  is  a 
remarkable  fact  that  fibrous  tumors  of  the  breast  are  rare  in  negresses, 
while,  on  the  other  hand,  fibroids  of  the  uterus  are  particularly  common. 
With  regard  to  sex,  practically  all  the  tumors  found  in  the  female 
breast  may  be  met  with  in  the  male,  but  with  much  less  frequency,  a 
fact  which  goes  a  long  way  to  support  the  view  just  enunciated  that  dis- 

1  Virch.  Archiv,  113:  1888:  535.  2  Virch.  Archiv,  18:  1860:  51. 

3  Cancers  and  Other  Tumors  of  the  Breast,  Williams  &  Norgate,  London,  1902. 


892  THE  MAMMARY  GLAND 

turbance  of  functional  and  structural  equilibrium  is  an  important  etio- 
logical  factor. 

Tumors  of  the  breast  may  be  conveniently  divided  into  benign  and 
malignant  forms.  Apart  from  plain  cysts,  among  the  former  we 
may  recognize  fibroma,  adenoma,  fibroadenoma,  adenofibroma,  cystade- 
noma,  lipoma,  myxoma,  myoma,  angioma,  osteoma,  chondroma;  among  the 
latter,  epithelioma  and  various  types  of  carcinoma  and  sarcoma.  Many 
of  the  cysts  are  merely  "retention"  cysts  and,  therefore,  properly  not 
tumors,  while  some  are  either  benign  or  malignant  cystadenomas,  cystic 
fibre  mas,  or  sarcomas.  An  overwhelming  proportion  of  mammary  growths, 
variously  estimated  by  White,  Williams,  Gross,  and  Senn  at  from  80 
to  95  per  cent.,  are  carcinomas.  An  analysis  of  cases  from  the  records 
of  the  Royal  Victoria  Hospital  gives  the  following  proportions  for  the 
various  forms:  Total  number  of  cases,  184;  fibroma,  1;  adenoma,  31; 
fibroadenoma,  11;  cystadenoma,  1;  sarcoma,  3;  epithelioma,  5;  carcinoma, 
132.  These  figures  agree  fairly  well  with  those  of  Williams,1  who  reports 
2430  cases  divided  as  follows:  fibroadenoma,  15.3  per  cent.;  myxoma, 
0.16;  sarcoma,  3.9;  carcinoma,  77.5.  The  great  preponderance  of  malig- 
nant forms  renders  it  imperative  that  all  mammary  tumors  should  be 
removed  early.  Even  the  fibroadenoma  has  been  known  to  give  rise 
to  metastases  not  withstanding  the  fact  that  the  histological  picture  has 
been  that  of  a  non-malignant  growth.  Hanseman2  has  recorded  a  case 
of  this  type,  and  many  German  pathologists,  therefore,  speak  of  "car- 
cinoma in  the  guise  of  adenofibroma."  In  any  case,  before  giving  an 
opinion  it  is  necessary  to  examine  every  portion  of  the  tumor,  and  even 
then  one  may  be  deceived.  An  adenofibroma  is  rarely  mistaken  for  a 
carcinoma,  but  no  doubt  the  reverse  frequently  occurs. 

Fibroma. — The  most  common  of  the  benign  new-growths  is  the 
fibrous  tumor.  This  is  composed  of  more  or  less  dense,  fibrous  tissue 
in  which  are  embedded  glandular  elements,  differing  but  little  from 
those  of  the  normal  gland  (fibroma),  although  in  some  cases,  while 
there  is  an  overgrowth  of  the  connective-tissue  elements,  there  is  a 
preponderance  of  glandular  structures  (fibroadenoma).  All  possible 
variations  between  the  two  extreme  types  may  occur  (adenofibroma). 
In  this  connection  it  should  be  remarked  that  considerable  confusion  in 
the  nomenclature  has  arisen  from  the  loose  way  in  which  these  various 
growths  have  been  regarded.  In  other  words,  authorities  have  not  been 
clear  as  to  what  constituted  an  adenoma  and  what  a  fibroma.  A 
little  thought  would  have  avoided  the  difficulty  A  fibroma  is  a  tumor 
composed  of  fibrous  tissue.  In  the  course  of  its  formation  it  naturally 
may  include  certain  ducts  and  acini  of  the  breast.  The  glandular 
structures  may  further  be  considerably  altered  from  traction  or  pressure, 
and  frequently  present  proliferative  or  degenerative  changes,  owing  to 
the  irritation  produced  by  mechanical  or  inflammatory  causes.  These 
changes  are,  however,  obviously  secondary  and  in  no  respect  to  be  con- 

1  Brit.  Med.  Jour.,  2: 1892:  576. 

2  Die  mikroskopische  Diagnose  der  bosartigen  Geschwiilste. 


FIBROMA  893 

sidered  as  evidences  of  independent  growth.  Such  a  tumor  can  only 
be  a  fibroma,  not  a  fibroadenoma.  It,  of  course,  takes  considerable 
practice  to  decide  whether  the  glandular  changes  in  any  given  case  are 
secondary  or  are  really  an  adenomatous  new-formation,  but  an  attempt 
should  be  made  in  every  case  to  settle  the  point.  An  adenoma,  on  the 
other  hand,  is  a  tumor  composed  chiefly  of  glandular  elements  derived 
from  the  acini  or  ducts,  but  varying  notably  from  those  characteristic  of 
the  normal  gland.  The  epithelial  cells  forming  the  acini  and  ducts 
have  proliferated  considerably,  so  that  solid  epithelial-cell  masses  are 
produced  or  lumina  enclosed  by  several  layers  of  cells,  in  contradistinc- 
tion to  the  normal  acini,  where,  at  least  in  the  active  condition,  the 
acini  are  composed  of  a  single  row  of  cells.  Farther,  in  adenoma,  and 
it  is  this  that  constitutes  the  main  difference  between  the  tumor  and 
those  secondary  changes  which  have  just  been  mentioned,  the  ducts 
and  acini  are  not  grouped  into  lobules,  nor  are  they  mere  offshoots 
from  the  lobules,  but  are  arranged  in  an  erratic  way  differing  more  or 
less  widely  from  the  orderly  arrangement  of  the  normal  gland.  The 
diffuse  enlargement  of  the  breasts,  referred  to  above  under  the  name  of 
"diffuse  hypertrophy,"  is  in  some  cases  associated  with  great  increase 
of  the  glandular  elements,  and  hence  has  been  called  by  some  "  diffuse 
adenoma."  It  is  in  no  sense,  however,  a  tumor,  but  a  form  of  hyper- 
plasia.  A  true  adenoma  is  unilateral,  circumscribed,  and  encapsulated. 
Such  a  tumor  may  be  associated  with  fibromatous  overgrowth  (fibro- 
adenoma, adenofibroma),  or  the  fibrous  tissue  may  undergo  mucoid  or 
sarcomatous  transformation  (adenomyxoma,  adenosarcoma,  etc.).  It  is 
the  imperfect  apprehension  of  these  considerations  that  has  led  to  the 
multiplicity  of  names  that  have  been  proposed  for  these  tumors,  such  as 
adenoid,  fibroadenoma,  and  adenocele. 

Pure  fibromas  without  any  admixture  with  glandular  structures  are 
rare.  As  a  rule,  certain  ducts  and  acini  become  entangled  in  the 
fibrous  overgrowth  and  appear  as  compressed  and  atrophied  remnants, 
or,  again,  become  distorted  and  dilated.  In  some  cases  the  dilatation 
amounts  to  cyst-formation,  so  that  clinically  we  can  differentiate  solid 
and  cystic  fibromas.  Fibromas  are  met  with,  as  a  rule,  in  early  adult 
life,  from  the  age  of  sixteen  to  thirty,  although  cases  have  been  met  with 
as  early  as  twelve  and  as  late  as  fifty-six.  The  cystic  forms  are  found 
somewhat  later  than  the  others.  The  tumor  usually  first  comes  in  evi- 
dence during  menstruation  or  the  puerperium,  owing  to  the  discomfort 
it  causes  at  such  times.  The  growth,  as  a  rule,  forms  a  circumscribed, 
rounded,  and  nodular  mass,  firm  and  elastic,  projecting  under  the  skin. 
The  most  frequent  site  is  at  the  periphery  of  the  breast  above  the  nipple, 
although  the  intracanalicular  variety  is  apt  to  be  more  deeply  situated. 
When  cysts  are  present  they  may  often  be  recognized  as  fluctuating  bosses. 
A  capsule  is  usually  formed,  so  that  the  growth  is  freely  movable.  Fi- 
bromas are  generally  solitary,  but  may  be  multiple,  or  even  affect  both 
mammae.  The  rate  of  growth  is  slow  and  the  tumor  rarely  attains  a 
large  size.  Cystic  forms  tend  to  enlarge  more  rapidly.  Cysts  are  more 
likely  to  be  found  in  the  older  patients.  After  removal  local  recurrence 


834 


THE  MAMMARY  GLAND 


has  been  observed  in  some  few  cases,  but  there  is  no  tendency  to  infiltra- 
tion.    Besides  the  nodular  form  just  described,  there  is  a  diffuse  variety 


FIG.  233 


Pericanalicular  fibroma  of  the  mammary  gland.  The  glandular  acini  and  ducts  are  prominent 
and  show  some  irregular  overgrowth  of  the  epithelium,  but  the  main  feature  is  the  development 
of  connective  tissue  both  periacinous  and  interstitial,  the  latter  not  sharply  defined.  (Ribbert.) 

FIG.  234 


Intracanalicular  fibroma  or  intracystic  papilloma  of  breast.     (Orth.) 

in  which  the  whole  breast  is  liable  to  be  involved.  Here,  the  fibrous 
tissue  forms  cylindrical  sheaths  along  the  ducts  and  acini,  the  so-called 
plexiform  fibroma  of  Nordmann. 


FIBROMA  895 

On  section,  fibromas  are  firm,  fasciculated,  and  of  a  grayish  or  grayish- 
red  color,  and,  if  of  the  pericanalicular  variety,  made  up  of  an  agglomer- 
ation of  hard  nodules.  Should  there  be  dilatation  of  the  ducts  this  is 
evidenced  by  the  presence  of  numerous  minute  fissures,  or  even  cysts. 

Histologically,  we  may  differentiate  two  main  types,  the  pericanalicular 
or  periglandular  and  the  intracanalicular. 

In  the  former  there  is  proliferation  and  often  hyaline  transformation 
of  the  adventitial  connective  tissue  about  the  gland-tubes,  which  are 
usually  altered  by  mechanical  pressure.  The  newly  formed  fibrous 
tissue  surrounds  the  glandular  elements  as  a  well-defined  sheath.  This 
produces  on  section  the  appearance  of  a  nodular  or  granular  surface. 
The  condition  of  the  interlobular  connective  tissue  varies,  at  one  time 
partaking  but  little  or  not  at  all  in  the  hyperplasia,  while  at  others  it  is 
increased  and  merges  almost  imperceptibly  into  the  adventitial  sheaths, 
thus  giving  rise  to  a  more  diffuse  fibrous  growth.  The  glandular 
tissue  preserves  more  or  less  completely  the  ordinary  arrangement  into 
lobules.  Should  the  various  ducts  become  obstructed,  as  not  infre- 
quently happens,  irregular  fissures  or  actual  cysts  are  produced  (fibroma 
cysticum).  The  fibrous  tissue  may  be  dense  or  cellular,  giving  rise  to 
hard  and  soft  fibromas. 

In  the  intracanalicular  form  there  is  a  remarkable  overgrowth  either 
of  the  periglandular  adventitia  or  of  both  this  and  the  interstitial  stroma 
into  the  lumina  of  the  ducts  and  acini,  so  that  a  kind  of  cystic  tumor 
is  produced,  the  cavities  of  which  are  filled  with  conical,  nodular,  or 
leaf-like  projections,  giving  the  tumor  a  warty,  papillomatous,  or  cauli- 
flower appearance.  These  papillae  are  covered  with  epithelium  similar 
to  that  of  the  normal  glands. 

Fibroadenoma  and  Adeno fibroma. — Here  we  have  a  combination  in 
varying  proportions  of  hyperplastic  connective  tissue  and  adenomatous 
new-formation.  Should  the  glandular  elements  predominate,  we  have 
a  fibroadenoma;  if  the  fibrous  tissue  is  more  developed,  then  we  have 
an  adenofibroma.  The  glandular  structures  recall  in  appearance  both 
the  acini  and  the  ducts,  but  differ  considerably  from  those  of  the 
normal  gland,  being  more  numerous,  wider,  and  irregular,  with  pro- 
liferation of  their  epithelial  lining.  With  this  there  is  a  more  or  less 
marked  increase  of  the  fibrous  elements. 

Taking  the  intracanalicular  fibroma  as  a  prototype,  we  have  a  cyst- 
adenofibroma  intracanaliculare  corresponding  to  it.  In  this  the  papillary 
excrescences  are  present  as  before,  but  the  fibrous  tissue  is  much 
reduced,  forming  merely  a  delicate  central  core,  while  the  epithelial  cells 
have  actively  proliferated  and  are  heaped  up  into  masses.  In  other 
cases  adenomatous  structures  are  found  within  the  substance  of  the 
fibrous  outgrowths  (adenocele,  Virchow).  Owing  to  the  rapid  develop- 
ment of  the  papillary  processes,  the  cavities  of  the  gland  become 
greatly  dilated,  so  that  cysts  would  be  produced  were  it  not  for  the  fact 
that  the  spaces  are  practically  filled  up  with  cauliflower-like  masses, 
reducing  the  cavities  to  fine,  collapsed,  and  ramifying  fissures.  The 
growth  may  be  so  exuberant  that  papillomatous  excrescences  appear  at 


896 


THE  MAMMARY  GLAND 


the  nipple  externally  or  burst  through  the  skin.     When  this  occurs  the 
cyst-walls   are  perforated,  and,  owing  to  the  dislocation  of  the   out- 


FIG.  235 


Fibroadenoma  of  the  mamma  of  the  acinous  type.     Winckel  obj.  No.  3,  without  ocular. 
(From  the  collection  of  Dr.  A.  G.  Nicholls.) 


FIG.  236 


^^m 


Adenofibroma  of  mamma.     Zeiss  obj.  DD,  without  ocular.      (From  the  collection  of  the 
Royal  Victoria  Hospital.) 


CHONDROMA  AND  OSTEOMA  897 

growths  from  their  original  position,  their  place  is  left  free,  and  distinct 
cystic  cavities  make  their  appearance.  The  resemblance  to  the  papil- 
lary cystadenoma  of  the  ovary  is  striking.  The  cysts  when  present  are 
filled  with  a  serous,  mucinous,  and  viscid  fluid,  often  stained  with 
blood,  and  sometimes  containing  fat-globules  and  cholesterin.  Rarely, 
keratinized  epithelial  masses  (cholesteatoma)  are  found.  Both  the 
fibromas  and  the  fibroadenomas  are  liable  to  undergo  secondary  mucoid 
or  even  sarcomatous  change,  while  hemorrhagic  infiltration  and  oedema 
are  also  common.  This  gives  the  tumors  on  section  a  very  variable 
appearance,  here  firm  and  fibrous,  there  soft,  grayish,  gelatinous,  and 
transparent. 

The  adenomatous  mixed  tumors  are  found  at  all  periods  of  life,  but 
are  most  common  about  the  third  and  fourth  decade.  They  may  be 
quite  small  or  may  attain  a  considerable  size.  A  weight  of  twenty  kilos 
has  been  recorded.  The  rate  of  growth  is  sometimes  slow,  sometimes 
rapid.  The  growths  are  hemispherical,  definitely  lobulated,  and  some- 
what warty  on  the  surface.  At  first  they  are  freely  movable,  but  later 
become  attached  to  the  skin.  While  they  tend  to  grow  rapidly  and 
produce  considerable  disturbance  in  the  neighborhood,  they  are  in  general 
to  be  regarded  as  benign  growths,  as  when  removed  they  do  not  tend  to 
recur  and  do  not  form  metastases.  When  they  do  return,  it  is  gener- 
ally because  of  the  subsequent  development  of  a  small,  independent 
growth  that  has  been  overlooked.  This  statement  is  not  without  ex- 
ceptions, however.  A  suspicious  feature  is  when  the  tumor  masses  are 
multiple  or  when  both  breasts  are  affected. 

Adenoma. — Pure  adenoma  is  a  relatively  rare  tumor  in  the  breast. 
It  consists  of  a  fibrous  stroma  in  which  are  embedded  glandular  elements 
of  the  type  of  acini  or  of  ducts  lined  by  cylindrical  epithelium.  Thus, 
we  can  recognize  two  forms,  the  adenoma  acinosum  and  the  adenoma 
tubulare.  In  the  first  form  there  is  a  great  numerical  increase  of  the 
acini,  which  deviate  considerably  from  the  normal  in  that  they  are  not 
arranged  into  lobules.  In  the  tubular  variety  the  duct-like  structures 
are  evenly  scattered  throughout  the  tumor  or  are  aggregated  into  groups. 
In  both  forms  the  interstitial  fibrous  stroma  is  looser  and  more  cellular 
than  that  of  the  normal  breast.  The  tumor  is  usually  small,  circum- 
scribed, and  encapsulated.  It  occurs  in  young  women,  and  starts  as 
a  small  nodule  in  the  upper  and  outer  quadrant  of  the  mamma.  As  it 
enlarges  it  becomes  round  or  oval.  On  section  it  is  firm,  smooth,  and 
grayish- white  in  color,  and  a  milk-like  fluid  may  sometimes  be  expressed. 

Lipoma. — Lipoma  does  not  occur  in  the  breast  proper,  but  in  the  con- 
nective tissue  behind  or  above  it. 

Myxoma. — The  myxoma  is  rare  as  a  pure  tumor  without  admixture 
with  glandular  elements,  but  mucinous  transformation  of  fibromas  and 
sarcomas  is  not  uncommon. 

Myoma. — Myomas  are  also  rare.  They  start  from  the  unstriped  muscle 
in  the  skin  or  about  the  nipple. 

Chondroma  and  Osteoma. — Chondromas  and  osteomas,  either  as 
pure  growths  or  associated  with  sarcoma  or  carcinoma,  are  decidedly 
57 


THE  MAMMARY  GLAND 

uncommon.  Chondromas  are  more  frequent  in  dogs.  Care  should  be 
taken  not  to  mistake  cysts  with  calcified  walls  for  these  growths. 

Angioma. — Angioma  is  so  rare  as  only  to  need  mention. 

Sarcoma. — Sarcoma  of  the  breast  is  a  comparatively  rare  affection, 
forming  scarcely  4  per  cent,  of  the  tumors  found  in  this  situation. 
Clinically,  as  in  the  case  of  the  fibromas,  we  may  have  solid  and  cystic 

FIG.  237 


Chondroma  from  mamma  of  bitch.     At  one  point  the  specimen  shows  a  calcareous  deposit. 
Winckel  obj.  No.  3,  without  ocular.     (From  the  collection  of  A.  G.  Nicholls.) 

forms.  Cystic  sarcoma  is  said  to  be  peculiar  to  the  breast.  Sarcomas 
form,  when  pure,  unilateral,  circumscribed,  and  movable  nodules,  and 
occur  by  preference  in  girls  and  youngish  women,  being  rarely  found 
after  the  menopause.  In  this  respect  they  differ  from  carcinomas. 
Occasionally,  they  form  diffuse  masses  and  are  found  in  both  breasts. 
Sarcomatous  transformation  is  not  uncommon  in  fibromas  and  in  fibrous 
and  adenomatous  mixed  tumors. 

Histologically,  the  solid  sarcomas  are  composed  of  mixed  cells,  some 
of  them  giant  cells,  round  or  spindle  cells.  Myxomatous  degeneration, 
hemorrhage,  necrosis,  calcification,  are  not  infrequent  complications, 
Sometimes  the  component  cells  assume  an  alveolar  arrangement. 

Angiosarcoma  (perithelioma  malignum)  has  been  described,  starting 
from  the  adventitia  of  the  vessels.  As  curiosities,  may  be  mentioned 
melanotic  sarcoma  and  round-celled  sarcoma  containing  striated  muscle. 

Cystic  sarcomas  are  strictly  comparable  to  the  cystadenofibroma 
intracanaliculare  before  referred  to,  which  they  greatly  resemble.  These 
tumors  are  found  in  early  adult  life,  and  form  large  bossy,  succulent 


CARCINOMA  899 

growths  imperfectly  encapsulated.  On  section  they  present  irregular 
clefts  or  spaces  filled  with  mucoid  or  blood-stained  fluid,  into  which 
project  numerous  villi  or  papillary  excrescences  (cystosarcoma  phyllodes). 

Microscopically,  these  papillae  have  a  central  core,  not  of  ordinary 
fibrous  tissue,  but  of  a  highly  cellular  and  vascular  tissue  composed  of 
round  and  spindle  cells.  Myxomatous  and  degenerative  changes  may 
be  found. 

Involvement  of  the  chest  wall  and  the  skin  is  much  less  frequent 
than  in  the  case  of  carcinoma,  although  the  pectoralis  major  is  occasion- 
ally infiltrated.  Lymphatic  enlargement  is  rare  in  sarcoma,  and  when 
it  occurs  is  due  usually  to  inflammatory  or  other  irritation  and  not  to 
sarcomatous  invasion.  The  metastases  arise  through  the  blood  stream 
and  the  lungs  are  early  affected. 

Carcinoma. — The  breast  is  one  of  the  most  frequent  sites  for  carcinoma, 
40  per  cent,  of  all  cases  of  carcinoma  being  found  in  this  region  (Williams). 
The  average  age  at  which  it  is  first  discovered  is  forty-eight,  most  cases 
being  met  with  shortly  before  the  menopause.  It  is  rare  before  the  age  of 
thirty-five  and  in  advanced  life.  Instances  have,  however,  been  recorded 
under  twenty  (Boussereau)  and  as  late  as  ninety-four  (Coley1).  When 
not  operated  upon  the  average  duration  of  the  disease  is  27.1  months 
(Gross).  Some  cases  have  been  known,  however,  to  run  a  chronic 
course  of  from  five,  ten,  fifteen,  or  more  years.  One  authentic  case  is 
recorded  where  the  disease  lasted  more  than  thirty  years.  The  scirrhous 
type  is  the  one  most  likely  to  run  a  prolonged  course. 

Carcinoma  may  commence  in  any  part  of  the  breast,  being  found 
deeply  seated  or  immediately  under  or  about  the  nipple.  A  favorite 
position  is  in  the  lateral  portions  of  the  gland.  It  occurs  both  as  a 
circumscribed  nodule  or  as  a  diffuse  infiltrating  growth.  Clinically, 
we  recognize  superficial  and  deep-seated  forms.  In  the  former  class 
are  included  epithelioma  and  miliary  carcinosis  (squirrhe  disseminee) ; 
in  the  latter,  hard  and  soft  forms,  such  as  scirrhus,  car.  medullare, 
adenocarcinoma,  car.  gelatinosum. 

A  rare  form  of  carcinoma  is  squamous  epithelioma  (Paget's  disease  of 
the  nipple;2  malignant  papillary  dermatitis;  superficial  carcinoma  of 
the  skin).  This  affection  at  first  assumes  the  appearance  of  a  chronic 
eczema  of  the  nipple.  In  time  the  nipple  is  destroyed  and  the  disease 
spreads  over  the  surface  of  the  breast  and  eventually  invades  the  deeper 
parts.  The  ulcer  produced  is  slightly  raised,  with  sharp  edges,  and  is 
of  a  bright  red,  raw  appearance.  Secretion  is  scanty.  At  first  there  is 
a  thickening  of  the  epithelium  of  the  nipple,  followed  by  an  inflammatory 
infiltration  of  the  subepithelial  layers.  After  a  long  time  the  prolifer- 
ating epithelial  cells  reach  the  galactophorous  ducts  and  form  a  more 
deeply  invading  growth  of  the  usual  squamous  type,  with  epithelial  cell 
nests  (Perlkugeln). 

Another  rare  variety  is  the  acute  miliary  carcinosis,  or  disseminated 

1  Refer.  Handb.  Med.  Sci.,  2:  1901:  629. 

2  St.  Bartholomew's  Hosp.  Rep.,  1874 :  87. 


900  THE  MAMMARY  GLAND 

scirrhus.     In  170  cases  recorded  by  Williams  only  2  were  of  this  type. 
It  begins  superficially  and  appears  to  spread  by  means  of  the  lymphatics. 

The  deeper  forms  of  carcinoma  are  conveniently  divided  according  to 
the  amount  of  connective  tissue  they  contain  into  scirrhous  carcinoma, 
c.  simplex,  and  c.  medullare.  The  clinicians  are  in  the  habit  of  classify- 
ing them  into  hard  or  soirrhous  and  soft  forms.  It  should  be  remarked, 
however,  that  many  tumors  that  have  all  the  characters  of  scirrhus  on 
physical  examination,  when  examined  microscopically  are  really  simple 
cancers.  In  fact,  a  pure  scirrhus,  histologically  considered,  is  one  of 
the  less  common  types  of  carcinoma.  Consequently,  there  is  a  liability 
for  some  confusion  to  arise  in  the  use  of  the  term.  A  further  point  of 
considerable  importance  is  that  any  given  carcinoma  is  rarely  of  one  type 
throughout.  It  may  be  scirrhous  in  one  part  and  simple  carcinoma  in 
another,  proving  the  necessity  of  a  careful  examination  of  all  parts  of 
the  growth. 

The  forms  just  mentioned  conform  more  or  less  perfectly  to  an  acinous 
type  of  growth,  but  there  are  other  forms  in  which  the  epithelial  cells 
are  arranged  in  a  tubular  fashion  somewhat  resembling  ducts  (adeno- 
caroinomd),  and  still  others  that  are  cystic. 

Scirrhous  carcinoma  begins  as  a  small  nodule  within  the  breast.  It  is 
not  so  sharply  defined  as  a  fibroadenoma,  as  it  is  not  encapsulated,  and 
soon  becomes  more  or  less  immovable.  It  is  knotty  on  the  surface  and 
somewhat  flattened,  with  rounded  outgrowths  from  the  margin.  In 
consistency,  it  is  extremely  hard,  without  much  elasticity,  and  conveys 
to  the  examining  hand  a  suggestion  of  weight  rather  than  size.  Later, 
fixation  to  the  skin  occurs,  which  becomes  immovably  adherent  with 
some  dimpling.  Only  when  advanced  does  the  tumor  project  above 
the  general  level  of  the  breast.  In  many  cases,  where  the  larger  ducts 
are  implicated,  it  is  impossible  to  draw  the  nipple  forward,  and  later 
on  the  nipple  is  actually  retracted.  In  cases  that  are  neglected  the 
tumor  becomes  attached  to  the  chest  wall  and  may  ulcerate  on  the 
surface.  The  breast,  as  a  whole,  is  often  flattened  and  its  volume 
diminished.  When  cut  into,  the  growth  is  hard,  nodular,  and  fibrous, 
often  showing  radiating  bands  of  connective  tissue  and  yellowish  patches. 
In  elderly  people  it  is  not  uncommon  to  find  small  involution  cysts  filled 
with  a  yellowish-green  fluid,  resembling  pus  or  colostrum. 

Histologically,  the  carcinoma  cells  proper  are  small,  mononuclear, 
and  atrophic  looking,  arranged  in  small  islets  or  elongated  rows.  The 
fibrous  tissue  is  relatively  greatly  increased,  so  that  the  epithelial  cells 
have  the  appearance  of  being  compressed.  Undoubtedly,  there  is  a 
proliferation  of  the  interstitial  stroma  as  well.  At  the  periphery  the 
type  of  growth  is  apt  to  be  softer  and  more  cellular  than  in  the  centre. 
Consequently,  in  scirrhus  the  metastases  are  frequently  of  the  simplex  or 
medullary  type.  The  amount  of  fibrous  tissue  may,  in  some  cases,  be 
so  great  that  at  first  sight  the  new-growth  resembles  scar  tissue  rather 
than  a  tumor.  In  the  most  advanced  forms  of  this  type  the  carcinoma 
cells  are  largely  degenerated  and  reduced  to  debris,  while  the  tissue 
around  is  atrophic  and  sclerosed  (cancer  atrophicans). 


CARCINOMA  901 

In  carcinoma  simplex  the  clinical  features  are  practically  the  same  as 
in  the  case  of  scirrhus,  except  that  the  growth  is  more  rapid.  The 
tumor  is  rounded,  nodular,  very  hard,  and  is  apt  to  be  much  larger 
than  in  the  scirrhus,  causing  marked  prominence  of  the  breast. 

Microscopically,  however,  the  appearances  differ  considerably.  The 
epithelial  cells  are  larger,  more  rounded,  and  with  relatively  more 
protoplasm.  They  are  also  much  more  abundant,  both  actually  and 
relatively,  so  that  cells  and  stroma  are  about  equal  in  amount.  As  a 
rule,  the  growth  tends  to  assume  an  alveolar  type. 

The  medullary  or  encephaloid  carcinoma  is  much  more  rapid  in  its 
development  than  the  scirrhous  form,  and  may  attain  a  large  size.  It 
is  soft,  vascular,  rounded  in  form,  and  cannot  be  differentiated  on  pal- 
pation from  the  breast  substance.  It  offers  to  the  hand  a  sensation  of 
fluctuation.  Retraction  of  the  nipple  does  not  occur. 

Histologically,  the  epithelial  elements  are  abundant  and  the  stroma 
is  reduced  to  fine  delicate  fibrillse.  In  all  the  softer  carcinomas  the 
interstitial  tissue  is  looser  and  more  cellular  than  in  the  scirrhous  form. 
It  is  common,  therefore,  to  find  a  marked  cellular  infiltration  at  the 
periphery  of  the  growth  and  round  about  it.  These  cells  do  not  suggest 
the  character  of  exudate  cells  or  leukocytes,  but  are  of  the  granulation 
type,  with  a  single  round  nucleus. 

Macroscopically,  the  softer,  more  acinous,  carcinomas  are  somewhat 
nodular  on  section  and  of  a  grayish-red  color.  A  milky  juice  may  be 
obtained  on  scraping.  The  connective  tissue  between  the  cancerous 
masses  appears  as  grayish,  glistening  bands.  The  medullary  form,  how- 
ever, is  pulpy  and  brain-like. 

Occasionally,  the  carcinoma  simplex  undergoes  a  colloid  or  gelatinous 
degeneration  (c.  gelatinosum).  This  occurs  chiefly  in  the  older  portions 
of  the  growth,  while  the  periphery  presents  the  ordinary  features  of 
carcinoma.  Here,  too,  there  are  nodular  and  diffuse  forms. 

In  the  larger  masses  of  epithelial  cells  it  is  not  unusual  to  find  fatty 
degeneration  and  even  extensive  necrosis  of  the  central  portions.  Hya- 
line degenerafion  of  the  fibrous  stroma  and  calcification  are  rare. 

Adenocarcinoma. — This  is  a  term  applied  to  a  growth  having  a  special 
histological  structure.  The  general  resemblance  to  the  acinous  and 
lobular  arrangement  of  the  normal  gland  may  be  fairly  well  preserved. 
The  epithelial  cells,  however,  have  proliferated  into  the  lumen  of  the 
ducts  and  into  the  lymphatic  spaces.  The  appearances  vary  in  different 
portions  of  the  tumor.  In  one  part  there  may  be  normal  gland-tissue; 
in  another,  numerical  increase  of  the  ducts  with  enlargement  and  dila- 
tation and  some  proliferation  of  the  connective  tissue.  The  amount  of 
carcinomatous  invasion  of  the  stroma  varies  considerably  in  different 
cases.  At  times  there  may  be  scarcely  any.  In  other  cases  the  epi- 
thelial elements  extend  into  the  lymph-channels  and  form  small  clusters. 
The  stroma  is  never  so  dense  and  fibrillar  as  in  the  case  of  the  scirrhus. 
The  epithelial  cells  are  similar  to  those  of  the  normal  acini,  but  are,  as 
a  rule,  larger  and  polymorphous. 


902  THE  MAMMARY  GLAND 

Clinically,  the  growth  is  rather  hard,  and  a  serous  discharge  from  the 
nipple  is  not  uncommon. 

One  or  two  cystic  forms  of  carcinoma  should  be  mentioned,  although 
they  are  rare.  In  the  first  variety,  there  is  a  single  main  cyst  with  smooth 
walls,  varying  in  size  from  that  of  a  walnut  to  that  of  an  apple.  At 
one  point  of  its  surface  there  crops  out  a  grape-like  or  papillomatous 
mass  sometimes  provided  with  a  pedicle.  The  warty  excrescences  may 
be  cedematous  and  juicy  and  on  microscopic  examination  prove  to  be 
carcinomatous,  being  composed  largely  of  columnar  cells.  Orth  thinks 
that  the  growth  originates  in  a  milk-duct,  which  then  becomes  dilated 
and  finally  hypertrophied.  In  other  cases  we  have  a  carcinomatous 
growth  combined  with  numerous  small  cysts  of  the  proliferation  type 
(cystadenocarcinoma) . 

There  can  be  little  doubt  that,  with  the  exception  of  epithelioma  and 
the  columnar-celled  variety,  carcinoma  of  the  breast  in  many  instances 
originates  in  the  lobules  of  the  gland,  for  in  the  older  portions  of  the 
growth  the  acinous  arrangement  can  still  often  be  recognized. 

The  epithelial  cells  proliferate,  filling  up  the  lumina  and  leading  to 
dilatation  of  the  spaces.  The  hyaline  membrane  of  the  tunica  soon 
disappears,  although  the  spindle-celled  layer  is  longer  preserved,  and 
finally  the  glandular  elements  break  through  the  membrane  and  appear 
in  the  stroma,  when  extension  continues  along  the  lymphatics.  Once  this 
process  is  well  established  it  soon  extends  into  the  neighboring  lobules, 
which  thus  become  infiltrated  with  carcinoma.  While  only  a  portion  of 
the  lobules  are  likely  to  be  involved  in  the  cancerous  overgrowth,  it  is 
usual  for  both  the  glandular  and  the  interstitial  structures  of  the  rest 
of  the  mamma  to  proliferate. 

Carcinoma  of  the  breast,  if  left  alone,  does  not  remain  confined  to 
the  glandular  substance,  but  gradually  extends  to  the  neighboring 
structures.  The  skin  becomes  involved  and  is  found  to  be  fixed  to  the 
tumor  mass.  It  is  reddened,  inflamed,  and  in  time  the  growth  bursts 
through,  forming  a  foul,  excavated,  and  suppurating  ulcer.  Secondary 
nodules  form  also  in  the  pectoralis  fascia,  the  pectoralis  itself,  and  in 
time  invade  the  thoracic  wall  and  even  the  pleural  cavity  and  the  lung. 
Thus,  the  tumor  becomes  attached  firmly  to  the  thoracic  wall.  This 
extension  takes  place  by  means  of  the  lymphatics,  and  distant  metastases 
are  produced  also  through  the  dissemination  of  small  masses  of  cancer 
cells  through  the  same  channels.  Occasionally,  small,  secondary 
nodules  form  in  the  skin  and  subcutaneous  tissues  followed  by  diffuse 
carcinomatous  infiltration,  whereby  the  anterior  thoracic  wall  becomes 
converted  into  a  stiff,  swollen,  sclercedematous  mass  (cancer  en  cuirasse, 
Panzerkrebs),  which,  in  time,  may  show  superficial  ulceration.  This 
is  rare,  being  only  observed  in  two  cases  of  our  series.  Some  writers 
have  taught  that  the  condition  is  here  confined  to  the  skin,  but  this  is 
undoubtedly  not  the  case. 

The  distant  metastases  are  met  with  first  in  the  lymph-glands  in  closest 
anatomical  relationship  with  the  part  of  the  breast  affected.  As  a  rule, 
the  axillary,  infraclavicular,  and  supraclavicular  glands  are  involved 


PLATE  X 


Neglected   Carcinoma  of    Breast.     (Brewer. 


CYSTS  903 

in  the  order  given,  and  become  hard  and  shotty.  It  should  be  noted, 
however,  that  the  enlargement  is  not  invariably  due  to  metastatic  deposit, 
for,  in  the  early  stages  at  least,  it  may  be  caused  by  inflammation  or  some 
other  as  yet  undetermined  irritation.  We  have  more  than  once  seen 
caseous  tuberculosis  of  the  axillary  glands  in  association  with  carcinoma 
of  the  breast.  When  the  growth  originates  in  the  inner  portion  of  the 
breast  the  glands  of  the  anterior  mediastinum  are  liable  to  be  involved, 
or  even  those  of  the  opposite  axilla.  In  the  former  case  extension  to  the 
liver  may  follow.  The  carcinomatous  invasion  begins  at  the  periphery 
of  the  nodes,  which  may  become  fused  together,  and  may,  in  time, 
extend  to  the  neighboring  tissues.  The  type  of  growth  produced  in  the 
glands  is  not  necessarily  identical  with  that  of  the  original  tumor.  Vis- 
ceral metastases  rarely  arise  until  after  the  involvement  of  the  lymphatic 
glands  is  well  marked.  They  occur  usually  in  the  lungs,  liver,  and 
brain,  and  are  most  probably  hematogenic  in  origin.  Metastatic  in- 
volvement of  the  bones  also  is  hematogenic,  and  is  relatively  more  common 
in  the  scirrhous  or  sclerosing  cancer.  The  bones  affected  are  the  head 
of  the  humerus  on  the  same  side  as  the  original  tumor,  the  vertebrae, 
the  sternum,  and  the  upper  end  of  the  femur.  The  growth  begins  in 
the  medulla,  but  the  shaft  may  in  time  be  eroded,  so  that  spontaneous 
fractures  or  compression  of  the  spinal  cord  sometimes  occur. 

It  might  further  be  remarked,  as  a  matter  of  interest,  that  the  mammse 
are  not  infrequent  sites  for  multiple  independent  growths.  We  have 
twice  observed  simple  carcinoma  attacking  both  breasts  simultaneously. 
These  multiple  growths  are  not  always  of  the  same  type,  however;  epi- 
thelioma  has  been  observed  in  one  breast  and  glandular  carcinoma  in  the 
other.  Two  cases  also  have  been  reported  of  carcinoma  in  one  breast 
and  angiosarcoma  in  the  other. 

Caseous  tuberculosis,  with  epithelioid,  giant  cells,  and  tubercle  bacilli, 
has  been  found  in  mammary  carcinomas  (Warthin1). 

Cysts. — The  simple  cysts  found  so  often  in  the  involution  period  of 
the  breast  and  the  cysts  associated  with  sarcoma  and  carcinoma  have 
already  been  referred  to.  Besides  these,  we  have  as  rarities  Echinococcus, 
Cysticercus,  and  Dermoid  cysts. 

Of  more  importance  are  those  dilatations  of  the  milk  ducts  and  acini 
that  contain  milk  (galactocele).  These  occur,  of  course,  only  in  the 
functioning  breast.  When  obstruction  takes  place  in  a  large  duct  near 
the  nipple,  a  cord-like  swelling  can  be  felt  beneath  the  areola  which 
gradually  extends  toward  the  periphery  of  the  breast.  In  the  event  of 
obstruction  in  the  lobules  the  enlargement  is  more  deep-seated.  There 
is  no  inflammation  and  but  little  pain.  The  increase  at  first  is  rapid, 
but  diminution  in  size  may  occur  after  lactation  has  ceased.  In  some 
cases  single  large  cysts  are  produced  having  an  oval  shape  and  smooth 
outline.  Occasionally,  if  the  wall  of  the  cyst  has  given  way  at  some 
part  and  its  contents  have  escaped  into  the  surrounding  tissues,  the 
cyst  is  more  lobulated.  Scarpa  has  recorded  a  well-known  case  in 

1  Amer.  Jour.  Med.  Sci.,  118  : 1899  :  25. 


904  THE  MAMMARY  GLAND 

which  the  cyst  contained  ten  pounds  of  milk.  After  a  time  absorption 
of  the  more  fluid  parts  of  the  milk  may  occur,  and  the  cyst  is  found  to 
contain  a  creamy  or  butter-like  substance.  Occasionally  these  cysts 
suppurate  or  hemorrhage  occurs  into  the  cavity. 

The  male  breast  is  liable  to  the  same  affections  as  the  female,  but,  of 
course,  much  less  frequently,  having  regard  to  its  ordinarily  rudimentary 
condition.  Chronic  mastitis  and  many  forms  of  tumors  have  been 
met  with. 

In  relative  frequency  as  compared  with  those  of  the  female  breast  they 
are  as  3  to  100.  The  most  common  tumor  is  carcinoma,  generally  of  the 
simple  type.1 

From  what  has  been  said  with  regard  to  the  various  forms  of  inflam- 
mation and  tumors  it  will  readily  be  gathered  that  there  must  often  be 
great  difficulty  in  making  a  differential  diagnosis  between  the  various 
conditions.  The  difficulty  is  not  so  great  for  the  morbid  anatomist,  who 
will  rarely  be  in  doubt,  and  in  any  case  will  have  the  microscope  to  help 
him,  but  during  life  the  most  careful  investigation  is  in  all  cases  necessary 
and  even  then  in  many  cases  the  clinician  will  often  be  at  fault.  In 
view  of  the  importance  of  the  subject  we  give  the  accompanying  table 
for  differential  diagnosis,  which  presents  the  chief  anatomical  and 
clinical  features  of  the  conditions  most  likely  to  be  confused. 

1  Warfield,  Carcinoma  of  the  Male  Breast,  Johns  Hopkins  Hospital  Bulletin, 
12  :  1901  :  305. 


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SECTION    VIII. 
THE  TEGUMENTARY  SYSTEM. 


CHAPTEE    XLI. 

THE  SKIN  AND  ASSOCIATED  STRUCTURES. 
THE  SKIN. 

THE  skin  is  a  somewhat  complicated  structure,  composed,  as  it  is,  of 
an  outer  epidermal  layer  of  flattened  and  horny  cells  resting  upon  a 
subcutaneous  cushion  of  fat  and  fibrous  tissue,  in  the  deeper  layers  of 
which  are  the  bloodvessels,  nerves,  tactile  corpuscles,  hair-follicles, 
sudoriparous  and  sebaceous  glands. 

In  accordance  with  its  structure,  the  skin  performs  numerous  and 
important  functions.  It  is  the  organ  of  tactile,  painful,  and  thermic 
sensation.  It  acts  as  an  external  protective  covering,  regulates  the 
bodily  heat,  and  to  some  extent  exercises  excretory  and  respiratory  func- 
tions. 

As  one  would  expect  from  its  structure  and  exposed  position,  as  well 
as  from  the  fact  that  it  is  in  close  physiological  relationship  with  many 
of  the  internal  organs,  such  as  the  heart,  kidneys,  nervous  system,  and, 
indirectly,  the  liver  and  sexual  apparatus,  the  skin  is  susceptible  to  a 
great  variety  of  disturbing  influences,  and  its  disorders  are  consequently 
numerous. 

CONGENITAL  ANOMALIES. 

A  remarkable  and  rare  congenital  affection  is  ichthyosis  congenita, 
a  condition  characterized  by  overgrowth  of  the  epidermis  with  a  marked 
tendency  to  cornification  of  the  superficial  layers  (keratosis).  The  dis- 
ease usually  involves  the  whole  or  the  greater  part  of  the  body,  but  may 
be  localized.  In  the  mildest  form,  there  are  merely  small,  papular  eleva- 
tions about  the  hairs,  due  to  proliferation  and  keratosis  of  the  cells 
at  the  mouths  of  the  follicles  (xerosis,  xeroderma),  and  all  intervening 
grades  may  be  found  up  to  the  production  of  large,  flattened  plaques 
and  scales  of  horny  texture,  so  that  the  skin  resembles  that  of  a  fish 
or  alligator  (ichthyosis  sauroderma).  As  the  growth  of  the  body  goes 
on,  these  scales  become  separated  more  and  more  one  from  the  other,  and 


908  THE  SKIN 

the  skin  becomes  lined  with  fissures  and  furrows,  while  the  scales  turn 
up  somewhat  at  the  edges.  The  fine  hairs  are  implicated  in  the  over- 
growth. The  fingers  and  toes  may  be  so  affected  that  they  remain 
stunted. 

Microscopically,  the  epidermis  is  greatly  thickened,  the  plaques  being 
composed  of  dense,  laminated,  keratinous  material,  which  extends  to  the 
papillae  and  even  into  the  dilated  hair-follicles.  The  cells  in  the  deeper 
layers  of  the  cutis  are  shrunken  looking.  Fragmentary  and  atrophied 
hairs  may  be  found  embedded  in  the  horny  substance. 

Absence  of  the  normal  pigment  of  the  skin  is  a  not  uncommon  con- 
genital peculiarity,  known  as  leukoderma  or  leukopathia  congenita  or 
albinism,  as  opposed  to  leukopathia  acquisita  or  vitiligo. 

FIG.  238 


Pigmented  nevus.     (Hyde.) 

Albinism  may  affect  the  pigmented  structures  of  the  body  as  a  whole, 
skin,  hair,  iris,  and  choroid.  The  affected  persons,  called  albinos, 
have  clear,  white  or  rosy  transparent  skins,  white  or  yellowish-white 
silky  hair,  and  pink  eyes.  In  partial  albinism  there  may  be  whitish 
streaks  on  the  skin  in  various  parts  of  the  body.  The  condition  is  said 
to  be  hereditary. 

An  excess  of  pigment  is  found  in  certain  of  the  nevi  or  birthmarks 
(naevi  pigmentosi).  They  vary  in  color  from  pale  brown  to  black. 

Congenital  hypertrophy  of  the  nails  (hyperonychia)  is  found  associated 


CIRCULATORY  DISTURBANCES 


909 


with  ichthyosis.  Absence  or  imperfect  development  of  the  nails  is  also 
met  with. 

The  hair  may  be  abnormally  scanty  or,  rarely,  completely  absent 
(alopecia  congenita  universalis).  This  is  often  accompanied  by  imperfect 
development  of  the  teeth  and  nails. 

Excess  of  hair,  hypertrichosis  (hirsuties,  polytrichia),  may  be  local  or 
involve  the  body  as  a  whole.  Universal  hypertrichosis  is  usually  inherited 
and  affects  several  individuals  in  the  same  family.  The  whole  body, 
except  perhaps  the  palms  of  the  hands  and  the  soles  of  the  feet,  may 
be  covered  with  long  hair,  giving  the  individual  a  striking  resemblance 
to  certain  of  the  lower  animals  (hairy  men,  dog- faced  or  baboon  men). 
Local  hypertrichosis  is  met  with  in  the  ncevus  pilosus,  and  on  the  sacrum 
in  association  with  concealed  spina  bifida. 

FIG.  239 


Extensive  verrucose  and  "port-wine"  nevus:   macrodactyly  and  microdactyly. 
(Dr.  A.  E.  Vipond's  case.) 

A  common  anomaly  of  the  skin  is  the  so-called  ''birthmark"  or  nevus, 
which  is  met  with  in  the  form  of  large  or  small,  well-defined,  reddish  or 
purple  patches  (ncevus  vasculosus,  port-wine  stain),  soft,  nodular  excres- 
cences or  warts  (ncevus  verrucosus),  or  local  diffuse  thickenings  of  the  skin 
(elephantiasis).  In  all  these  there  is  a  local  anomaly  in  the  arrangement 
and  development  of  the  bloodvessels.  Some  nevi,  again,  are  level  with 
the  skin  (ncevus  spilus);  others  hairy  (ncevus  pilosus). 

Seborrhcea,  or  excessive  secretion  of  the  sebaceous  follicles,  is  occasion- 
ally met  with  at  birth  and  afterward.  Here,  the  vernix  caseosa  or 
smegma,  which  is  normally  present  on  the  skin  of  the  newborn  infant, 
persists  into  later  life. 


CIRCULATORY  DISTURBANCES. 

The  amount  of  blood  in  the  skin  varies,  of  course,  widely  at  different 
times,  even  under  physiological  conditions,  as,  for  instance,  under  the 
influence  of  exercise,  heat,  cold,  and  emotion.  Pathological  hyperemia 
occurs  as  a  diffuse  blush  over  an  extended  area,  or  in  small  spots  and 
patches. 


910  THE  SKIN 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  is  found  in  the 
first  stage  of  inflammation,  in  vasomotor  disturbances,  exposure  to  exces- 
sive heat  or  cold  (erythema  pernio),  and  as  a  result  of  slight  injuries, 
such  as  are  caused  by  mechanical  or  chemical  irritation.  Large  patches 
of  hyperemia  are  termed  erythema;  small  spots,  roseola.  The  color 
is  a  pale  rose  pink,  and  disappears  on  pressure,  only  to  return  instantly 
when  the  pressure  is  removed.  Erythema  is  not  infrequently  associated 
with  exudation  of  plasma  and  swelling  (inflammatory  oedema),  and  when 
long-continued  or  repeated  may  lead  to  pigmentation  of  the  skin,  owing 
to  diapedesis  of  the  red  cells  and  metamorphosis  of  the  hemoglobin. 

Passive  Hyperemia. — Passive  hyperemia  is  well  seen  in  chronic  valvular 
disease  of  the  heart  and  other  conditions  which  favor  blood-stasis,  such 
as  pneumonia,  toxic  states,  and  sunstroke.  The  lips,  face,  neck,  and  the 
extremities  often  present  a  diffuse  dusky  blue  or  leaden  color  (cyanosis). 
A  small  spot  of  cyanosis  or  lividity  is  termed  livedo.  After  death  the 
blood  stagnates  to  the  dependent  parts  of  the  body  (postmortem  lividity). 

Local  passive  congestion  of  the  skin  may  be  brought  about  by  the 
pressure  of  tumors  or  inflammatory  products  on  the  efferent  vessels  of 
a  part. 

(Edema. — The  natural  result  of  prolonged  passive  congestion  is 
oedema.  This  is  found  more  especially  in  connection  with  chronic 
stasis  in  the  blood-  or  lymph-systems.  The  skin  and  subcutaneous 
structures  are  infiltrated  with  plasma,  are  firmer  than  normal,  and  pit 
on  pressure.  The  skin  is  commonly  tense  and  shiny.  In  severe  cases 
blisters  are  formed,  or  the  skin  may  burst  through  overdistension  and  the 
fissures  weep  clear,  watery  fluid.  Secondary  infection  and  inflamma- 
tion are  not  uncommon  sequels. 

Angioneurotic  (Edema. — Angioneurotic  oedema  is  a  vasomotor  disturb- 
ance of  the  skin  and  subcutaneous  tissues  found  in  neurotic  individuals. 
It  is  notably  a  hereditary  affection,  reappearing  in  several  generations. 
It  is  characterized  by  the  sudden  onset  of  local  swellings,  generally 
about  the  eyelids,  ears,  lips,  or  cheeks,  but  which  may  also  be  found  in 
the  hands,  feet,  breast,  genitalia,  or  back.  The  attack  may  be  preceded 
by  slight  itchiness  and  redness  of  the  skin.  The  condition  may  shift 
about  from  one  place  to  another,  and  usually  passes  off  as  suddenly  as 
it  came.  According  to  Osier,  giant  urticaria  is  the  same  disease. 

Teleangiectasis. — Dilatation  of  capillaries  (teleangiectasis)  is  due  to 
obstruction  to  the  free  outflow  of  blood  from  any  part,  as,  for  instance, 
from  the  pressure  of  tumors  or  contracting  fibrous  tissue  on  the  efferent 
vessels. 

Rosacea,  a  condition  due  to  the  dilatation  of  the  superficial  capillaries, 
is  met  with  more  especially  in  those  addicted  to  alcohol,  or  who  are 
exposed  to  wind  and  weather. 

Hemorrhage. — Hemorrhage  into  the  skin  is  commonly  the  result  of 
traumatism,  or  is  a  symptom  in  certain  of  the  infectious  fevers.  Not 
infrequently,  too,  it  comes  on  spontaneously  or  "idiopathically." 

Hemorrhages  vary  greatly  in  size,  are  of  reddish  or  purplish-red  color, 
and  do  not  disappear  on  pressure.  Small,  irregular  spots  about  the  size 


PURPURA 


911 


of  a  pin-head  are  termed  petechiae.  Elongated  streaks  or  branching  lines 
are  called  vibices.  Large,  irregular  patches  of  considerable  superficial  ex- 
tent are  called  ecchymoses.  Occasionally,  the  amount  of  blood  effused  is 
sufficient  to  produce  nodules  (purpura  papulosa)  or  actual  tumor  (hema- 
toma).  In  some  cases  the  epidermis  is  elevated,  forming  a  blood  blister. 
The  blood  may  also  be  effused  into  the  sweat-glands,  causing  bloody 
perspiration  (hematidrosis).  The  extravasation  takes  place  into  the 
corium  or  papillary  layer. 

In  course  of  time  the  effused  blood,  which  is  at  first  reddish,  is  trans- 
formed, and,  as  in  the  familiar  instance  of  the  "black"  eye,  the  affected 
patch  passes  through  all  stages  of  reddish-brown,  brown,  yellowish- 
green,  and  yellow.  In  many  cases,  the  blood  is  completely  absorbed 
and  the  only  trace  of  its  presence  may  be  a  little  pigmentation  of  the 
skin,  due  to  the  deposit  of  hemosiderin. 


FIG.  240 


Purpura  rheumatica.     (From  the  Montreal  General  Hospital.) 

Purpura. — Spontaneous  hemorrhage  is  usually  included  under  the 
general  term  purpura.  By  this  is  meant  a  condition  in  which  there  are 
multiple  hemorrhages  in  the  skin,  either  petechial  or  ecchymotic,  some- 
times associated  with  bleeding  from  the  various  mucous  surfaces,  such 
as  the  nose,  lips,  gums,  stomach,  intestines,  kidneys,  or  uterus. 


912  THE  SKIN 

Purpura  may  be  conveniently  divided  into  the  following  types  i1 

1.  Essential  purpura,  including  peliosis   rheumatica,  morbus   Werl- 
hofii,  purpura  simplex,  purpura  urticans,  and,  possibly,  scorbutus. 

2.  Symptomatic  purpura,  such  as  is  found  in  the  infectious  fevers, 
typhus,  variola,  scarlatina,  measles,  bubonic  plague,  sepsis,   typhoid 
fever,  and  icterus  gravis. 

3.  Cachectic  purpura,  in  pernicious  anemia,  Bright's  disease,  leukemia, 
and  carcinoma. 

4.  Toxic  purpura,  as  in  snake  bite  and  poisoning  with  phosphorus, 
antipyrine,  copaiba. 

5.  Multiple  sarcomatosis   of   the   vessels.2    To   which   may  perhaps 
be  added, 

6.  Neuropathic  purpura,  as  in  hysterical  "stigmata." 

No  doubt,  "purpura"  ought  to  be  regarded  merely  as  a  symptom, 
for  it  may  be  produced  by  a  variety  of  causes.  As  a  rule,  more  than  one 
cause  is  at  work.  It  is  safe  to  say  that  in  all  cases,  save  possibly  the 
neuropathic,  there  is  some  abnormal  condition  of  the  walls  of  the  smaller 
vessels  and  capillaries,  such  as  fatty  degeneration,  which  leads  to  hemor- 
rhage per  diapedesin  or  per  rhexin.  Actual  rupture  of  the  vessels  is 
probably  rare,  but  has  been  demonstrated.  The  importance  of  diseased 
vessels  in  the  production  of  these  hemorrhages  is  well  seen  in  elderly 
persons  with  arterial  sclerosis,  who  sometimes  develop  purpuric  spots 
on  the  lower  extremities.  In  some  cases  thrombi  and  emboli  have  been 
found  obstructing  the  vessels.  The  ring-shaped  petechise,  occasionally 
seen,  are  of  this  nature.  Or,  again,  as  in  a  case  of  purpura  complicating 
acute  endocarditis,  which  one  of  us  (A.  G.  N.)  studied,  the  condition 
is  due  to  hematogenous  infection  (mycotic  infarct),  the  minute  vessels 
of  the  parts  being  filled  with  bacteria  and  surrounded  by  leukocytic 
infiltration. 

The  majority  of  cases,  including  all  the  symptomatic  and  probably 
some  of  the  essential  purpuras,  are  the  result  of  infective  processes. 
Some  of  the  cachectic  forms,  notably  those  occurring  in  nephritis, 
cancer,  and  leukemia,  are  possibly  to  be  attributed  to  terminal  infection. 
The  toxic  forms  are  most  likely  due  to  profound  changes  in  the  blood, 
which  lead  to  rapid  disintegration  of  the  vessel  walls,  or  to  slower  hyaline 
and  fatty  degeneration.  In  many  instances,  however,  a  combination 
of  factors  is  at  work.  Circulating  toxins,  of  whatever  kind,  may  cause 
degeneration  of  the  vessel  walls,  with  consecutive  dilatation,  together 
with  a  diminution  of  the  coagulating  power  of  the  blood.  Local  obstruc- 
tion, as  from  thrombosis  or  embolism,  will  tend  to  damage  the  vessel 
walls  and  raise  the  blood  pressure  at  that  point,  so  that  dilatation  and 
rupture  readily  take  place. 

Anemia. — Anemia  of  the  skin  is  manifested  by  pallor.  General 
anemia  is  one  of  the  commonest  pathological  conditions,  being  found  in 

1  Nicholls  and  Learmonth,  The  Hemorrhagic  Diathesis  in  Typhoid  Fever  and  its 
Relationship  to  Purpuric  Conditions  in  General,  Lancet,  London,  1 : 1901:305. 

2  Martin  and  Hamilton,  Jour.  Exper.  Med.,  1:1896:4. 


DERMATITIS, 


913 


chlorosis,  pernicious  anemia,  leukemia,  hemorrhage,  after  fevers,  and  in 
all  chronic  wasting  diseases.  Local  anemia  is  due  to  exposure  to  cold, 
pressure,  or  may  be  neuropathic,  as  in  neuralgia  and  fainting. 


INFLAMMATIONS. 

An  entirely  satisfactory  classification  of  the  various  forms  of  derma- 
titis, or  inflammation  of  the  skin,  has  yet  to  be  made.  This  is  accounted 
for  by  the  fact  that  authorities  have  not  always  agreed  as  to  the  lesions 
present  in  any  given  case,  nor  as  to  the  interpretation  of  the  appearances. 
Inflammations  of  the  skin  are  of  the  most  protean  character.  One  and 
the  same  cause  may,  on  occasion,  give  rise  to  the  most  diverse  clinical 
manifestations,  and,  conversely,  one  definite  clinical  picture  may  be 
the  result  of  widely  differing  etiological  factors.  Again,  inflammation 
may  originate  not  only  in  the  skin,  but  in  its  appendages,,  and  in  the  sub- 
cutaneous tissues.  In  the  rarer  affections,  moreover,  the  exact  sequence 
of  events  has  not  always  been  made  out.  In  many  cases,  finally,  the 
cause  or  causes  is  obscure  or  quite  unknown.  The  classification  which 
we  here  present  is  simple  and  convenient,  and,  we  believe,  in  harmony 
with  the  facts  as  they  are  at  present  known. 

Owing  to  its  exposed  position  and  its  function  as  a  protective  covering, 
the  skin  is  liable  to  a  great  variety  of  insults,  not  only  from  mechanical 
trauma,  but  from  variations  in  temperature,  the  effects  of  light,  and  the 
irritation  of  chemical  and  other  toxic  substances.  Again,  interference 
with  its  action  as  an  excretory  organ  sometimes  results  in  inflammation. 
The  vascular  system  conveys  to  it  various  microorganisms,  microbic  and 
other  toxins.  Lesions  of  the  nervous  system  often  result  in  congestive 
hyperemia  and  sometimes  inflammation,  or,  again,  in  disorders  of 
nutrition. 

We  may  divide  dermatitis,  or  inflammation  of  the  skin,  into  two  main 
varieties :  primary  or  essential  dermatitis,  in  which  the  lesions  originate 
in  the  skin  or  its  associated  structures,  and  are  confined  to  it;  and 
secondary  or  symptomatic  dermatitis,  in  which  the  cutaneous  mani- 
festations are  simply  one  phase  of  a  generalized  systemic  disorder. 


(a)  Traumatic,  from 

1.  Mechanical  injury. 

2.  Physical  agents,  such  as  light, 

heat,    cold,    moisture,     filth, 
chemical  and   other  external 
toxic  substances. 
(6)  Infectious,  from 

1.  Bacteria,  yeasts,  and  moulds. 

2.  Animal  parasites. 

(c)  Neuropathic. 

(d)  Of  unknown  or  doubtful  etiology. 


Primary  or  Essential  Dermatitis     .     . 


f(a)  Exanthematous  eruptions. 
Secondary  or  Symptomatic  Dermatitis    <*>  *&tt£ 

I          sonous  substances. 
58 


914  THE  SKIN 

The  lesions  produced  by  dermatitis  are  extremely  variable,  depending 
upon  the  nature,  extent,  localization,  and  chronicity  of  the  disease. 
The  cardinal  features  of  inflammation,  namely,  redness,  swelling,  heat,  and 
pain,  are  particularly  well  exemplified  in  the  case  of  the  skin.  Diffuse 
redness  and  swelling  is  termed  erythema.  The  color  is  bright  and  vivid, 
quite  in  contrast  with  the  dull  lividity  of  passive  congestion,  disappearing 
momentarily  on  pressure.  The  more  circumscribed  areas  of  infiltration 
are  known  as  papules,  wheals,  nodes,  or  tubercles.  Papules  are  small 
elevations,  due  to  infiltration  in  the  skin,  which  vary  in  size  from  that  of  a 
millet-seed  to  that  of  a  pea.  Larger  elevations,  up  to  a  hazelnut  in 
size,  are  called  nodes,  nodules,  or  tubercles.  Still  larger  ones  are  some- 
times termed  phyma.  Wheals  are  broad,  flattened  elevations,  quite  well 
defined,  which  appear  and  disappear  rapidly.  They  are  dull  reddish  in 
color,  or,  in  the  case  of  the  larger  ones,  with  whitish,  anemic-looking 
centres.  Histologically,  one  finds  in  such  mild  forms  of  dermatitis,  infil- 
tration of  the  tissues  with  serum,  together  with  diapedesis  of  leukocytes 
and,  occasionally,  of  red  cells.  The  epithelium  is  usually  but  little 
affected,  although  certain  of  the  cells  may  be  swollen  and  hydropic,  and 
there  may  be  slight  proliferation. 

Where  the  serous  exudation  is  more  intense,  local  collections  of  fluid 
occur,  which,  provided  that  the  superficial  epidermis  remain  intact, 
lead  to  the  separation  of  the  outer  layers  from  the  underlying  portions, 
thus  forming  elevations  commonly  known  as  blisters,  blebs,  or  vesicles. 
Such  vesicles  may  be  single  or  lobulated.  They  contain  a  clear,  trans- 
lucent serum,  almost  devoid  of  cellular  elements.  In  other  cases,  the 
fluid  is  slightly  turbid  from  the  admixture  of  leukocytes,  or  reddish  from 
extravasation  of  blood  (blood  blisters).  Not  infrequently,  also,  the 
exudation  is  turbid,  whitish,  and  purulent,  the  vesicle  then  being  known 
as  a  pustule.  When  the  pustule  dries  it  forms  a  crust  or  scab. 

In  other  cases,  where  the  corium  is  markedly  infiltrated  with  fluid, 
the  exudation  spreads  to  the  papillary  layer,  and  finally  to  the  epidermis. 
The  cells  involved  are  swollen,  vacuolated,  and  hydropic,  to  some  extent 
compressed,  and  eventually  dissociated.  When  cornification  is  not 
marked  the  fluid  exudes  upon  the  surface,  where  it  may  coagulate  or 
dry,  giving  rise  to  crusts  or  scabs.  This  exudate  may  be  gelatinous, 
fibrinous,  mixed  with  leukocytes  or  red  cells.  Superficial  defects  in  the 
epidermis,  fissures  or  excoriations,  are  not  uncommon  under  such 
circumstances,  or  even  cracks  or  rhagades,  extending  through  the  entire 
thickness  of  the  skin.  In  more  extreme  conditions,  especially  where  the 
circulation  is  much  interfered  with,  we  get  actual  ulcers,  abscesses,  or 
gangrene. 

Acute  inflammation,  if  mild,  may  quickly  pass  off,  leaving  little  or  no 
traces  behind.  Frequently,  however,  even  after  so  trifling  an  affection  as 
erythema,  the  superficial  epidermis  is  cast  off  or  desquamates.  The 
loosened  cuticle  may  come  away  in  the  form  of  a  fine  white  powder  com- 
posed of  minute  dry  scales  (desquamatio  furfuracea),  or  as  larger,  but 
still  delicate,  whitish,  silvery  flakes,  or,  again,  as  thick,  dirty  white 
shreds  or  membrane  (desquamatio  membranacea).  The  scales  are,  for 


PRIMARY  OR  ESSENTIAL  DERMATITIS  915 

the  most  part,  exfoliated,  horny  epithelium,  but  in  many  instances  it 
is  pathologically  altered  as  well.  Pigmentation  is  also  a  common  sequel 
of  dermatitis,  due  to  the  deposit  of  blood  coloring  matter. 

In  cases  where  there  has  been  a  loss  of  substance,  as,  for  instance,  in 
vesicles,  pustules,  or  fissures,  there  is  a  regeneration  of  the  epithelium 
from  the  cells  at  the  periphery  of  the  lesion,  from  any  remnants  of  the 
epidermis  that  remain,  and  even  from  the  epithelium  of  the  hair-follicles, 
sweat  and  sebaceous  glands.  A  thin,  bluish,  semitranslucent  covering 
is  thus  produced,  which  ultimately  is  converted  into  ordinary  horny  epi- 
thelium. Should,  however,  the  papillary  layer  or  the  corium  be  damaged, 
regeneration  is  not  so  perfect,  and  the  loss  of  substance  is  made  good  by 
the  production  of  new  fibrous  tissue.  The  papillae  are  commonly  not 
reproduced  or  are  stunted,  while  the  superficial  epithelium  is  smooth, 
shiny,  and  largely  or  entirely  devoid  of  hair- follicles  and  glands.  A 
pigmented  scar  is  a  common  result. 

In  chronic  inflammations  atrophy  and  hypertrophy  may  be  combined. 
The  formation  of  epithelium  may  be  in  abeyance,  inadequate,  or  exces- 
sive, and  the  normal  process  of  cornification  may  be  interfered  with  in 
various  ways.  The  papillae  frequently  hypertrophy,  becoming  elongated 
and  branched,  while  the  corium  and  subcutaneous  tissues  are  thickened. 
In  other  cases  the  papillae  are  atrophic  and  flattened,  while  the  corium 
is  thinned. 

Primary  or  Essential  Dermatitis. — Traumatic  Dermatitis. — Trau- 
matic dermatitis  in  its  widest  sense,  may  be  taken  to  include  all  those 
forms  of  inflammation  of  the  skin  due  to  mechanical  injury,  exposure 
to  light,  heat,  or  cold,  and  the  action  of  chemical  and  other  irritating 
substances.  Not  infrequently,  several  etiological  factors  are  combined. 
Owing  to  the  exposed  position  of  the  lesion,  secondary  infection  is  apt 
to  be  superadded.  Thus,  for  example,  in  the  moist  condylomas  found 
so  often  about  the  genitals  we  have  the  combined  effects  of  moisture, 
heat,  dirt,  toxic  irritation,  and  infection.  Dermatitis  is  often  met  with 
in  contusions,  abrasions,  and  lacerations. 

Under  the  heading  of  dermatitis  from  the  effects  of  light  may  be  men- 
tioned the  well-known  x-ray  dermatitis.  In  the  mildest  grades  we  get 
little  more  than  a  transient  irritation,  which,  in  time,  after  repeated 
application  of  the  rays,  is  followed  by  pigmentation,  glossiness  of  the 
skin,  and  loss  of  hair.  Of  the  frank  inflammations,  perhaps  the  com- 
monest is  a  simple  erythema,  which  appears  after  a  variable  period, 
from  a  few  hours  to  some  days.  Vesicular,  bullous,  and  hemorrhagic 
forms  are  more  frequently  met  with  than  are  the  papular  and  pustular 
varieties.  Occasionally,  deep  ulcers  or  eschars  are  formed  which  are 
exquisitely  painful  and  difficult  to  heal.  Weeks,  or  months  or  even 
one  or  two  years  may  elapse  before  cicatrization  is  complete.  Among 
other  effects  may  be  noted,  canities,  and,  rarely,  leukoderma.  Occa- 
sionally the  ulcers  become  malignant. 

Histologically,  our  knowledge  is  incomplete.  Darier,  in  the  milder 
grades  of  the  affection,  found  marked  thickening  of  the  stratum  cor- 
neum;  the  stratum  granulosum  showed  both  hypertrophy  and  hyper- 


916  THE  SKIN 

plasia  of  its  cells,  which  contained  numerous  eleidin  granules;  the  cells 
of  the  stratum  spinosum  were  also  hyperplastic  and  hypertrophic,  pre- 
senting mitoses,  while  the  hair  papillae,  arrectores  pilorum,  and  seba- 
ceous glands  had  disappeared. 

The  simplest  form  of  dermatitis  due  to  heat  (dermatitis  calorica), 
and,  perhaps,  to  some  extent  also,  to  light,  is  the  erythema  caused  by 
exposure  to  the  sun  (sunburn).  Here,  the  injury  has  not  been  sufficient 
to  destroy  the  tissue,  but  has  led  to  relaxation  of  the  bloodvessels, 
congestive  hyperemia,  and  slight  exudation.  After  the  process  has 
subsided,  desquamation,  and  more  or  less  pigmentation  is  apt  to  follow. 
The  susceptibility  to  "sunburn"  varies  greatly  in  different  individuals, 
being  dependent  apparently  to  some  extent  on  the  amount  of  pigment 
normally  in  the  skin.  People  of  fair  complexions  and  those  with  leuko- 
derma  suffer  more  than  others. 

In  all  respects  similar  is  the  inflammation  due  to  burning  in  its  mildest 
degree  (dermatitis  ambustionis  erythematosa,  burn  of  the  first  degree). 
Where  the  injury  has  been  more  severe,  owing  to  a  higher  temperature 
or  a  more  prolonged  contact,  considerable  exudation  takes  place  from 
the  papillary  bodies  beneath  the  epidermis,  which  is  thereby  elevated 
into  vesicles  (dermatitis  ambustionis  bullosa,  burn  of  the  second  degree^. 
Again,  where  there  is  loss  of  substance  of  the  cutis,  we  speak  of  a  burn 
of  the  third  degree,  or  in  the  case  of  charring,  of  a  burn  of  the  fourth 
degree.  Burns  of  the  second  degree  heal,  provided  infection  does  not 
take  place,  with  simple  regeneration  of  the  epithelium.  In  the  cases 
where  there  is  actual  loss  of  substance,  the  burn  heals  by  granulation  and 
the  formation  of  a  scar. 

Closely  allied  to  dermatitis  calorica  is  the  dermatitis  due  to  frost-bite 
(pernio,  dermatitis  congelationis).  Here,  again,  there  may  be  merely  ery- 
thema, with  swelling,  and  later  desquamation,  vesiculation  (dermatitis 
congelationis  bullosa),  or  even  gangrene  (dermatitis  congelationis  gangrce- 
nosa).  In  the  severer  form,  the  affected  part  is  red  and  livid,  later  dark 
red,  and  finally  becomes  surrounded  by  a  line  of  demarcation. 

Inflammation  of  the  skin  may,  also,  be  caused  by  contact  with  a  great 
variety  of  chemical  and  other  toxic  agents  (dermatitis  venenata).  A 
great  number  of  substances,  derived  from  the  vegetable,  animal,  and 
mineral  kingdoms,  may  on  occasion  be  at  fault.  A  few  that  may  be 
mentioned  are  the  poisons  of  poison-ivy  and  poison-oak,  the  nettle, 
the  venom  of  certain  reptiles  and  insects,  various  dyes,  caustic  alkalies 
and  acids,  and  certain  substances  used  in  medical  practice,  such  as 
cantharides,  croton  oil,  turpentine,  mustard,  iodoform,  formalin,  and 
carbolic  acid.  Some  of  these  substances  may  be  absorbed  into  the 
system  and  produce  dermatitis  elsewhere. 

Infectious  Dermatitis  of  Bacterial  Origin. — Under  this  heading  we 
may  conveniently  and  properly  include  all  those  inflammations  of  the 
skin  and  subcutaneous  tissues  due  to  vegetable  parasites,  which  are 
not  dependent  on  constitutional  disease.  Dermatitis  in  this  sense 
may  be  local  or  diffuse. 

A  local  dermatitis,  involving  all  the  elements  of  the  skin  and  its 


INFECTIOUS  DERMATITIS  OF  BACTERIAL  ORIGIN 


917 


related  structures,  is  common  as  a  result  of  wounds,  bruises,  blisters, 
and  abrasions,  particularly  when  they  have  become  infected. 

Hospital  gangrene,  a  disease  probably  never  met  with  now,  but  com- 
mon under  the  unhygienic  conditions  of  former  days  and  before  the 
advent  of  antiseptic  surgery,  was  a  form  of  gangrene  which  was  liable 
to  attack  even  the  most  trifling  wounds.  The  tissues  at  the  edge  and 
in  the  immediate  neighborhood  of  the  wound  assumed  a  dirty  yellowish- 
gray  color  and  were  converted  into  a  foul,  slimy  mass.  The  destruction 
of  tissue  was  rapid,  and  the  necrotic  material  was  cast  off  in  the  form 
of  an  offensive,  shreddy,  serous  discharge. 

FIG.  241 


Impetigo  contagiosum.     (Hyde.) 


Impetigo,  formerly  divided,  but  erroneously  so,  into  impetigo  simplex 
and  impetigo  contagiosum,  is  a  pustular  dermatitis,  found  in  badly 
nourished  children  who  live  under  unhygienic  conditions.  It  is  due 
to  infection  with  pyogenic  cocci.  The  pustules  are  found  in  the  derma. 
They  do  not  tend  to  infiltrate,  but  the  infection  may  be  carried  from 


918  THE  SKIN 

one  part  to  another  by  scratching.  Uncomplicated  cases  heal  without 
scarring.  The  disease  may  become  chronic,  and  may  result  in  the 
formation  of  vesicles,  pustules,  and  crusts,  with  desquamation  of  the 
epidermis.  It  chiefly  affects  the  head  and  extremities. 

Ecthyma  is  a  more  severe  form  of  impetigo.  The  pustules  are  larger, 
the  infiltration  is  more  extensive.  It  is  rare  to  have  scarring,  but  pig- 
mentation may  result. 

The  soft  chancre  (ulcus  molle,  chancroid,  venereal  ulcer)  is  a  local 
infectious  inflammation  of  the  skin  and  mucous  surfaces,  transmitted 
from  one  person  to  another  by  immediate  contagion,  usually  by  coitus. 
Within  twenty-four  hours  of  the  infection  there  appears  on  some  part 
of  the  genitalia  a  small  vesicle  or  pustule,  which  rapidly  breaks  down 
into  an  ulcer.  The  base  and  edge  are  yellowish,  soft,  and  purulent, 
and  the  ulcer  is  bounded  by  a  reddish  hyperemic  zone. 

Microscopically,  one  sees  extensive  infiltration  of  the  tissues  with  in- 
flammatory round  cells,  the  more  superficial  of  which  are  in  various 
stages  of  degeneration  and  molecular  disintegration. 

Under  suitable  treatment,  soft  chancre  heals  with  the  formation  of  a 
small  scar.  Lymphangitis  and  inguinal  lymphadenitis  (bubo)  are  not 
infrequent  complications  of  this  form  of  ulceration.  Syphilis  does  not 
result  except  in  instances  where  there  is  mixed  infection.  In  such  cases 
the  chancroid  does  not  disappear,  but  in  three  or  four  weeks  is  con- 
verted into  the  true  chancre  (hard  chancre,  ulcus  induratum).  Soft 
chancres  are  apt  to  be  multiple,  and  the  virus  may  be  transplanted  from 
one  part  to  another.  A  small  bacillus,  described  by  Ducrey,  has  by 
some  been  regarded  as  the  specific  cause  of  the  affection.  Pyogenic 
cocci  are,  however,  to  be  found  in  the  ulcer  as  well,  and  are  present 
exclusively  in  the  buboes. 

Chancre  (true  chancre,  hard  chancre,  ulcus  induratum)  is  the  primary 
manifestation  of  syphilis  in  the  skin  and  mucous  membranes.  It  appears 
usually  from  ten  to  thirty  days  after  infection,  and  may  assume  several 
forms.  In  some  cases  a  sharply  defined,  firm  area  of  infiltration  is  found 
beneath  the  superficial  epithelium,  composed  of  an  accumulation  of 
small  round  cells,  and  sometimes  large  epithelioid  and  giant  cells.  In 
other  instances,  the  lesion  begins  as  a  papule,  the  size  of  a  shot  or 
larger,  of  dark  bluish  or  pale  red  appearance.  At  first  it  is  hemi- 
spherical, but  tends  gradually  to  spread  laterally.  When  on  parts  of 
the  body  that  are  kept  dry  the  superficial  epidermis  is  heaped  up  and 
desquamates,  while  the  surface  may  be  covered  with  a  scab.  On 
moist  situations  the  chancre  is  soft  and  moist  also.  Owing  to  loss  of 
substance  an  ulcer  may  result.  When  healing  takes  place  small 
whitish  flecks  and  scars  are  left  behind,  or  firm  fibrous  papules.  The 
typical  hard  chancre  begins  as  a  papule  or  vesicle.  Very  soon  the 
superficial  epithelium  is  cast  off  and  a  shallow  erosion  is  the  result. 
The  ulcer  extends  into  the  corium,  is  sharply  defined,  with  smooth 
base  and  clean-cut  edges.  Infiltration  is  usually  marked  and  so  that 
when  the  ulcer  is  palpated  laterally,  it  feels  as  if  there  were  a  bit  of 
parchment  in  the  base.  The  ulcer  is  quite  indolent,  and  does  not 


INFECTIOUS  DERMATITIS  OF  BACTERIAL  ORIGIN  919 

tend  to  spread.  It  is  single,  and  the  virus  cannot  be  inoculated  in  any 
other  part  of  the  body.  The  secretion  is  scanty,  consisting  of  thin  pus. 
Rarely,  granulation  tissue  forms  in  abundance  and  small  papillary 
excrescences  are  found  on  the  base.  Occasionally,  when  the  ulcer  is 
infected  secondarily  with  pus  cocci,  it  resembles  closely  the  soft  chancre, 
but  the  course  of  the  affection  and  the  sequelae  will  distinguish.  The 
hard  chancre  heals  with  the  formation  of  a  scar,  but  induration  persists 
for  a  long  time.  Recently,  the  specific  organism  of  syphilis,  the  Spiro- 
chseta  pallida,  has  been  detected  repeatedly  in  scrapings  from  chancres 
and  in  the  tissues  about  them  (Schaudinn,  Hoffmann,  Levaditi,  Buschke 
and  Fischer,  Burent  and  Vincent). 

Tuberculosis  of  the  skin  is  primary  and  secondary.  Primary  tuber- 
culosis includes  the  "postmortem  wart"  (verruca  tuberculosa)  and 
lupus  vulgaris.  The  secondary  forms  are  the  so-called  scrofuloderma 
of  the  older  writers,  and  certain  ulcers  of  the  skin  and  mucous  surfaces 
about  the  various  orifices  of  the  body,  found  sometimes  in  tuberculous 
subjects. 

The  verruca  tuberculosa,  or  anatomist's  wart,  is  occasionally  found 
in  those  who  are  brought  into  close  contact  with  the  bodies  of  individuals 
affected  with  tuberculosis,  such  as  morbid  anatomists,  surgeons,  and 
butchers.  Washerwomen  have  also  been  known  to  become  infected 
from  handling  infected  linen.  Human  beings  are  usually  infected 
with  bacilli  of  human  derivation,  but,  as  Ravenel  has  shown  recently, 
butchers  may  be  infected  from  bovine  tubercle.  The  bacilli  enter 
through  punctures  and  incised  wounds,  or  through  abrasions.  The 
wart  appears  as  a  small,  rough,  elevated  papule,  of  purplish  color,  which 
is  extremely  indolent  in  its  development. 

Microscopically,  there  is  overgrowth  of  the  papillse,  with  hypertrophy 
and  desquamation  of  the  epidermis.  Tubercles  are  found  in  the  rete 
and  in  the  subepithelial  tissues.  Bacilli  are  particularly  scanty. 

Should  infection  with  pyogenic  cocci  take  place  at  any  time,  a  deeper, 
more  widely-spreading,  ulceration  takes  place,  with,  possibly,  a  lym- 
phangitis. Systemic  infection  with  death  from  disseminated  tubercu- 
losis has  been  known  to  occur.  The  skin,  however,  appears  to  be  a 
particularly  unfavorable  soil  for  the  growth  of  the  tubercle  bacillus,  so 
that  the  resulting  lesion  remains  for  a  long  time  strictly  localized. 

Lupus  vulgaris  has  now  been  demonstrated  beyond  question  to  be 
a  chronic  tuberculosis  of  the  skin  and  subcutaneous  tissues.  Two 
processes  are  at  work,  one  of  destruction  of  tissue,  and  one  of  hyper- 
plasia,  and  the  disease  assumes  several  clinical  forms  according  to  the 
relative  predominance  of  one  or  the  other.  The  affection  usually 
begins  in  early  childhood  and  may  last  for  many  years.  The  lesion 
is  single,  more  rarely  multiple,  and  is  commonly  found  onTthe  face, 
less  frequently  on  the  extremities  and  trunk. 

The  process  begins  in  the  lower  layers  of  the  corium  with  the  forma- 
tion of  typical  tuberculous  granulomas.  These  in  time  undergo  necrosis 
and  may  become  absorbed,  but  commonly  extend  through  to  the  surface, 
so  that  open  ulcers  discharging  pus  and  covered  with  crusts  are  pro- 


920  THE  SKIN  ' 

duced.  The  older  foci*  show  central  caseation,  and  the  process  spreads 
by  the  coalescence  of  neighboring  granulomas  and  the  formation  of 
new  ones  at  the  periphery.  Some  of  the  granulomas  may  be  absorbed, 
while  others,  after  the  discharge  of  the  necrotic  material,  heal  with  the 
formation  of  dense  fibrous  scars.  Thus,  the  picture  is  presented  of 
scarring  and  more  or  less  distortion  of  the  parts  at  the  centre  of  the 
lupus  patch,  while  at  the  margin  the  disease  is  active  and  progressive. 
The  connective-tissue  hyperplasia  may  be  so  extreme  that  a  form  of 
elephantiasis  results. 

FIG.  242 


Tuberculous  granulation  tissue.  A  node  .of  round-celled  infiltration  with  two  giant  cells. 
An  early  stage.  Zeiss  obj.  A,  without  ocular.  (From  the  collection  of  the  Royal  Victoria 
Hospital.) 

Microscopically,  there  is  marked  infiltration  with  round  cells.  Giant 
cells  are  fairly  numerous,  but  epithelioid  cells  are  scanty.  Tubercle 
bacilli  are  but  few  in  numbers.  The  sebaceous  glands  are  often 
filled  with  inflammatory  cells,  and  the  various  glands  and  follicles 
show  varying  grades  of  atrophy  and  destruction.  The  epithelium  is 
involved  secondarily,  the  cells  of  the  rete  being  swollen,  degenerated, 
or  in  other  cases  proliferating.  The  papillae  are  frequently  enlarged 
and  extend  downward,  suggesting  in  appearance  an  epithelioma, 
which,  indeed,  in  some  cases  is  superadded.  In  other  parts  the  epithelial 
layer  is  thinned,  atrophic,  and  even  destroyed. 

According  to  the  gross  appearance  presented,  several  clinical  forms 
have  been  recognized.  In  the  early  stages,  before  there  is  destruction 
of  tissue,  the  tubercles  in  the  cutis  may  be  indicated  by  reddish  or 
yellowish-brown,  smooth  or  scaly  spots  (lupus  maculosus).  Later, 


INFECTIOUS  DERMATITIS  OF  BACTERIAL  ORIGIN 


921 


several  foci  are  in  close  proximity,  the  central  portion  of  the  area,  owing 
to  absorption,  becomes  depressed,  and  the  skin  over  it  is  brownish- 
red  or  brownish-yellow,  fissured,  and  desquamating  (lupus  exfoliativus). 
When  loss  of  substance  has  taken  place,  so  that  an  open  ulcer  secreting 
pus  and  covered  with  crusts  is  produced,  we  speak  of  lupus  exulcerans. 
In  many  cases  the  process  tends  to  heal  at  the  centre,  with  the  formation 
of  smooth,  stellate  scars,  while  it  extends  at  the  periphery  (lupus  ser- 
piginosus).  Or,  under  the  epithelium  and  in  the  base  of  the  ulcer 
papillary  excrescences  may  form  (lupus  frambcesioides,  papillaris,  verru- 


FIG.  243 


Ulcerating  lupus  of  the  face.     (From  the  Skin  Clinic  of  the  Montreal  General  Hospital.) 

cosus),  or  nodules  (lupus  nodosus,  tuberosus,  tumidus,  hypertrophicus), 
covered  with  crusts  and  epithelial  scales.  In  the  course  of  time,  the 
disease  extends  to  the  deeper  structures,  even  to  the  bone,  and  the  larger 
part  of  the  face,  eyes,  and  lips  may  be  destroyed,  while  from  the  extensive 
scarring  marked  deformity  is  produced. 

Secondary  tuberculosis  is  not  uncommon  in  tuberculous  subjects, 
in  parts  of  the  body  which  are  liable  to  be  contaminated  with  infective 
discharges.  Small  superficial  ulcers,  of  oval  or  rounded  shape,  with 
slightly  infiltrated  edges,  and  surrounded  by  minute  granulomas, 


922  THE  SKIN 

are  found  usually  about  the  orifices  of  the  body,  the  lips,  genitalia, 
anus,  but  also  on  the  head  and  other  parts. 

The  so-called  scrofuloderma  is  found  in  cases  of  widespread  chronic 
tuberculosis  of  various  organs,  and  is  found  particularly  in  children  the 
subjects  of  tuberculous  lymphadenitis.  The  affection  takes  the  form  of 
well-defined,  isolated,  nodular  granulomas,  usually  in  the  subcutaneous 
tissues.  The  disease  may  burrow  deeply,  or  gradually  extend  to  the 
surface.  In  this  way  an  excavated  ulcer  is  produced  with  livid  under- 
mined edges,  having  its  base  covered  with  granulations  and  necrotic 
material,  and  discharging  a  thin  yellowish-white  fluid. 


FIG.  244 


Serpiginous  lupus  of  the  face.      (From  Dr.  Shepherd's  Skin  Clinic,  Montreal  General  Hospital.) 

Miliary  tuberculosis  of  the  skin  has  been  observed  in  cases  of  general- 
ized hematogenic  infection. 

Rhinoscleroma  (Pfundnase)  is  a  rare  disease,  which  appears  to  be 
practically  confined  to  the  continent  of  Europe.  Its  distinguishing 
feature  is  that  it  is  an  inflammatory  granuloma  possessing  little  or  no 
tendency  to  necrosis.  The  generally  accepted  etiological  factor  is  the 
Bacillus  rhinoscleromatis  of  v.  Frisch,  which  is  a  short  capsulated 
bacillus  with  rounded  ends,  not  unlike  the  Friedlander's  bacillus. 

The  disease  affects  the  skin  of  the  nose  and  the  mucous  membrane 
of  the  throat  and  larynx.  Large  tumor-like  growths,  which  are  hard, 
grayish-red  in  color,  and  covered  by  relatively  but  little  altered  epi- 


INFECTIOUS  DERMATITIS  OF  BACTERIAL  ORIGIN  923 

thelium,  are  produced.  These,  microscopically,  consist  of  cellular  con- 
nective-tissue, arranged  in  rounded  masses  or  strands,  enclosing  cells  of 
varying  appearance.  Some  are  large,  swollen,  reticulated,  and  stain 
badly.  Others  are  colloid  or  hyaline  in  appearance  and  contain  bacilli, 
which  are  also  to  be  found  scattered  throughout  the  tissues.  Lymphatic 
channels  are  numerous.  The  disease  remains  strictly  local  and  extends 
extremely  slowly. 

Lepra. — Leprosy  is  a  disease,  which,  with  tuberculosis,  syphilis, 
actinomycosis,  and  rhinoscleroma,  is  classed  among  the  infectious 
granulomas.  The  specific  cause  is  the  Bacillus  leprae  of  Hansen, 
which  is  found  in  great  numbers  in  the  lesions.  The  affection  is  feebly 
contagious,  being,  so  far  as  is  known,  only  transmitted  by  close  personal 
contact,  or  inoculation  with  infectious  discharges. 

Two  main  forms  are  recognized,  identical  in  pathogenesis,  and  differ- 
ing only  in  localization,  namely,  lepra  tuberculosa,  tuberosa,  or  nodosa, 
and  lepra  ancesthetica. 

Tuberculous  leprosy  usually  affects  the  face,  the  extensor  surfaces  of 
the  knees  and  elbows,  and  the  extremities.  The  lesion  consists  in  the 
formation  of  granulomas,  similar  to  those  in  tuberculosis  and  syphilis. 
These  are  composed  of  the  usual  lymphoid  and  epithelioid  cells,  with 
the  addition  of  large  granular  and  vacuolated  cells  known  as  "lepra  cells." 
Giant  cells  are  also  present.  The  bacilli  are  found  both  in  the  lepra 
cells  and  in  the  lymphatics.  A  scraping  from  the  skin  lesions,  stained 
with  carbol-fuchsin,  as  shown  by  the  late  Wyatt  Johnston,  reveals  the 
specific  bacilli  in  abundance  and  affords  a  ready  means  of  diagnosis. 
The  process  begins  in  the  corium  and  gradually  spreads  through  it  to 
the  subcutaneous  tissues  and  to  the  surface.  The  various  glands  and 
follicles  at  first  show  hyperplasia  but  eventually  are  destroyed.  The 
papillae  are  gradually  obliterated,  and  the  epidermis  is  thinned  or  exfoli- 
ating. In  the  more  advanced  cases  ulceration  takes  place.  Objectively, 
the  disease  manifests  itself  at  first  by  reddish  patches  on  the  skin,  which 
may  retrograde,  leaving  merely  a  pigmented  spot,  or  are  gradually 
transformed  into  discolored  nodules  or  tumor-like  masses.  The  disease 
may  for  a  long  time  remain  stationary,  but  in  many  cases  the  infiltration 
becomes  extreme  and  the  various  nodules  coalesce,  so  that  the  tissues 
of  the  face  are  greatly  thickened  and  deformed  (elephantiasis  Grcecorum, 
fades  leontina).  Redness  and  swelling  of  the  skin,  of  erysipelatoid 
type,  are  found  about  the  lesions,  indicating  the  onset  of  fresh  leprous 
infiltration. 

In  the  anesthetic  form  the  nodules  form  upon  the  nerves,  in  the  peri- 
and  endoneurium.  The  lesions  affect  at  first  the  distal  portions  of 
the  smaller  nerves  and  spread  centripetally.  As  a  result,  trophic 
changes  become  manifest  in  the  skin  in  the  form  of  whitish  or  brownish 
streaks  (lepra  maculosa,  morphcea  nigra  et  alba).  Anesthesia  of  the  part 
is  a  prominent  symptom,  resulting  from  the  disintegration  of  the  nerve 
fibers.  As  a  result  of  the  trophic  disturbance,  or  of  traumatism,  ulcers 
readily  form,  which  penetrate  deeply  and  lead  to  the  loss  of  portions 
of  the  body,  such  as  the  fingers  and  toes  (lepra  mutilans). 


924 


THE  SKIN 


Leprosy  has  been  recovered  from,  but,  as  a  rule,  the  disease  remains 
stationary  for  years,  or,  at  most,  is  slowly  progressive.  As  a  consequence, 
those  affected  commonly  die,  not  of  leprosy,  but  of  some  intercurrent 
disease.  Secondary  septic  infection  of  the  ulcers  may  occur. 


FIG.  245 


Case  of  nodular  leprosy  (from  a  patient  of  Dr.  John  V.  Shoemaker's  in  the  Medico-Chirurgical 
College,  Philadelphia).     The  leonine  expression  is  well  shown.     (McFarland.) 

Actinomycosis  of  the  skin  may  be  primary,  but  is  usually  secondary 
to  disease  of  the  deeper  parts.  In  man  the  affection  most  often  begins 
somewhere  in  the  buccal  mucous  membrane,  usually  at  the  alveolar 
process,  and  extends  to  the  cervical  lymphatic  glands  and  the  skin  of 
the  face  and  neck.  The  lesion  is  a  chronic  destructive  granuloma, 
the  skin  being  ulcerated  or  penetrated  by  discharging  sinuses.  Nodular 
foci  with  central  cicatrization  or  diffuse  infiltration  may  also  occur. 
In  the  necrotic  material  and  the  discharge  the  specific  organism — the 
actinomyces — is  usually  to  be  found.  It  may,  however,  be  few  in 
numbers  and  difficult  to  detect.  Primary  actinomycosis  of  the  skin  over 
the  mamma  has  been  noted,  due  to  the  application  of  a  poultice. 

Closely  allied  to  actinomycosis  is  the  Madura  foot  disease  (mycetoma 
pedis,  fungous  foot  of  Indict),  found  in  India  and  other  tropical  countries. 


INFECTIOUS  DERMATITIS  OF  BACTERIAL  ORIGIN  925 

It  is  rare  in  America,1  only  5  cases  having  been  recorded  to  date.  The 
disease  usually  begins  in  the  ball  of  the  great  toe,  and  is  generally  believed 
to  result  from  trauma,  such  as  the  pricking  of  the  toe  with  a  thorn. 
The  injured  part  begins  to  swell,  and  a  firm,  nodular  mass  forms  beneath 
the  skin,  which  assumes  a  purple  color,  and  becomes  indurated  and 
adherent.  The  progress  of  the  disease  is  excessively  slow,  and,  curiously 
enough,  painless.  Secondary  nodules  form  in  other  parts  of  the  foot, 
which  in  the  course  of  a  year  or  so  break  down,  and  finally  discharge 
externally,  producing  numerous  intercommunicating  sinuses.  The 
discharge  is  a  thin  pus  containing  numerous  spherical  grains,  which 
have  been  compared  to  salmon  roe.  Two  varieties  of  these  are  de- 
scribed, the  first  pale  yellow  or  pinkish  in  color,  the  second  black. 
Etiologically,  they  are  different.  The  pale  variety  is  due  to  the  actino- 
myces  Madurse  of  Vincent;  the  black  or  melanoid,  to  a  hyphomyces.2 
The  foot  may  be  converted  eventually  into  an  enormous  distorted  mass, 
full  of  necrotic  material  and  riddled  with  cavities  while  the  skin  on  the 
surface  has  a  somewhat  velvety  appearance. 

Anthrax  of  the  skin  (malignant  pustule)  is  a  local  inflammation  pro- 
duced by  the  anthrax  bacillus.  It  is  an  affection  of  sheep,  horses, 
and  cattle  rather  common  in  certain  countries.  Human  beings  become 
affected  through  contact  with  infective  discharges,  or  from  handling 
the  hides  or  wool  of  diseased  animals.  The  virus  enters  usually  through 
a  scratch  or  abrasion,  or,  occasionally,  from  insect  bites.  The  lesion 
takes  the  form  of  a  pustule  or  carbuncle  at  the  site  of  inoculation.  The 
affected  area  is  elevated  above  the  general  surface  of  the  skin,  and  of 
a  reddish  or  yellowish,  often  hemorrhagic  appearance.  On  the  surface 
of  this  are  often  to  be  seen  vesicles,  bullse,  or  pustules.  Slight  erosion 
may  take  place  with  the  effusion  of  a  small  amount  of  blood  and  serum, 
which  dries  into  crusts.  Consequently,  the  margin  of  the  area  is  higher 
than  the  centre.  The  skin  in  the  neighborhood  is  swollen,  reddened, 
and  oedematous,  and  may  present  small  blebs.  General  infection  and 
death  often  result,  but  where  this  does  not  occur,  a  gangrenous  slough 
is  formed  which  gradually  separates.  Occasionally,  instead  of  a  car- 
buncle being  formed,  there  is  a  diffuse  cedematous  swelling. 

Histologically,  the  papillary  layer  and  the  corium  are  infiltrated  with 
inflammatory  cells  and  exudate,  together  with  hemorrhagic  extra- 
vasation, and  contain  numbers  of  the  specific  bacilli.  The  superficial 
epidermis  is  in  places  elevated  into  vesicles.  The  deeper  layers  are 
also  more  or  less  infiltrated. 

Glanders  is  a  disease,  occasionally  involving  the  skin,  due  to  infection 
with  the  bacillus  mallei,  which  enters  through  some  small  cut  or  abra- 
sion. The  disease  is  usually  derived  from  horses.  At  the  site  of  inocula- 
tion an  area  of  inflammatory  swelling  is  formed,  which  quickly  breaks 
down  into  an  ulcer,  secreting  thin  pus,  and  having  ragged  eroded  edges. 
The  bacilli  are  apt  to  extend  from  the  primary  lesion  along  the  lymphatics, 

1  Adami  and  Kirkpatrick,  Trans.  Assoc.  Amer.  Phys.,  10:  1895:  92. 

2  Wright,  Jour.  Exper.  Med.,  3:  1898:  421. 


926  THE  SKIN 

producing  diffuse  erysipelatoid  inflammation,  or,  again,  secondary 
pustules  and  ulcers.  Along  the  lymphatics,  and  in  the  glands,  inflam- 
matory nodules  may  be  formed  (farcy  buds),  which  break  down,  giving 
rise  to  deep  ulcers.  When  systemic  infection  occurs,  abscesses  may 
form  in  the  internal  organs,  and,  in  fact,  in  any  part  of  the  body.  Pustu- 
lar areas,  resembling  the  pocks  of  variola,  or  pemphigoid  blebs,  may 
be  formed  in  the  skin,  which  break  down  and  discharge  a  viscid  blood- 
stained pus,  often  having  an  offensive  odor. 

Glanders  may  run  an  acute  course  of  from  two  to  four  weeks,  or, 
again,  may  last  for  many  months.  Acute  glanders  is  almost  invariably 
fatal.  The  chronic  form  may  be  recovered  from.  It  may  be  associated 
with  amyloid  degeneration  of  the  viscera.1 

Oriental  furuncle  (tropical  ulcer,  Aleppo  evil,  Delhi  boil,  biskra 
bouton)  is  a  local  inflammation  of  the  skin,  which  is  contagious  and 
believed  to  be  due  to  a  microorganism.  The  affection  begins  as  a 
papule,  which  soon  becomes  a  pustule.  This  breaks  down  into  an 
ulcer.  Healing  takes  place  with  the  formation  of  a  bluish-white  cicatrix. 
Histologically,  the  lesion  resembles  a  tubercle. 

Tropical  phagedena  is  a  rapidly  progressing  gangrenous  ulceration  of 
the  skin  found  in  certain  tropical  regions.  The  disease  is  supposed 
to  be  due  to  some  germ  which  enters  the  skin  through  a  slight  wound. 

Frambesia  (Yaws)  is  a  curious  affection,  found  chiefly  among  negroes 
in  some  tropical  countries.  The  disease  is  infectious  and  has  some 
analogies  with  syphilis.  It  begins  with  a  local  manifestation  at  the  site 
of  inoculation,  which  after  a  variable  time  is  followed  by  lesions  of  the 
skin,  some  constitutional  disturbance,  and  often  general  enlargement 
of  the  glands.  The  disease  may  be  acute  or  chronic.  The  skin  lesions 
are  found  in  the  upper  part  of  the  cutis,  and  resemble  the  other  infectious 
granulomas.  The  later  manifestations  present  the  appearance  of 
fungoid  nodules  covered  with  a  scab,  which  when  removed  leaves  a 
warty  surface  resembling  a  raspberry  or  cauliflower.  The  disease 
has  been  thought  to  be  due  to  a  bacillus,  but  spirochetes  have  been 
discovered  recently  in  the  lesions. 

Erysipelas. — The  chief  diffuse  dermatitis  is  erysipelas,  an  acute 
infectious  and  contagious  disease,  due  to  infection  of  the  skin  by  the 
streptococcus  erysipelatis  of  Fehleisen,  believed  to  be  a  variety  of  the 
streptococcus  pyogenes.  The  affection  begins  suddenly,  with  chill,  fever, 
and  considerable  constitutional  disturbance.  It  is  often  primary,  but 
may  complicate  other  diseases.  Previously  existing  chronic  skin  diseases 
and  previous  attacks  predispose.  The  infection  spreads  along  the 
lymphatics.  It  is  usual  to  classify  the  disease  under  three  heads,  accord- 
ing to  the  severity  of  the  lesions,  namely,  cutaneous  erysipelas,  cellulo- 
cutaneous  erysipelas,  and  cellulitis.  The  last-mentioned  form  should 
not,  however,  be  regarded  as  necessarily  due  to  the  same  etiological 
factor  that  is  at  work  in  the  other  types. 

In  the  first  form  the  skin  is  smooth,  shiny,  and  of  a  vivid  red  color, 

:See  Robins,  Studies  from  the  Royal  Victoria  Hospital,  Montreal,  2:  1906:  No.  1. 


INFECTIOUS  DERMATITIS  OF  BACTERIAL  ORIGIN  927 

the  blush  disappearing  momentarily  on  pressure.  Later,  the  color 
changes  to  a  dusky  bluish-  or  brownish-red.  To  the  feel  the  affected 
part  is  hot,  painful,  and  brawny.  The  spreading  margin  is  sharply 
defined  and  more  elevated  than  the  remainder  of  the  patch.  The 
amount  of  exudation  varies  according  to  the  intensity  of  the  inflamma- 
tion and  its  situation.  In  the  case  of  loose  tissues  the  oedema  may 
be  extreme.  Frequently,  the  effused  fluid  collects  beneath  the  super- 
ficial epidermis,  which  it  raises  into  blisters  or  bullse  (erysipelas  vesicu- 
losum  et  bullosum).  Occasionally,  the  vesicles  contain  seropus  (ery- 
sipelas pustulosum).  The  exudation  may  dry  into  a  scab  upon  the 
surface  (erysipelas  crustosum).  As  the  inflammation  subsides,  the  skin 
becomes  less  swollen  and  indurated,  the  color  gradually  disappears  and 
desquamation  of  the  epidermis  occurs. 

Histologically,  one  finds  in  the  corium,  and  even  in  the  deeper 
structures,  evidences  of  a  cellulofibrinous  exudation.  The  bloodvessels 
and  lymphatics  are  congested.  Inflammatory  leukocytes  are  found  in 
clusters  about  the  vessels.  The  cells  of  the  epidermis  are  swollen, 
cloudy,  vacuolated,  or  may  have  undergone  colliquation  necrosis.  The 
vesicles,  if  present,  contain  fibrin,  and  a  few  inflammatory  cells.  Strep- 
tococci are  found  chiefly  in  the  lymphatics,  but  also  to  some  extent  in 
the  tissues. 

In  cellulocutaneous  erysipelas  the  subcutaneous  tissues  are  involved 
as  well  as  the  skin.  The  parts  are  greatly  swollen,  cedematous,  and 
of  a  dull  red  color.  When  incised  the  tissues  suggest  the  appearance  of 
wet  washleather.  The  skin  may  present  numerous  blebs  or  may  actually 
slough.  Abscesses  form  in  the  subcutaneous  structures  which  often 
point  and  discharge  externally.  Gangrenous  necrosis  of  certain  parts 
may  result,  and  the  process  spreads  laterally  and  to  the  deeper  parts, 
advancing  along  the  lymphatics.  General  pyemia  sometimes  follows. 

Besides  the  forms  of  dermatitis  of  frankly  bacterial  origin,  there  are 
others  due  to  the  action  of  various  kinds  of  moulds.  These  are  generally 
known  as  the  dermatomy coses.  Chief  among  these  are  the  affections 
called  favus,  ringworm,  pityriasis,  erythrasma,  and  blastomycetic 
dermatitis. 

Favus  (tinea  favosa)  is  an  affection  of  the  skin  due  to  the  activity 
of  a  vegetable  parasite,  the  achorion  Schonleinii.  The  hairy  parts 
of  the  body,  notably  the  head  and  beard,  are  those  usually  affected, 
but  other  regions  may  be  attacked,  as,  for  instance,  the  nails.  The 
disease  is  common  in  certain  of  the  lower  animals,  and  is  readily  trans- 
mitted to  man  and  from  man  to  man.  The  fungus  takes  the  form  of 
a  mycelium,  composed  of  threads,  frequently  branching,  which  vary 
considerably  in  length  and  breadth.  Some  are  thin,  delicate,  and 
homogeneous;  others  larger,  moniliform,  or  divided  into  compartments 
by  transverse  divisions,  and  contain  spores.  The  spores  also  vary  con- 
siderably in  appearance,  some  being  round  or  oval,  others  irregular, 
polyhedral,  or  oblong.  They  are  found  chiefly  at  the  ends  of  certain 
of  the  mycelial  fibers,  but  are  also  free  or  in  little  clumps.  The  organism 
is  found  chiefly  in  the  shaft  and  bulb  of  the  hairs  and  in  the  hair-follicles. 


928 


THE  SKIN 


It  insinuates  itself  between  the  horny  cells  of  the  epidermis,  forming  a 
small,  yellowish  area  just  beneath  the  surface,  penetrated  by  a  hair. 
As  it  grows,  a  sulphur-yellow,  concave  or  cup-like  disk  (scutuld),  the 
size  of  a  lentil  or  larger,  is  produced.  This  disk  is  convex  below  and 
lies  in  a  corresponding  excavation  in  the  skin.  When  the  growth  is 
removed  the  underlying  skin  is  moist  and  red,  owing  to  reactive  inflam- 
mation. In  long-standing  cases,  atrophy  of  the  hair-papillae  and  various 
glands,  and  of  the  rete  and  upper  part  of  the  corium  results.  Loss  of 
hair  and  scarring  may  be  permanent.  When  exposed  to  the  air,  the 
favus  clump  becomes  dried  into  a  dirty,  yellowish- white,  crumbling 
mass,  which  is  readily  broken  up.  The  hairs  of  the  affected  part  are 
dry,  lustreless,  and  easily  pulled  out.  The  fungus  by  suitable  methods 
can  be  demonstrated  in  the  hair-sheaths. 


FIG.  246 


Portion  of  a  hair  invaded  by  the  trichophyton,  endoectothrix.  X  500.  a,  a,  chains  of  spores 
in  focus.  6,  a  chain  situated  farther  within  the  hair,  and  hence  not  in  focus.  (From  a  photo- 
micrograph.) (Hyde.) 

In  favus  of  the  nails — onychomycosis  favosa — the  fungus  grows  between 
the  keratinous  layers  of  the  nail-plate,  which  is  thickened,  brittle,  and 
infiltrated  with  yellowish  masses.  Complete  disintegration  of  the  nail 
may  result. 

Ringworm  is  a  dermatomycosis  of  which  several  forms  are  recognized 
according  to  the  nature  of  the  parasite  at  work.  At  least  two  distinct 
varieties  of  fungus  have  been  demonstrated,  one  with  small  spores — 
microsporon  Audouini;  the  other  with  large  spores — the  trichophyton. 
In  the  first  case  the  mycelium  is  developed  within  the  hair  itself,  and 
the  filaments  after  division  terminate  on  the  outer  aspect  of  the  hair 


INFECTIOUS  DERMATITIS  OF  BACTERIAL  ORIGIN  929 

shaft.  The  spores  are  entirely  external  to  the  hair.  In  the  case  of  the 
trichophyton  the  spores  are  found  in  rows,  parallel  to  the  long  axis  of 
the  hair.  The  mycelium  is  not  produced  within  the  hair.  Three 
subvarieties  of  the  trichophyton  are  now  recognized,  according  to  the 
position  of  the  spores  within  or  without  the  hair,  viz.,  the  endothrix, 
the  ectothrix,  and  the  endo-ectothrix. 

Several  forms  of  ringworm  (tinea)  are  recognized  clinically,  according 
to  the  localization  of  the  lesions. 

Tinea  tonsurans  (ringworm  of  the  scalp)  is  due  to  the  presence  of  the 
microsporon,  less  often  of  the  endothrix,  within  the  hairs  and  hair- 
follicles.  The  affection  results  in  the  formation  of  an  area  of  slight 
inflammation  on  the  scalp,  in  which  the  hairs  are  brittle  and  readily 
break  off,  leaving  brush-like  stumps  projecting  just  above  the  surface 
of  the  skin.  This  is  due  to  the  distortion  of  the  bulb  and  shaft  of  the 
hair,  together  with  its  separation  into  fibrils.  The  disease  is  commoner 
in  children  than  in  adults,  is,  of  course,  contagious,  and  is  liable  to 
appear  in  epidemics. 

Tinea  circinata  is  found  on  the  hairless  portions  of  the  body  and  is 
due  to  the  presence  of  the  trichophyton  in  the  deeper  parts  of  the  horny 
layer  and  in  the  upper  parts  of  the  rete.  The  fungus  extends  centrifu- 
gally,  forming  the  characteristic  circinate  lesions  of  ringworm.  A 
reactive  inflammation,  marked  by  hyperemia,  is  also  present,  and  varies 
in  severity  according  to  the  amount  of  moisture,  warmth,  and  irritation 
to  which  the  part  involved  is  subjected.  In  parts  of  the  body  where 
two  surfaces  rub  upon  one  another,  and  the  epithelium  is  macerated 
from  retained  and  decomposing  sweat,  a  severe  inflammation  with  the 
formation  of  vesicles  and  scabs  (eczema  marginatum)  results. 

Tinea  sycosis  (barber's  itch)  is  a  ringworm  of  the  hairy  parts  of  the 
face,  caused  by  the  trichophyton,  usually  the  ectothrix  variety,  or, 
possibly,  the  endoectothrix.  The  lesion  is  a  folliculitis  and  perifol- 
liculitis.  The  hairs  become  loosened  and  fall  out.  The  inflammation 
is  more  intense  than  in  other  forms  of  ringworm.  The  hair-follicles 
and  sebaceous  glands  are  destroyed,  and  small  abscesses  may  form  here 
and  there  in  the  deeper  parts.  This  is  possibly  the  result  of  secondary 
infection  with  pyogenic  microorganisms. 

Tinea  imbricata  (Tokelau  ringworm)  is  found  in  tropical  countries 
and  is  due  to  a  form  of  trichophyton.  The  fungus  is  found  only  in  the 
epidermis  and  the  follicles  are  unaffected. 

Tinea  versicolor  (pityriasis  versicolor,  dermatomycosis  furfuracea) 
is  a  mycotic  disease  of  the  skin,  affecting  chiefly  the  covered  parts  of 
the  body.  The  organism  at  work  is  the  microsporon  furfur  which  is 
found  in  the  upper  portion  of  the  stratum  corneum.  It  produces 
desquamation,  but  the  reactive  inflammation  is  practically  nil.  The 
hair-follicles  are  not  involved.  The  affected  part  presents  an  irregular, 
yellowish-brown,  blotchy  appearance,  with  slight  desquamation  of  the 
epidermis. 

Erythrasma  is  a  somewhat  similar  affection,  believed  by  some  to  be 
due  to  the  microsporon  minutissimum,  although  others  think  that  it 
59 


930 


THE  SKIN 


is  produced  by  bacteria.  The  disease  is  found  in  parts  of  the  body 
which  are  warm  and  moist,  as,  for  instance,  in  the  groins  and  axillae, 
where  two  skin  surfaces  come  together.  The  patches  are  fairly  well 
defined,  and  present  slight  redness  and  desquamation. 

Blastomycetic  dermatitis  is  a  curious  and  rare  affection  of  the  skin, 
first  described  by  Gilchrist,1  due  to  a  fungus  related  to  the  yeasts.  When 
fully  developed,  the  lesion  bears  a  somewhat  close  resemblance  to  ver- 
rucose  tuberculosis,  and  occasionally  to  superficial  papillary  epithelioma. 
With  care,  however,  differentiation  is  possible.  The  affection  begins 
with  the  formation  of  a  papule  or  pustule,  which  gradually  spreads 
until,  in  the  course  of  months  or  years,  considerable  areas  of  skin  are 


FIG.  247 


Filaments  and  spores  of  the  trichophyton  from  the  beard  of  a  patient  affected  with 
tinea  sycosis.     (Hyde.) 

involved.  A  warty,  tumor-like  outgrowth,  presenting  occasional 
superficial  ulcerations  covered  with  crusts,  is  the  result.  The  lesions 
may  be  extensive  and  are  apt  to  be  multiple. 

Histologically,  the  appearances  are  those  of  an  inflammatory  granu- 
loma.  The  most  marked  changes  are  found  in  the  rete,  the  epithelium 
of  which  shows  hyperplasia  in  the  form  of  extensive,  elongated  and 
branching,  downgrowths  of  its  cells,  producing  finger-like  processes 
practically  identical  with  those  seen  in  epithelioma.  Polymorpho- 
nuclear  leukocytes  are  found  scattered  about,  both  between  and  within 
the  cells,  or  in  small  clumps  which  are  really  minute  abscesses.  These, 
according  to  Montgomery,  are  characteristic  of  the  process.  When 
disintegration  is  complete,  the  abscesses  contain  degenerating  leukocytes, 
red-blood  cells,  epithelial  cells,  particles  of  chromatin  and  other  cell- 

1  Johns  Hopkins  Hosp.  Rep.,  1  :  1896  :  269. 


PLATE    XII 


Clinical  Types   of   Cutaneous    Blastomycosis 


DERMATITIS  DUE  TO  ANIMAL  PARASITES  931 

detritus,  the  specific  microorganism,  and,  usually,  giant  cells.  The 
rete-cells  are  large  and  swollen,  somewhat  dissociated,  and  the  prickles 
are  particularly  well  defined.  Those  in  the  immediate  neighborhood 
of  the  abscesses  are  more  or  less  flattened.  Single  giant  cells  are  occasion- 
ally to  be  observed,  surrounded  by  a  few  leukocytes.  Isolated  cells, 
or  groups  of  cells,  show  pathological  keratinization.  A  layer  of  columnar 
cells,  occasionally  showing  mitoses,  can  usually  be  traced,  more  or 
less  perfectly  between  the  epithelium  and  the  corium. 

In  the  corium  inflammatory  infiltration  may  be  very  marked.  Leuko- 
cytes, endothelial  cells,  and  plasma  cells  are  to  be  observed.  Miliary 
abscesses  may  occur  here  also.  Mast-cells  and  giant  cells  may  be 
found  in  varying  numbers. 

The  horny  layer  of  the  skin  varies  much  in  thickness.  In  some 
places  it  is  eroded,  in  others  thickened,  penetrating  downward  between 
the  papillae,  which  are  thereby  dislocated  and  distorted.  On  the  sur- 
face can  be  seen  scab-like  masses  of  dried  secretion,  composed  of  pus, 
blood,  desquamated  epithelium,  and  bacteria. 

The  specific  organism  can  readily  be  demonstrated  by  placing  portions 
of  tissue  or  pus  in  a  strong  solution  of  caustic  potash.  In  stained 
sections  the  parasite  is  a  round,  oval,  or  slightly  irregular  body,  possess- 
ing a  highly  refractile,  doubly  contoured,  homogeneous  capsule.  The 
protoplasm  is  somewhat  granular,  and  separated  from  the  capsule 
by  a  space  of  varying  width.  In  the  cell-body,  a  clear  vacuole  can 
often  be  made  out.  The  parasite  lies  singly  or  in  pairs,  and  budding 
forms  in  all  stages  can  usually  be  seen.  It  is  found  in  the  abscesses, 
between  the  epithelial  cells,  and  in  the  giant  cells. 

The  organism  has  been  successfully  cultivated,  although  it  grows 
with  some  difficulty,  and  when  inoculated  into  the  lower  animals  has 
produced  abscesses  or  inflammatory  granulomas.  It  seems  fairly  well 
established  now  that  the  parasite  is  specific,  although  it  is  possible  that 
there  may  be  several  subvarieties.1 

In  all  of  these  mycotic  diseases  the  specific  organisms  can  usually 
be  demonstrated  by  placing  the  affected  hairs  or  scales  in  a  5  to  10 
per  cent,  solution  of  caustic  potash  for  a  variable  period  and  examining 
with  the  microscope. 

Dermatitis  Due  to  Animal  Parasites. — The  most  important  disease 
to  be  considered  here  is  scabies,  an  inflammatory  affection  due  to  the 
action  of  the  itch-mite  or  acarus  scabiei.  The  impregnated  female 
mite  penetrates  the  epidermis  and  burrows  its  way  into  the  rete,  or 
even  as  far  as  the  upper  layers  of  the  corium,  forming  a  curved,  some- 
what sacculated  track.  In  the  early  stages  the  parasite  can  usually 
be  recognized  at  the  blind  end  of  the  burrow  as  a  whitish  speck  the  size 
of  a  pin-point.  In  the  burrow  are  also  to  be  found  brownish  or  blackish 
granules,  the  feces,  and  sometimes  more  or  less  numerous  immature 

1  For  a  very  full  consideration  of  this  form  of  dermatitis,  see  Ricketts,  Oidiomycosis, 
(Blastomycosis)  of  the  Skin  and  its  Fungi,  Jour,  of  Med.  Research  (new  series) 
1  :  1901  :  373. 


932  THE  SKIN 

mites.  Owing  to  the  irritation  caused  by  the  presence  of  the  parasites, 
and  also  from  the  scratching  induced  thereby,  considerable  inflammation 
is  set  up,  so  that  an  eczematous  eruption  is  usually  the  result,  character- 
ized by  the  formation  of  pustules  and  vesicles.  In  long-standing  cases 
the  skin  may  undergo  a  marked  change.  The  stratum  corneum  is 
hypertrophied,  the  cutis  is  thickened  and  infiltrated  with  inflammatory 
products,  and  the  papillae  are  increased  in  length. 

Pediculosis  is  an  inflammatory  affection  of  the  skin  produced  by  the 
agency  of  lice  upon  the  skin  or  in  the  hair.  The  inflammation  is  usually 
a  mild  one,  and  is  produced,  in  part,  by  the  irritation  caused  by  the 
insect,  and,  in  part,  by  scratching.  Three  different  forms  are  described. 
In  pediculosis  capitis  the  parasite  is  found  upon  the  scalp  or  on  the 
hairs,  while  the  ova  can  be  detected  attached  to  the  hairs  by  a  chitin- 
ous  sheath.  In  long-standing  cases  marked  inflammation  results. 
Pustules  or  even  small  abscesses  may  form  in  the  scalp.  There  is 
considerable  exudation  and  the  hair  may  be  considerably  matted 
together  (plica  polonica).  This  is  seen  most  often  just  above  the  nape 
of  the  neck.  The'  irritation  in  the  case  of  pediculosis  corporis  is  less 
marked.  The  lice  infect  the  clothes  and  wander  on  the  skin  to  feed. 
Owing  to  the  long-continued  mild  inflammation,  the  skin  becomes 
somewhat  thickened  and  pigmented  and  scored  with  scratches  (vaga- 
bond's disease).  Pediculosis  pubis  is  usually  found  in  the  pubic  region, 
but  may  affect  the  other  hairy  parts  of  the  body.  The  ova  are  similar 
to  those  of  the  head-louse,  but  are  smaller.  The  secondary  inflammatory 
disturbance  is  trifling  in  this  variety. 

A  somewhat  similar  disturbance  is  caused  by  certain  other  parasites, 
among  which  may  be  mentioned  the  pulex  irritans,  or  common  flea, 
cimex  lectularius,  or  bedbug,  the  pulex  penetrans,  or  sand-flea.  The 
larvae  of  certain  diptera  are  sometimes  found  beneath  the  skin.  The 
most  common  offender  is  the  gad-fly  (sestrus  bovis). 

Cysticerci  and  echinococci  have  been  found  in  the  skin. 

Filaria  medinensis  (Guinea-worm)  is  a  parasite,  found  in  warm  coun- 
tries, which  gains  access  to  the  body  through  the  ingestion  of  water 
containing  the  larvae.  The  female  worm,  which  is  the  one  causing 
the  disease,  is  from  fifteen  to  forty  inches  long,  and  one-tenth  of  an 
inch  in  diameter.  For  ten  to  fifteen  months  the  worm  remains  lodged 
in  the  muscles  and  then  gradually  works  its  way  to  the  surface  of  the 
body,  where  it  can  be  felt  as  a  soft  coil  under  the  skin.  The  skin 
over  the  worm  ultimately  gives  way  and  a  small  ulcer  is  produced. 
Abscess-formation  may  result  in  some  cases  in  lymphangitis,  septicemia, 
and  gangrene. 

Craw-craw  is  a  rare  disease,  in  the  form  of  an  acute  superficial  der- 
matitis, which  affects  principally  the  negroes  of  the  west  coast  of  Africa. 
It  is  supposed  to  be  due  to  a  worm,  allied  to  the  filaria  medinensis. 

Dermatitis  of  Neuropathic  Origin. — It  is  a  well-known  fact  that  in  certain 
disorders  of  the  central  nervous  system  and  of  the  peripheral  nerve- 
trunks  the  skin  may  undergo  striking  changes.  These  may  be  simply 
minor  alterations  in  the  appearance  and  texture  of  the  skin,  or  may 


DERMATITIS  OF  NEUROPATHIC  ORIGIN  933 

amount  to  actual  inflammation  or  other  profound  disturbance.  Thus, 
in  cases  of  rheumatoid  arthritis,  the  skin  over  the  affected  joints  is 
often  found  to  be  soft,  velvety,  and  of  smooth,  glistening  appearance. 
In  certain  organic  diseases  of  the  spinal  cord,  gangrene  of  the  skin 
and  subcutaneous  tissues  (bedsores)  often  come  on  with  great  rapidity. 
So  also  in  tabes  dorsalis,  penetrating  ulcers  on  the  extremities,  usually 
over  the  great  toe,  are  occasionally  met  with.  Besides  these,  there  is 
a  considerable  number  of  disorders  of  the  skin,  which,  with  more  or 
less  certainty,  may  be  attributed,  either  wholly  or  in  part,  to  neuro- 
trophic  influences. 

FIG.  248 


Urticaria  factitia  (angioneurosis).      (Hyde  and  Ormsby.) 

Among  the  conditions  which  may,  in  some  instances  at  least,  be 
attributed  to  nervous  disturbances  should  be  mentioned  urticaria.  This 
is  an  angioneurosis  in  which  an  idiosyncrasy  of  the  individual  plays  an 
important  predisposing  role.  The  disease  is  found  in  those  of  a  neurotic 
temperament,  and  has  been  known  to  follow  upon  mental  worry.  In 
other  cases,  disorders  of  digestion,  gastro-intestinal  intoxication,  are 
at  work.  Thus,  some  persons  are  intolerant  of  certain  foods,  such  as 
cheese,  shellfish,  fruits,  or,  again,  certain  drugs.  In  those  predisposed 
external  irritation,  even  of  the  slightest  description  may  precipitate 
an  attack.  For  example,  in  urticaria  factitia  (dermatographia)  the 
simple  drawing  of  the  finger-nail  over  the  skin  is  followed  in  a  few 
moments  by  the  production  of  a  wheal,  exactly  conforming  to  the  region 
of  irritation. 

The  exact  pathogeny  of  the  condition  is  not  altogether  agreed  upon. 
Most  seem  to  think  that  there  is  a  preliminary  brief  contraction  of  the 


934  THE  SKIN 

vessels.  This  is  followed  by  paretic  dilatation  with  exudation  of  plasma, 
and  local  stasis.  At  first  there  is  produced  an  elevated,  somewhat 
firm  nodule,  of  dull  red  color,  which  soon  becomes  paler  in  the  centre 
(the  wheal).  This  last  change  is  attributed  to  the  compression  of  the 
capillaries  of  the  part  by  the  increasing  exudation.  The  effusion  of 
plasma  may  be  so  great  as  to  elevate  the  superficial  epidermis,  thereby 
forming  blisters  (urticaria  bullosa).  Or,  again,  the  oedema  may  be 
followed  by  extravasation  of  blood  (urticaria  hemorrhagica,  purpura 
urticans).  In  still  other  cases  there  is  an  accumulation  of  pigment 
in  the  deeper  layers  of  the  rete,  with  persistence  of  papules  and  nodules 
after  the  wheals  have  disappeared  (urticaria  pigmentosa).  In  the  obsti- 
nate urticaria  of  childhood  small,  inflammatory  papules  may  be  formed, 
apparently  follicular  in  origin  (urticaria  papulosa,  lichen  urticatus 
strophulus). 

A  closely  allied,  if  not  identical,  condition,  is  angioneurotic  oedema  (see 
p.  113).  This  probably  does  not  depend  upon  external  irritation  and  is 
believed  to  originate  in  the  subcutaneous  tissues. 

Prurigo. — The  term  "prurigo"  is  rather  loosely  employed  by  dermat- 
ologists to  designate  any  pruritic  dermatitis  associated  with  the  forma- 
tion of  more  or  less  persistent  papules.  The  prurigo  of  Hebra  is  an 
affection  of  early  childhood,  which  persists  mostly  throughout  life.  It 
begins  with  the  formation  of  urticarial  nodes  on  the  extensor  surfaces 
of  the  extremities,  associated  with  the  most  intense  itching.  In  course 
of  time,  owing  to  the  irritation  produced  by  scratching,  small  inflam- 
matory nodules  are  formed,  over  which  the  skin  is  excoriated  and  often 
covered  with  scabs.  It  may  be  complicated  by  eczema  and  erysipelas. 
Auspitz,v.  Hebra,  Schwimmer,  among  others,  class  it  among  the  neuroses. 

Herpes  is  an  affection  of  the  skin  characterized  by  the  formation 
of  papules,  which  are  soon  converted  into  vesicles,  and  after  some 
days  into  pustules.  The  efflorescence  corresponds  fairly  accurately 
with  certain  nerve  tracts.  Various  parts  of  the  body  may  be  involved, 
the  face  (herpes  facialis),  the  forehead  (herpes  frontalis),  the  conjunc- 
tion (herpes  ophthalmicus) ,  the  lip  (herpes  labialis),  the  genitalia  (herpes 
progenitalis  and  preputialis),  and  the  trunk  (herpes  zoster).  The  exact 
cause  is  not  known,  but  is  believed  to  be  some  local  or  reflex  nervous 
disorder.  Herpes  of  the  face  and  lips  is  found  in  certain  infectious 
diseases,  notably  pneumonia,  and  gastro-intestinal  derangements.  Herpes 
progenitalis  may  be  reflex  or  the  result  of  some  local  irritation. 

In  herpes  zoster  the  lesions  are  found  along  the  course  of  one  or  more 
intercostal  nerves.  It  is  usually  unilateral.  The  affection  is  believed 
by  some  to  be  bacterial  in  origin.  Others  consider  it  an  angioneurosis. 
The  pathological  condition  most  frequently  found  is  inflammation  of 
the  posterior  ganglion  and  of  the  nerve-trunk  supplying  the  affected 
portion  of  the  skin.  Changes  of  various  kinds  have  also  been  reported 
in  the  central  nervous  system. 

Histologically,  the  rete  cells  undergo  rapid  proliferation  and  certain 
of  them  degenerate,  forming  small  cavities.  The  papillary  bodies 
in  turn  become  inflamed  and  the  cavities  coalesce  to  form  vesicles. 


PLATE  XIII 


Herpes  Zoster. 

(From  the  Skin  Clinic  of  the  Montreal  General  Hospital.) 


DERMATITIS  OF  UNKNOWN  AND  DOUBTFUL  ETIOLOGY     935 

Gilchrist1  found  in  the  papular  stage,  before  the  formation  of  the  vesicle, 
a  notable  multiplication  of  the  nuclei  in  the  rete  without  any  increase 
in  the  mitotic  figures. 

Herpes  iris  is,  according  to  Kaposi,  identical  with  erythema  iris. 
The  vesicles  are  found  on  the  hands  and  feet. 

Dermatitis  herpetiformis  (dermatitis  multiformis,  herpes  gestationis, 
hydroa  herpetiforme,  Duhring's  disease,  pemphigus  pruriginosus)  is 
an  obscure  affection,  regarded  by  Duhring  as  intermediate  between 
pemphigus  and  erythema  multiforme.  It  is  most  likely  neuropathic 
in  nature.  It  has  been  attributed  to  many  causes,  neurasthenia,  general 
debility,  renal  and  other  organic  disease,  and  pregnancy.  The  process 
is  an  acute  inflammation  of  the  papillary  layer.  There  is  a  marked 
exudation  of  plasma,  leukocytes,  and  red  blood-cells.  The  exudate 
is  fibrinous  in  nature.  Large  numbers  of  eosinophiles  are  found  in 
the  blood  and  in  the  local  lesions. 

Dermatitis  of  Unknown  and  Doubtful  Etiology. — Dermatitis  exfoliativa 
is  the  term  rather  loosely  employed  to  designate  any  inflammation  of 
the  skin  associated  with  desquamation  of  the  epidermis.  The  con- 
dition sometimes  complicates  eczema,  psorLsis,  lichen  ruber,  and 
other  varieties  of  dermatitis.  Occasionally,  it  arises  idiopathically. 

In  the  early  stages  there  is,  according  to  Crocker,  a  superficial  inflam- 
mation of  the  corium,  characterized  by  congestion,  oedema,  and  cellular 
infiltration.  The  rete  is  thinned  and  there  is  separation  of  the  upper 
portion  of  the  stratum  corneum.  Later,  there  is  proliferation  of  the 
rete  cells  with  some  connective-tissue  hyperplasia.  In  the  most  ad- 
vanced forms  the  horny  layer  is  thickened  and  there  is  more  or  less 
atrophy  of  the  rete  and  corium  and  of  the  hair-follicles  and  sebaceous 
glands.  The  pityriasis  rubra  of  Hebra  is  by  many  considered  to  be 
a  severe  form  of  dermatitis  exfoliativa.  It  is  a  rare  affection,  which 
may  continue  for  many  years,  and  is  usually  fatal  from  marasmus. 
Histologically,  it  seems  to  correspond  fairly  accurately  with  the  more 
advanced  form  of  exfoliative  dermatitis.  Exfoliative  dermatitis  has 
been  found  in  young  infants  (Ritter)  and  may  be  epidemic  (Savill). 

Pityriasis  rosea  (herpes  tonsurans  maculosus,  pityriasis  maculata  et 
circinata)  is  a  somewhat  similar  disease,  a  simple  superficial  inflam- 
mation running  an  acute  course,  and  associated  with  branny  desqua- 
mation of  the  epidermis.  Kaposi  believed  it  to  be  due  to  the  local 
action  of  a  parasite,  but  it  is  more  probably  due  to  a  systemic  infection. 
According  to  Unna,  there  is  a  superficial  inflammation  with  oedema 
and  cellular  infiltration  of  the  cutis  and  lower  portions  of  the  rete, 
oedema  of  the  upper  rete  cells,  acanthosis  and  parakeratosis,  together 
with  the  formation  of  minute  "subcorneal  pressure  vesicles." 

Eczema  is  perhaps  the  most  common  form  of  inflammation  of  the  skin. 
The  disease  may  be  acute  but  is  often  chronic,  and  has  a  notable  tendency 
to  relapse.  The  lesions  are  strikingly  polymorphous.  The  skin  is 
more  or  less  reddened  and  swollen,  and  may  be  covered  with  papules, 

1  Johns  Hopkins  Hosp.  Rep.,  1. 


936  THE  SKIN 

vesicles,  pustules,  and  crusts.  The  affected  part  is  very  itchy,  may 
be  exfoliating,  and  often  is  moist  from  exudation.  In  the  chronic  cases 
the  skin  is  thickened  and  often  fissured. 

Although  the  condition  has  been  frequently  and  carefully  studied, 
the  etiology  is  still  to  a  great  extent,  obscure.  Many  cases  are  to  be 
attributed  to  external  irritation,  such  as  may  be  caused  by  parasites, 
scratching,  moisture,  or  acrid  discharges.  Some  individuals  are  more 
susceptible  to  the  action  of  these  causes  than  are  others.  Other  cases 
are  the  result  of  some  systemic  disorder  or  of  disturbed  metabolism, 
such  as  gout,  rheumatism,  toxemias,  gastro-intestinal  derangements, 
and  malnutrition.  In  still  other  instances  there  seems  to  be  a  neurotic 
agency  at  work. 

In  the  mildest  form,  where  the  irritation  is  not  extreme  or  the  skin 
is  not  particularly  sensitive,  small  nodular  elevations  are  found  in  the 
skin  (eczema  papulosum).  In  somewhat  more  severe  cases  vesicles 
may  be  produced  (eczema  vesiculosum).  When  the  contents  of  the 
blisters  are  absorbed  or  dry  up  the  superficial  epidermis  may  be  exfoliated. 
When  the  inflammation  is  more  intense,  considerable  areas  of  skin  may 
become  painful,  red,  and  swollen  (eczema  erythematosum).  On  this 
inflamed  base  may  be  formed  blisters,  which  subsequently  may  become 
purulent  (eczema  pustulosum).  Should  the  epidermis  be  lost,  as  from 
rubbing  or  scratching,  we  get  a  weeping  surface  (eczema  madidans). 
Where  considerable  patches  of  epidermis  are  lost,  the  underlying  surface 
presents  a  dusky  red  appearance  (eczema  rubrum).  Frequently,  the 
serous  or  purulent  discharge  dries  upon  the  surface  into  yellowish 
crusts  (eczema  crustosum).  Under  the  crusts  pus  may  accumulate 
(eczema  impetiginosum).  In  other  cases  the  epithelium  may  proliferate 
beneath  the  scabs,  so  that  the  surface  appears  to  be  reddened,  thick- 
ened, and  scurfy  (eczema  squamosum).  Eczema  characterized  by 
somewhat  larger  pustules  covered  with  crusts  is  often  called  impetigo. 
When  the  pustules  and  crusts  are  still  larger  the  condition  is  known  as 
ecthyma. 

Histologically,  in  acute  eczema,  one  finds  congestion  of  the  blood 
and  lymphatic  vessels  in  the  corium,  with  exudation  of  plasma,  and 
diapedesis  of  leukocytes.  The  connective-tissue  fibers  are  swollen 
and  compressed,  and  there  is  to  some  extent  proliferation  of  the  con- 
nective-tissue corpuscles.  In  the  milder  forms,  the  process  is  chiefly 
confined  to  the  papillary  regions  and  upper  layers  of  the  corium,  but 
in  severer  cases  extends  to  the  subcutaneous  tissues.  The  cells  of  the 
rete  are  swollen,  and  there  is  effusion  of  fluid,  with  more  or  less  numer- 
ous wandering  cells.  The  rete-cells  may  also  undergo  a  colliquative 
necrosis  with  the  formation  of  cavities.  When  the  exudation  is  marked, 
vesicles  or  bullse  may  form  between  the  rete  and  the  horny  layer,  which 
contain  serum,  fibrin,  leukocytes,  and  detritus.  In  the  pustular  variety 
the  number  of  leukocytes  is  very  considerable.  Desquamation  of 
the  superficial  epidermis  takes  place  in  many  instances  and  is  a  char- 
acteristic change  in  eczema  squamosum  and  rubrum. 

In  chronic  eczema,  proliferation  of  connective  tissue  in  the  corium, 


DERMATITIS  OF  UNKNOWN  AND  DOUBTFUL  ETIOLOGY     937 

with  deposit  of  pigment,  together  with  hyperplasia  of  the  rete,  dominate 
the  picture.  The  process  may  extend  to  the  subcutaneous  tissues, 
producing  atrophy  of  the  fat,  hair-follicles,  and  glands.  Occasionally, 
owing  to  the  obstruction  of  veins  and  lymphatics,  a  form  of  elephantiasis 
results.  The  acute  form,  however,  may  heal  without  producing  much 
permanent  change,  save,  possibly,  a  little  pigmentation. 

Erythema  exudativum  multiforme  is  an  affection  of  unknown  etiology, 
the  characteristic  features  of  which  are  congestion,  cedema,  and  some- 
times hemorrhage  in  the  skin.  It  is  found  usually  in  young  persons 
and  those  of  a  rheumatic  tendency.  In  some  cases  the  disease  is  symp- 
tomatic, being  found  in  such  affections  as  rheumatism,  typhus,  syphilis, 
and  gonorrhoea.  Here,  it  is  possibly  of  the  nature  of  an  infective 
metastatic  dermatitis.  In  other  cases  it  is  thought  to  be  an  angioneu- 
rosis  caused  by  some  reflex  disturbance  or  a  circulating  toxin. 

In  the  mildest  form  of  the  affection  there  is  simple  congestion  of  the 
skin.  In  others,  there  is  an  inflammatory  exudation  into  the  papillary 
and  middle  layers  of  the  corium,  and  sometimes  even  into  the  deeper 
portions  of  the  corium  and  the  subcutaneous  tissues.  The  cells  of  the 
rete  may  show  proliferation.  Where  the  serous  exudation  is  marked 
it  may  lead  to  the  accumulation  of  fluid  in  the  rete,  or  the  elevation 
of  the  epidermis  into  blisters  (herpes  iris,  herpes  circinatus,  erythema 
bullosum,  hydroa  vesiculosum).  There  is  always  more  or  less  extra- 
vasation of  the  red  blood-cells. 

In  erythema  nodosum,  rather  deeply  seated  painful  nodes  are  formed 
in  the  cutis  and  subcutaneous  tissues.  These  often  project  slightly 
above  the  surface  as  firm,  dusky  red  infiltrations.  As  the  process 
subsides,  the  color  changes  to  bluish-red,  green,  and  yellow.  In  excep- 
tional cases  the  inflammation  is  so  intense  as  to  result  in  gangrene. 
The  disease  is  usually  associated  with  inflammatory  rheumatism,  but 
may  occur  without  arthritic  manifestations.  It  is  probably  a  sporadic 
myositis  of  infective  origin. 

Pemphigus  is  a  somewhat  obscure  affection  of  the  skin,  the  chief 
feature  of  which  is  the  efflorescence  of  numerous  vesicles,  varying  in 
size  from  that  of  a  pea  to  that  of  a  goose  egg.  The  vesicles  appear 
occasionally  on  apparently  healthy  skin,  but,  as  a  rule,  develop  in 
patches  of  erythema  or  urticaria-like  nodes.  They  contain  a  clear 
watery  fluid,  or  sometimes  blood,  which  eventually  becomes  cloudy  and 
purulent.  The  vesicles  then  dry  up  with  the  formation  of  crusts 
(pemphigus  vulgaris).  In  some  cases  the  superficial  epidermis  is  cast 
off  so  that  the  corium  is  exposed  over  considerable  areas  (pemphigus 
foliaceus).  A  red  weeping  surface  is  thus  produced.  In  such  cases 
the  corium  is  more  or  less  infiltrated  and  there  may  be  a  certain  amount 
of  necrosis  (pemphigus  diphtheriticus).  Fresh  granulations  may  be 
formed,  which  in  their  turn  are  destroyed.  In  the  so-called  pemphigus 
vegetans,  which  is  the  most  severe  form  of  the  affection  and  progresses 
rapidly  toward  death,  the  inflammatory  phenomena  are  more  marked. 
The  vesicles  appear  at  first  on  the  skin  of  the  genitals,  on  the  inner  side 
of  the  thighs,  the  axillae,  and  the  mucous  membrane  of  the  mouth, 


938  THE  SKIN 

eventually  spreading  to  the  other  mucous  surfaces  and  the  skin  of  the 
whole  body.  When  the  superficial  epidermis  is  exfoliated,  numerous 
polypoid  or  warty  excrescences,  closely  set  together,  are  produced, 
which  become  bounded  by  an  excoriated  zone,  and  later,  are  surrounded 
by  other  blisters.  A  foul-smelling  discharge  is  the  result,  which  dries 
into  crusts. 

The  histological  appearances  in  pemphigus  have  been  variously 
interpreted.  Some  think  that  the  process  is  a  primary  dermatitis. 
Auspitz,  among  others,  believes  that  there  is  first  a  sudden  liberation 
of  fluid  from  the  vessels  of  the  derma,  which  mechanically  separates  the 
cells  of  the  rete.  Spaces  are  thus  formed  which  coalesce  to  form  the 
vesicles.  The  vesicles  or  bullae  may  originate  between  the  rete  and 
the  superficial  horny  layers,  but,  according  to  Crocker,  may  also  be 
found  entirely  within  the  rete.  Later  the  signs  of  a  secondary  inflam- 
mation set  in. 

Numerous  etiological  factors  have  been  held  to  be  the  cause  of 
pemphigus.  Many  cases  are  attributable,  directly  or  indirectly,  to 
disorders  of  the  central  nervous  system  and  peripheral  nerves,  so  that 
such  cases  may  be  regarded  as  trophoneuroses.  Chills  (Crocker), 
hysteria  (Dumesnil),  traumatism,  and  local  wound-infection  (Fernet 
and  Bullock)  have  also  been  assigned  as  causes.  A  curious  point  is 
the  marked  increase  of  the  eosinophile  cells  in  the  blood  and  in  the 
fluid  of  the  vesicles.  There  is  some  evidence  in  favor  of  a  microbic 
origin  for  the  disease. 

Closely  related  to  pemphigus  is  the  disease  known  as  pompholyx 
(cheiropompholyx,  dysidrosis).  Here  the  vesicles  are  more  deeply 
seated  in  the  palms  of  the  hands  and  soles  of  the  feet.  The  condition 
is  an  inflammatory  one.  The  vesicles  form  in  the  upper  layers  of  the 
rete,  the  cells  of  which  are  compressed  and  pushed  apart,  and  are  filled 
with  serum,  fibrin,  and  later  with  leukocytes.  The  sweat-glands, 
almost  without  exception,  are  unaltered.  The  upper  layers  of  the  corium 
show  slight  inflammation.  The  affection  is  foun'd  most  often  in  those 
of  a  nervous  temperament,  or  who  suffer  from  worry  or  overwork. 

In  hydroa  vacciniforme  vesicles  form  upon  the  skin,  not  unlike  those 
found  in  variola.  They  present  a  central  dark  depression  and  ulti- 
mately heal,  forming  a  scar.  The  disease  is  a  rare  one,  beginning 
usually  in  infancy,  and  appears  to  follow  exposure  to  the  sun's  rays, 
and  to  heat  and  cold.  The  process  seems  to  begin  with  inflammation 
of  the  upper  layers  of  the  corium,  followed  by  the  formation  of  vesicles 
in  the  middle  portion  of  the  rete.  This  gives  place  to  a  sharply  defined 
necrosis,  involving  the  rete,  the  upper  part  of  the  corium,  and  the  lower 
layers  of  the  stratum  corneum. 

Psoriasis  is  a  rather  common  affection  of  the  skin  which  begins  with 
the  formation  of  small  brownish-red  papules  that  in  the  course  of  a 
few  days  become  topped  with  a  whitish  silvery  scale.  This  when 
removed  leaves  a  somewhat  reddish-brown  surface  in  which  may  be 
seen  a  small  bleeding  point.  In  some  cases  the  patches  may  be  of 
considerable  size.  As  the  process  heals,  the  scale  is  exfoliated  and  the 


DERMATITIS  OF  UNKNOWN  AND  DOUBTFUL  ETIOLOGY     939 


FIG.  249 


epidermis  returns  to  its  normal  condition,  save  that  a  certain  amount 
of  pigmentation  usually  remains.  Sometimes  the  patches  heal  at  the 
centre  while  extending  at  the  periphery  (psoriasis  annularis  sive  gyrata). 
The  lesions  of  psoriasis  usually  affect  chiefly  the  extensor  surfaces  of 
the  limbs  in  the  neighborhood  of  the  joints,  the  sacral  region,  and  on 
the  hairy  parts  of  the  head. 
Any  part  of  the  body,  how- 
ever, may  be  involved,  even 
the  nails. 

Histological  examination 
shows  the  horny  layer  to  be 
considerably  thickened,  the 
cells  being  more  or  less  dis- 
sociated from  each  other,  so 
that  spaces  are  formed  which 
contain  cell-debris,  bodies 
that  are  thought  by  some  to 
be  micrococci,  and  air.  This 
presence  of  air  gives  the  scales 
their  characteristic  silvery 
sheen.  Cornification  is  im- 
perfect. The  stratum  granu- 
losum  is  in  places  thickened, 
in  others,  thinned  or  absent. 
The  cells  of  the  rete  show 
proliferative  changes.  There 
are  effusion  of  serum  and 
extravasation  of  cells  into 
the  corium.  In  advanced 
cases  there  is  overgrowth  of 
the  papillae,  together  with 
hyperplasia  of  connective 
tissue,  and  the  process  may 
spread  even  to  the  subcuta- 
neous structures.  These  ap- 
pearances have  been  variously 
interpreted.  Some  have  held  that  the  inflammation  in  the  corium 
is  primary,  but  later  observers  think  that  it  is  secondary  to  the  changes 
in  the  rete  and  the  deeper  layers  of  the  epidermis. 

The  disease  is  an  obstinate  one,  and  certain  individuals  seem  to  have 
a  special  predisposition  to  it.  In  such  persons  slight  external  irritation 
may  precipitate  an  attack.  It  is  common  also  in  those  of  a  rheumatic 
or  gouty  tendency. 

Lichen  ruber  acuminatus  (Kaposi)  is  characterized  by  the  formation 
of  minute,  hard,  reddish  papules,  covered  at  their  summits  with  thickened 
epidermis.  These  enlarge  by  peripheral  growth,  until  large,  diffuse, 
reddish,  scaly  patches  are  produced.  Eventually  the  whole  body  may 
be  involved. 


Psoriasis.     (From  Dr.  Shepherd's  Skin  Clinic,  Mon- 
treal General  Hospital.) 


940 


THE  SKIN 


Histologically,  one  finds  overgrowth,  and  imperfect  cornification  of 
the  horny  layer,  involving  the  outlets  of  the  sebaceous  follicles.  There 
is  a  cellular  infiltration  about  the  vessels  of  the  corium,  the  sweat- 
glands,  and  the  papillary  bodies.  The  rete  is  thickened  and  the  inter- 
papillary  processes  are  irregularly  hypertrophied.  The  disease  is  now 
generally  believed  to  be  the  same  as  the  pityriasis  rubra  pilaris  of  the 
French  school.  The  cause  is  not  known. 


FIG.  250 


•fetot 


Lichen  ruber  acuminatus  in  a  negro.      (Howard  Fox's  case.) 

In  lichen  ruber  planus  the  nodes  are  flat,  somewhat  concave,  and  of 
a  glistening,  pale  or  reddish  waxy  appearance.  Crocker1  holds  that 
the  process  is  an  inflammation  of  the  upper  layers  of  the  corium  with 
secondary  involvement  of  the  epidermis. 

The  disease  is  said  to  occur  most  frequently  in  those  of  a  neurotic 
disposition  or  who  are  suffering  from  some  derangement  of  the  nervous 
system. 

Hypertrophic  and  verrucous  forms  are  described. 

Lupus  erythematosus  is  a  rather  uncommon  affection  of  the  skin, 
found  most  often  in  women,  and  during  the  third  decade  of  life.  The 
etiology  is  quite  obscure.  Some  believe  the  disease  to  be  a  form  of 
tuberculosis,  but  this  view  is  not  supported  by  a  study  of  the  lesions. 

According  to  Kaposi,  lupus  erythematosus  begins  with  the  formation 
of  small,  elevated,  reddish  patches  the  size  of  a  pin-head  up  to  that  of 
a  lentil.  These  are  somewhat  depressed  in  the  centre,  which  has  a 
glistening  scar-like  appearance  or  is  covered  with  thin  adherent  scales. 
The  process  may  heal  at  the  centre  and  advance  at  the  periphery  (lupus 
erythematosus  discoides).  In  other  cases  the  disease  progresses  by 


Diseases  of  the  Skin,  second  edition,  1893. 


SECONDARY  OR  SYMPTOMATIC  DERMATITIS  941 

the  formation  of  secondary  foci  (lupus  erythematosus  disseminatus). 
The  lesions  may  be  found  on  the  face,  the  fingers,  toes,  knees,  and  elbows. 
When  on  the  face  a  somewhat  characteristic  appearance  is  produced 
in  the  form  of  a  butterfly-shaped  patch  of  redness  extending  across  the 
bridge  of  the  nose  over  both  cheeks. 

The  lesion  appears  to  consist  in  an  inflammation  of  the  upper  layers 
of  the  corium,  in  the  neighborhood  of  the  hair-follicles,  sebaceous 
glands,  and  bloodvessels.  This  is  shown  by  the  presence  of  a  more 
or  less  extensive  infiltration  of  the  tissues  with  leukocytes,  and  hyper- 
plasia  of  the  connective  tissue.  The  specific  cells  of  the  glands  show 
some  overgrowth,  but  in  course  of  time,  owing  to  retrograde  changes 
and  the  fibrous-tissue  overgrowth,  the  glands  tend  to  atrophy  and  dis- 
appear. The  papillae  and  interpapillary  process  also  are  destroyed. 
Small  hemorrhages  may  be  observed,  and  the  vessels  show  some  end- 
arteritis,  with  occasionally,  thrombosis  or  embolism.  The  epidermis 
is  swollen,  heaped  up  into  scales,  or  elevated  into  vesicles.  Later, 
it  becomes  thin  and  atrophic.  The  caseous  nodes  with  giant  cells, 
characteristic  of  lupus  vulgaris,  are  not  met  with  here. 

Secondary  or  Symptomatic  Dermatitis. — The  Exanthemata. — There 
are  a  number  of  systemic  diseases,  some  of  them  certainly  and  others 
probably  of  the  nature  of  infections,  in  which  skin  lesions  are  a  more  or 
less  constant  and  characteristic  accompaniment.  The  cutaneous  mani- 
festations may  be  comparatively  trifling,  as  in  the  diffuse  erythemas 
which  are  occasionally  met  with  in  sepsis,  or,  again,  may  be  so  striking 
as  to  dominate  the  clinical  picture.  The  diseases  belonging  to  the  latter 
group  are  known  as  the  exanthemata.  Chief  among  them  are  measles, 
scarlatina,  varicella,  variola,  typhoid,  and  typhus,  to  which  may  perhaps 
be  added  secondary  syphilis. 

The  pathogenesis  of  such  manifestations  is,  perhaps,  to  be  explained 
as  the  effect  of  circulating  toxins  upon  the  bloodvessels,  either  directly 
or  through  the  vasomotor  system,  and  by  the  local  action  of  the  toxins 
upon  the  epidermis.  According  to  Unna,  in  such  cases  when  inflam- 
matory changes  are  present  they  are  secondary  and  unessential. 

The  exanthem  of  measles  is  met  with  first  on  the  mucous  membrane  of 
the  lips  and  mouth  in  the  form  of  small  bright  red  spots,  in  the  centre 
of  which  are  minute  bluish- white  points  (Koplik's  spots).  These  may 
appear  from  twenty-four  or  forty-eight  hours  to  three  or  even  five  days 
before  the  eruption  appears  on  the  skin.  Occasionally,  they  may  be 
found  before  any  catarrhal  symptoms  manifest  themselves.  The  skin 
lesions  appear  upon  the  neck,  face,  forehead,  trunk  and  limbs,  in  the 
order  named. 

The  spots  are  dull  red  blotches  in  the  skin,  or  sometimes  slightly 
raised  above  the  general  level.  They  are  irregular,  often  crescentic  in 
shape,  and  in  places  may  become  confluent.  The  skin  and  subcuta- 
neous tissues  are  in  parts  somewhat  cedematous.  In  the  course  of  a 
few  hours,  or  two  or  three  days,  the  eruption  begins  to  pale,  and  gradually 
disappears,  leaving  a  slight  yellowish  pigmentation,  with  fine  desquama- 
tion  of  the  epidermis.  Rarely,  the  eruption  may  be  hemorrhagic. 


942  THE  SKIN 

Histologically,  one  finds  usually  congestion  of  the  bloodvessels  and 
lymphatics  with  oedema  of  the  corium.  In  the  papular  form  there  is 
in  addition  slight  diapedesis  of  leukocytes  about  the  vessels,  around 
the  glands,  and  in  the  papillary  bodies.  Minute  hemorrhages  are  also 
occasionally  met  with.  In  the  most  severe  forms  there  may  be  necrosis 
of  the  epithelium. 

The  eruption  in  scarlatina  makes  its  appearance  first  upon  the  neck 
and  upper  part  of  the  thorax  in  front,  extending  rapidly  to  the  rest  of 
the  trunk,  and  finally  to  the  extremities.  The  skin  presents,  at  first, 
the  appearance  of  a  fiery,  reddish-pink  blush.  When  examined  more 
closely,  the  rash  is  found  to  consist  of  innumerable  fine  reddish  points 
closely  set  together.  Occasionally,  the  exanthem  is  more  distinctly 
papular  (scarlatina  papulosa),  or  may  be  associated  with  the  formation 
of  vesicles  and  blebs  (scarlatina  vesicularis  et  pemphigoides).  Hemor- 
rhages into  the  skin  may  also  occur  (scarlatina  hemorrhagica).  After 
a  variable  period,  from  one  to, seven  days,  the  eruption  assumes  a  more 
dusky  red  or  livid  appearance  and  gradually  fades,  leaving  a  slightly 
pigmented  surface.  Finally,  the  epidermis  desquamates  in  the  form 
of  fine  impalpable  scales  (desquamatio  furfuracea)  or  in  large  flakes 
(desquamatio  membranacea). 

Histologically,  the  bloodvessels  of  the  skin  are  found  to  be  con- 
gested and  in  a  state  of  paralytic  dilatation.  It  is  said  by  most,  although 
this  is  denied  by  Unna,  that  there  is  an  inflammatory  exudate  in  the 
interstitial  tissues,  consisting  of  plasma,  leukocytes,  and  extravasated 
red  blood-cells.  The  rete  cells  appear  also  to  be  rapidly  proliferating. 

Recently,  Mallory1  has  described  certain  bodies  in  and  between 
the  epithelial  cells  of  the  epidermis  and  free  in  the  superficial  lymph- 
vessels  and  spaces  of  the  corium,  which  he  is  inclined  to  think  are 
protozoa  and  the  specific  cause  of  scarlatina. 

In  variola  the  eruption  appears  about  the  fourth  day  in  the  form  of 
small,  firm,  shotty  papules  of  a  reddish  color.  The  papules  are  met 
with  first  on  the  wrists  and  on  the  forehead  about  the  border  of  the  hair, 
but  scattered  papules  quickly  form  elsewhere  on  the  face  and  on  the 
trunk  (discrete  smallpox).  On  the  fifth  or  sixth  day  the  papules  are 
transformed  into  vesicles,  which  are  elevated,  rounded,  and  depressed 
in  the  centre  or  umbilicated.  About  the  eighth  day  the  vesicles  become 
pustules.  These  are  rounded,  have  lost  the  central  umbilication, 
becoming  thereby  more  spherical,  and  are  bounded  by  a  zone  of  hyper- 
emia.  The  intervening  skin  is  usually  somewhat  swollen.  In  the  course 
of  ten  or  eleven  days,  in  favorable  cases,  the  pustules  gradually  dry 
up,  forming  crusts,  under  which  regeneration  of  tissue  gradually  goes 
on.  After  the  crusts  are  cast  off  there  does  not  usually  result  any 
scarring  unless  the  papillary  layer  has  been  involved. 

In  the  form  of  smallpox  modified  by  vaccination,  known  as  varioloid, 
vesiculation  and  maturation  take  place  more  rapidly,  and  the  pocks  are 
much  fewer  in  number. 

1  Jour.  Med.  Research,  10:  No  4:  1904:  483. 


SECONDARY  OR  SYMPTOMATIC  DERMATITIS  943 

Much  more  severe  is  the  confluent  smallpox.  Here  the  papules  are 
much  more  numerous  and  closely  set,  and  while  at  first  they  may  be 
discrete,  they  soon  become  more  or  less  fused,  so  that  large  areas  of  the 
skin  are  transformed  practically  into  an  extensive  superficial  abscess. 
The  skin  is  greatly  swollen  and  oedematous.  The  separation  of  the 
scabs,  in  the  cases  that  recover,  is  a  slow  process,  and  scarring  is  often 
extreme. 

Occasionally,  the  eruption  assumes  a  hemorrhagic  type.  Of  this, 
two  varieties  are  recognized;  the  first,  purpura  variolosa,  in  which  the 
hemorrhagic  extravasation  appears  early,  and  the  second,  in  which 
the  effusion  of  blood  occurs  after  the  vesicles  and  pustules  have  been 
formed,  variola  pustulosa  hemorrhagica. 

The  process,  as  studied  histologically,  begins  with  local  hyperemia 
of  the  papillaB  with  exudation  of  inflammatory  products  into  the  rete. 
The  exudation  leads  to  cedema  of  the  cells,  which  are  also  from  the 
pressure  of  the  out-poured  fluid,  dissociated  more  or  less  from  each  other, 
and  compressed  into  a  filamentous  meshwork.  In  this  way,  owing  in 
part  to  interstitial  exudation,  and  in  part  to  colliquative  necrosis  of 
the  rete  cells,  a  vesicle  is  formed  which  is  peculiar  in  that  it  is  traversed 
by  a  number  of  delicate  bands  composed  of  the  compressed  epithelial 
cells.  The  vesicles  contain  plasma,  cell  detritus,  nuclear  fragments, 
and  fibrin.  The  superficial  epidermis  is  in  this  way  elevated  above 
the  general  level.  About  the  periphery  the  tissues  are  congested  and 
more  or  less  infiltrated  with  leukocytes.  These  gradually  work  their 
way  into  the  vesicle,  and,  gradually  increasing  in  numbers,  convert  it 
into  a  pustule.  The  delicate  septa,  before  referred  to,  give  way  and 
the  pustule  assumes  a  more  spherical,  elevated  appearance.  The 
papillary  layer  is  usually  intact,  but  in  the  more  severe  cases  there 
may  be  infiltration  of  the  corium  with  necrosis,  resulting  in  the  forma- 
tion of  a  scar  in  the  process  of  healing.  Certain  spherical  bodies, 
believed  to  be  sporozoa,  have  been  described  by  numerous  observers 
as  occurring  both  within  the  nucleus  and  in  the  protoplasm  of  the  epi- 
thelial cells  in  the  pock,  which  have  been  held  to  be  the  specific  cause 
'of  the  disease.  The  most  recent  and  important  work  on  these  lines 
is  that  of  Councilman,  Magrath,  and  Brinckerhoff,1  who  conclude  that 
they  have  discovered  the  specific  cause  of  variola.  The  life  history 
of  their  organism  has  been  studied  by  Calkins,2  who  identifies  it  with 
the  cytoryctes  of  Guarnieri.  Inasmuch  as  it  has  hitherto  proved  to 
be  impossible  to  cultivate  the  sporozoa  on  artificial  media,  the  deter- 
mination of  the  etiological  importance  of  these  organisms  is  rendered 
excessively  difficult,  and  we  feel  that  until  further  light  is  forthcoming 
the  whole  subject  must  remain  sub  judice. 

The  lesions  of  vaccinia  are  in  all  respects  comparable  to  those  of 
variola,  both  in  their  clinical  progression  and  in  histological  structure. 

1  The  Pathological  Anatomy   and   Histology   of  Variola,  Jour.  Med.  Research, 
80:1904:  12. 

2  The  Life-history  of  Cytoryctes  Variolse,  Guarnieri,  ibid.,  136. 


944  THE  SKIN 

Varicella  bears  a  general  resemblance  to  variola,  but  the  pocks  are 
formed  more  rapidly,  are  usually  more  scanty  than  in  unmodified 
smallpox,  and  the  disease  altogether  is  attended  with  but  trifling  dis- 
comfort. The  pocks  begin  as  reddish  papules,  which  rapidly  are  con- 
verted into  clear,  pearly  vesicles.  They  quickly  become  slightly  turbid, 
or  occasionally,  pustular,  and  finally  dry  up.  Each  vesicle  is  sharply 
defined,  considerably  elevated,  and  surrounded  by  a  hyperemic  zone. 
Scarring,  as  a  rule,  does  not  take  place. 

Histologically,  the  lesion  somewhat  closely  resembles  that  of  variola, 
but  is  more  superficial.  The  vesicle  formation  both  in  varicella  and 
in  zoster  is  attributed  by  Unna  to  a  peculiar  form  of  cell-degeneration 
which  he  terms  "ballooning  colliquation."  Certain  multinucleated 
cells  have  been  found  in  the  vesicles  in  both  the  diseases  just  mentioned, 
and  have  been  regarded  by  some  as  parasites,  although  this  is  believed 
by  Gilchrist1  to  be  erroneous. 

The  rash  in  typhoid  fever  is  characterized  by  the  formation  of  isolated, 
slightly  elevated  papules,  of  a  rose  pink  color  (rose  spots),  which  appear 
first  upon  the  abdomen  and  the  lower  thoracic  zone.  As  a  rule,  the  spots 
are  rather  few  in  number,  but  in  some  cases  the  eruption  is  abundant, 
involving  the  back,  and  even  the  extremities.  The  papules  come  out 
in  successive  crops  after  the  end  of  the  first  week  and  in  two  or  three 
days  gradually  disappear,  leaving  a  slight  brownish  stain.  The  spots 
are  from  2  to  4  mm.  in  diameter,  are  palpable,  and  disappear  momen- 
tarily on  pressure.  Occasionally,  the  rash  is  sudaminal  in  character. 
Rarely,  the  eruption  is  hemorrhagic.2  After  the  rash  has  disappeared 
the  skin  may  desquamate.  The  spots  are  largely  congestive  in  char- 
acter and  contain  the  specific  bacillus. 

In  typhus  fever  the  rash  is  rather  characteristic.  It  appears  usually 
between  the  third  and  fifth  days,  and  in  the  course  of  two  or  three  days 
more  is  completely  out.  The  eruption  is  composed  of  two  elements, 
papular  rose  spots  and  petechise.  The  rose  spots  and  the  petechise 
appear  together,  or  else  many  of  the  rose  spots  become  hemorrhagic. 
The  skin  between  the  spots  presents  a  curious,  apparently  deep-seated 
or  subcuticular  mottling  of  a  dusky-red  color.  Sudamina  are  not  com- 
mon. In  the  case  of  children  the  disease  has  been  mistaken  for  measles. 
The  rash  does  not  disappear  after  death. 

Syphilis. — In  the  secondary  stage  of  syphilis,  that  is  to  say,  after  the 
infection  has  become  systemic,  skin  eruptions  are  an  almost  constant 
feature.  It  is  characteristic  of  syphilitic  exanthemata  that  they  are 
extremely  polymorphous;  so  much  so  that  the  lesions  may  simulate 
almost  any  of  the  ordinary  skin  eruptions.  Consequently  many  writers 
speak  of  syphilitic  roseola,  lichen,  eczema,  psoriasis,  impetigo,  herpes, 
pemphigus,  and  so  on. 

The  most  common  efflorescence  upon  the  skin  occurring  in  secondary 

1  Johns  Hopkins  Hosp.   Rep.,   1. 

2  See  Nicholls  and  Learmonth,  The  Hemorrhagic  Diathesis  in  Typhoid  Fever, 
Lancet,  London,  1 :  1901 :  305. 


SECONDARY  OR  SYMPTOMATIC  DERMATITIS 


945 


lues  is  the  so-called  syphilitic  roseola,  or  maculopapular  syphilide. 
This  appears  usually  upon  the  trunk,  but  may  extend  to  the  arms  and 
other  parts  of  the  body.  The  face  may  be  exempt.  The  patches  are 
reddish-brown  in  color,  somewhat  elevated  above  the  general  surface, 
varying  in  size  from  that  of  a  lentil  to  that  of  a  bean,  and  tend  to  be 
symmetrically  distributed.  After  one  or  two  weeks  the  spots  become 
a  dirty  brown  or  gray  color  and  gradually  disappear. 

FIG.  251 


Secondary  macular  syphilide.      (From  Dr.  Shepherd's  Skin  Clinic,  Montreal  General  Hospital.) 

Microscopically,  the  superficial  vessels  are  dilated  and  show  both 
endothelial  and  perithelial  proliferation.  There  is  more  or  less  extra- 
vasation of  leukocytes  about  the  vessels  and  glands,  together  with  some 
cedema.  Veiklon  and  Girard,  among  others,  have  found  the  Spirochaeta 
pallida  in  this  lesion. 

The  papular  syphilide  assumes  various  forms.  It  begins  with  the 
formation  of  reddish  patches,  the  size  of  a  pin-head  or  larger,  within 
which  acuminate  or  flattened  papules  develop.  When  the  papules 
are  small  the  condition  bears  a  general  resemblance  to  lichen  ruber, 
and  is  known  as  lichen  syphiliticus.  In  some  cases,  vesicles  or  pustules 
form  upon  the  papules  (herpes  syphiliticus,  impetigo  syphilitica),  which 
dry  up  into  scales  or  crusts.  This  is  less  common  than  the  acniform 
eruption.  Where  the  lesions  involve  the  soles  of  the  feet  and  palms 
60 


946 


THE  SKIN 


of  the  hands,  the  papules  are  flattened,  and  when  involution  has  taken 
place  are  attended  with  the  production  of  abundant  scales  (psoriasis 
plantar  is  et  palmaris  syphilitica). 

In  regions  where  warmth  and  moisture  are  complicating  conditions 
the  papules  are  converted  into  the  so-called  condylomas,  which  are  flat 
elevations,  of  a  grayish  color,  with  a  moist,  shiny  surface,  sometimes 
secreting  an  offensive  discharge. 

Microscopically,  in  the  papular  syphilide  the  process  is  chiefly  to  be 
observed  in  the  papillary  layer,  but  may  also  extend  to  the  deeper  parts 
of  the  corium. 

The  Spirochseta  pallida  has  repeatedly  been  found  in  secondary  papules 
(Levaditi,1  Buschke,  and  Fischer),  and  in  the  condyloma  (Blaschko2). 

FIG.  252 


Syphilitic  rupia  on  the  arm.     (From  the  Skin  Clinic  of  the  Montreal  General  Hospital.) 

The  tubercular  syphilide  is  slower  in  development  and  more  inveterate 
than  the  papule.  The  process  may  be  diffuse  but  is  more  often  cir- 
cumscribed. There  may  be  more  or  less  absorption  in  the  centre  of 
the  nodules,  or,  again,  central  necrosis  may  result  in  the  formation  of 
an  ulcer.  In  the  transition  period  from  the  secondary  to  the  tertiary 
stage,  the  lesions  may  be  covered  with  extensive  crusts,  presenting  a 
curious  and  characteristic  concentric  arrangement,  something  like 
an  oyster  shell — syphilitic  rupia. 

1  Comptes  rend.  Soc.  de.  Biol.,  Paris,  59:  1905:  527. 

2  Blaschko,  Med.  klin.  Woch.,  1906. 


DERMATITIS  FROM  DRUGS  OR  OTHER  TOXIC  SUBSTANCES      947 

The  lesion  results  in  some  destruction  of  tissue  so  that  more  or  less 
scarring  results. 

Gummas  frequently  originate  in  the  subcutaneous  structures  and 
later  involve  the  skin.  They  sometimes  result  in  much  destruction  of 
tissue  and  are  followed  by  scarring  and  pigmentation. 

Frambcesia  (yaws)  is  an  endemic  and  infectious  disease,  found  in 
certain  tropical  regions,  which  bears  a  general  close  resemblance  to 
syphilis,  and,  indeed,  by  some  is  regarded  as  being  identical  with  it. 
As  in  syphilis  there  is  a  preliminary  period  of  incubation,  after  which 
a  primary  lesion  appears  at  the  point  of  inoculation,  followed  later  by 
various  skin  manifestations,  and  often  general  glandular  enlargement. 
The  later  lesions  are  usually  to  be  found  in  the  upper  layers  of  the  cutis 
and  are  fungoid  in  character.  When  the  superficial  crust  is  removed 
they  present  the  appearance  of  a  raspberry.  Histologically,  they 
resemble  the  other  infective  granulomas. 

Dermatitis  from  the  Ingestion  of  Drugs  or  Other  Toxic  Substances. — A 
great  variety  of  skin  lesions  have  been  described  as  the  result  of  the 
ingestion  of  certain  drugs  and  allied  substances,  taken  either  unwittingly 
or  for  medicinal  purposes.  In  some  cases  these  are  due  to  the  direct 
action  of  the  drug;  in  others,  to  certain  toxic  principles  elaborated  in 
the  process  of  absorption  and  elimination.  The  character  of  the  lesion 
attributable  to  any  one  drug  is  by  no  means  constant.  Acniform 
eruptions  are  fairly  characteristic  of  the  iodine  and  bromine  compounds ; 
scarlatiniform  rashes,  of  belladonna,  antipyrine,  and  quinine,  and 
diphtheria  antitoxin.  Purpura  has  been  described  after  the  exhibition 
of  copaiba,  quinine,  belladonna,  ergot,  mercury,  and  iodide  of  potassium. 
Salicylic  acid  and  its  compounds  may  produce  erythema,  purpura,  or 
even  necrosis. 

It  should  be  mentioned  that  these  results  sometimes  follow  doses  of 
the  drugs  well  within  therapeutic  limits,  and  do  not  always  imply  care- 
less or  extravagant  dosage.  Personal  idiosyncrasy  must  often  be  taken 
into  account. 

Ergotism  results  from  the  use  of  grain  infested  with  the  ergot  fungus. 
Two  forms  are  described,  the  convulsive  or  spasmodic,  and  the  gan- 
grenous. In  the  latter  variety  the  fingers  and  toes,  less  commonly 
the  nose  and  ears,  become  gangrenous. 

Allied  to  ergotism  is  pellagra,  a  disease  found  in  certain  parts  of 
Europe  among  the  peasantry  who  eat  decomposing  grain,  usually  maize. 
According  to  an  Italian  observer,  Ceni,1  the  specific  cause  is  a  fungus 
belonging  to  the  aspergillus  family.  One  of  the  earliest  manifestations 
is  erythema  of  the  skin.  Later,  the  skin  becomes  dry  and  harsh  and 
exfoliates.  Sometimes  crusts  are  formed  under  which  pus  is  secreted. 
With  this  there  is  gastro-intestinal  disturbance  followed  in  aggravated 
cases  by  paraplegia  and  sometimes  melancholia. 

1  Riv.  sper.  di  fremat.,  Reggio-Emilia,  33:  1907:  1. 


948  THE  SKIN 


RETROGRESSIVE  METAMORPHOSES. 

Atrophy. — Simple  atrophy  of  the  skin  is  characterized  by  the  wasting 
of  almost  all  its  elements.  The  condition  may  be  local  or  generalized. 
It  may  be  primary  or  the  result  of  some  preexisting  pathological  state. 
As  a  type,  may  be  taken  the  physiological  atrophy  found  in  old  age. 
Here  the  cutis  becomes  thinner,  and  the  papillae  tend  to  be  flattened 
and  may  disappear,  while  the  epidermis  becomes  dry  and  brittle.  The 
subcutaneous  fat  is  to  some  extent  absorbed,  and  the  skin  is  thus 
thrown  into  folds.  The  elastic  tissue  involutes  and  the  superficial 
vessels  undergo  degeneration.  It  is  not  uncommon  to  find  granules  of 
pigment  of  brownish  color  in  the  cells  of  the  rete  and  about  the  vessels 
of  the  cutis.  The  deeper  layers  of  the  epidermis  are  atrophied,  so  that 
the  stratum  corneum  is  less  widely  separated  from  the  papillary  layer. 
The  hair-follicles  also  partake  in  the  process  and  the  hairs  become 
downy  and  eventually  fall  out.  The  openings  of  the  follicles  not  infre- 
quently become  blocked,  owing  to  the  accumulation  of  epidermal  scales, 
and  they  may  be  dilated  into  cysts.  The  sebaceous  glands  similarly 
are  obstructed,  and  the  hair-follicles  and  sebaceous  glands  are  dis- 
tended into  one  cavity  containing  hairs,  fat,  and  epithelial  debris, 
the  so-called  atheroma.  Eventually,  the  sebaceous  glands  atrophy  and 
finally  disappear.  Not  infrequently  the  superficial  epidermis  is  heaped 
up  here  and  there  into  branny  scales  (pityriasis  simplex). 

Local  atrophy  is  often  brought  about  by  distension  of  the  skin  from 
whatever  cause.  The  regions  usually  affected  are  the  breasts,  abdomen, 
and  thighs.  The  commonest  cause  is  pregnancy,  but  similar  effects 
are  sometimes  produced  by  tumors,  lactation,  ascites,  and  anasarca. 
During  pregnancy  the  abdomen  is  covered  with  reddish  livid  streaks, 
which  after  delivery  are  transformed  into  whitish  silvery  lines  or  scars 
(linese  albicantes).  On  examining  such  a  scar,  the  papillse  are  seen  to 
be  flattened  or  absent,  the  connective-tissue  fibers  of  the  corium  are 
dissociated,  the  elastic  fibers  and  bloodvessels  are  atrophic. 

A  somewhat  similar  local  atrophy  of  the  skin  is  found  after  the 
absorption  of  the  subcutaneous  fat  in  the  course  of  chronic  wasting 
diseases,  and  even  in  certain  acute  febrile  processes,  notably  typhoid 
(Osier).  Linese  atrophicse  are  also  met  with  on  the  thighs  and  abdomen 
as  the  result  of  the  pressure  of  corsets  or  other  articles  of  apparel. 

Idiopathic  diffuse  symmetrical  atrophy  has  been  reported  by  several 
observers  (Bronson,1  Elliott,  and  Fordyce). 

Neurotrophic  Atrophy. — In  certain  nervous  affections,  such  as  leprosy, 
neuralgia,  and  neuritis,  the  skin  supplied  by  the  nerves  involved  is  often 
found  to  be  thin,  smooth,  and  shiny,  and  there  may  be  wasting  of  the 
glands  and  hair-follicles  (Paget,  Weir-Mitchell). 

Ulceration. — Bedsores. — Bedsores  (decubitus)  are  a  form  of  necrosis 
of  the  skin  and  underlying  parts  due  largely  to  pressure  in  those  who  are 

1  Jour.  Cutan.  and  Genitourinary  Dis.,  13 :  1895 :  1. 


AINHUM  949 

greatly  reduced  in  health.  Impoverished  blood,  a  weak  heart,  and 
the  recumbent  position  are  the  most  important  predisposing  causes. 
Neurotrophic  influences,  however,  often  play  a  part,  for  bedsores  are 
apt  to  develop  with  exceptional  rapidity  in  nervous  affections,  such  as 
tabes  dorsalis  and  myelitis.  The  regions  usually  involved  are  the 
sacrum,  trochanters,  heels,  and  scapulae.  The  affected  part  becomes 
bluish-black  or  black  in  color,  the  skin  cracks,  and  then,  owing  to 
infection  with  putrefactive  germs,  a  spreading  ulcer  or  gangrenous 
patch  is  formed,  which  extends  to  the  subcutaneous  soft  tissues  and 
even  to  the  bone. 

Perforating  Ulcer. — Somewhat  allied  to  this  is  the  perforating 
ulcer  of  the  foot,  met  with  in  locomotor  ataxia  and  other  diseases  of 
the  spinal  cord.  A  deeply-penetrating  ulcer,  which  extends  rapidly, 
is  produced,  usually  at  the  metatarsophalangeal  joint  of  the  great  toe. 
The  process  may  lay  bare  and  erode  the  bone.  It  may  possibly  be 
due  to  pressure,  but  in  the  majority  of  instances  is  of  neurotrophic 
origin. 

Small  ulcerations  are  also  found  on  the  hands  in  syringomyelia, 
an  affection  of  the  spinal  cord  in  which  the  sensory  fibers  are  largely 
interfered  with.  These  ulcers  are  probably,  to  some  extent  at  least, 
to  be  referred  to  trauma  or  irritation,  which  more  readily  occur  in  cases 
where  sensation  is  impaired. 

Varicose  Ulcers. — Ulceration  also  occurs  as  a  result  of  impaired  circu- 
lation, as,  for  example,  in  the  lower  extremities  as  a  result  of  varicose 
veins. 

Gangrene. — Gangrene  of  the  skin  and  other  tissues  is  met  with  in 
chronic  ergotism. 

Senile  Gangrene. — Senile  gangrene  is  a  form  occurring  in  old  persons 
who  have  advanced  arteriosclerosis.  It  generally  begins  in  one  of  the 
toes  and  may  spread  gradually  upward.  It  is  usually  of  the  dry  variety, 
and  is  often  induced  by  some  slight  injury,  such  as  may  be  caused  by 
paring  a  corn,  or  a  rag-nail.  Gangrene  also  is  met  with  occasionally 
in  diabetes  mellitus. 

Raynaud's  Disease. — Raynaud's  disease,  when  extreme,  leads  to  sym- 
metrical gangrene  of  the  extremities,  supposed  by  some  to  be  due  to  a 
primary  nervous  disturbance.  It  is  also  attributed  to  anemia  brought 
about  by  repeated  spasm  of  the  arterioles. 

Ainhum. — Ainhum  is  a  rare  disease  of  unknown  etiology,  first 
described  by  Clark1  and  found  especially  in  the  African  races.  A 
circular  groove  is  formed,  usually  on  the  fourth  or  fifth  toes,  at  the 
point  where  the  toe  joins  the  foot.  The  toe  swells,  and  the  affected 
part  becomes  rough  and  scaly.  Finally  spontaneous  amputation  of 
the  member  takes  place. 

Histologically,  it  has  been  regarded  by  several  observers,  notably 
Unna,  as  a  form  of  scleroderma,  in  which  the  epidermis  is  thickened, 
the  papillae  are  narrowed  and  lengthened,  and  the  overgrowth  finally 

1  Clark,  Trans.  Epidemiol.  Soc.,  Lond.,  1860. 


950  THE  SKIN 

leads  to  strangulation  of  the  deeper  layers  with  consecutive  necrosis. 
The  vessels  of  the  cutis  are  dilated  and  there  is  some  round-celled 
infiltration,  with  obstruction  to  the  lymphatics.  Cases  have  been 
reported  in  this  country  by  F.  J.  Shepherd. 

Colloid  Transformation. — Colloid  transformation  of  the  skin  (colloid 
milium)  is  a  rare  disease  in  which  the  cells  of  the  derma  and  the  con- 
nective tissue  undergo  colloid  changes.  The  degeneration  is  most 
marked  about  the  bloodvessels,  nerves,  and  sebaceous  glands. 


ABNORMAL  PIGMENTATION. 

The  anomalies  of  pigmentation  fall  into  two  classes :  the  first,  in  which 
there  is  a  diminution  or  increase  in  the  amount  of  the  normal  coloring 
matter  of  the  skin,  and  the  second,  in  which  there  is  a  deposit  of  patho- 
logical or  extraneous  pigments. 

Congenital  absence  of  the  pigment,  leukopathia  congenita  or  albinism, 
has  already  been  referred  to  (p.  908). 

Acquired  leukopathia,  leukoderma,  or  vitiligo  occurs  spontaneously,  or, 
occasionally,  after  the  infectious  fevers.  It  is  apt  to  begin  in  early 
life,  and  is  commoner  in  the  black  races.  In  certain  regions,  as  in 
Turkestan,  it  is  said  to  be  endemic.  The  disease  is  characterized  by 
the  production  of  whitish,  pigment-free,  blotches,  often  symmetrical, 
usually  on  the  face,  neck  and  hands.  The  condition  tends  to  spread, 
and  by  the  confluence  of  the  patches  large  areas  of  skin  become  white. 
The  hair  upon  the  affected  regions  also  becomes  bleached  (poliosis  cir- 
cumscriptd). 

Histologically,  one  finds  lack  of  pigment  in  the  decolorized  areas, 
with  occasionally  an  increase  of  that  in  the  surrounding  more  normal 
skin,  usually  in  the  corium. 

The  etiology  is  unknown.  Leloir  was  of  the  opinion  that  the  affection 
should  be  referred  to  nervous  influences.  Perhaps  less  obscure  are 
those  cases  of  local  symptomatic  leukoderma  resulting  from  inflamma- 
tion, such  as  the  forms  which  occur  in  boils,  eczema,  lupus,  leprosy, 
and  syphilis.  Here,  the  skin  is  smooth  and  sometimes  cicatrized. 

Lentigo. — Lentigo  is  a  term  somewhat  loosely  employed  to  designate 
sharply  defined  spots,  of  yellowish  or  brownish-black  color,  varying  in 
size  from  that  of  a  pin-head  to  that  of  a  lentil.  They  somewhat  resemble 
small  nevi,  appear  shortly  after  birth,  and  persist  throughout  life. 

Ephelides. — Ephelides,  or  freckles,  are  small,  irregularly  shaped, 
yellowish-brown  blotches,  which  are  found  usually  on  the  face,  hands, 
and  arms,  but  occasionally  on  other  parts  of  the  body.  They  are  most 
common  in  childhood  and  early  life,  and  are  due  to  the  action  of  the 
light,  which  causes  an  increase  of  the  pigment. 

Chloasma. — In  women  who  are  pregnant,  menstruating,  or  who  are 
suffering  from  disease  of  the  genital  organs,  it  is  not  uncommon  to  get 
pigmentation  of  the  skin,  usually  on  the  forehead,  temples,  cheeks,  abdo- 
men, and  breasts.  Bright  brown  or  blackish-brown  patches  of  varying 


PLATE   XI 


Leukoderma   and   Leukonyehia  in   a   Negro.     (Howard   Fox.) 


PIGMENTATION  951 

size,  often  becoming  confluent,  are  produced.  This  is  termed  chloasma 
uterinum.  A  similar  coloration  is  sometimes  found  in  those  suffering  from 
chronic  wasting  disease,  especially  tuberculosis  (chloasma  cachecticorum). 

Addison's  Disease. — In  Addison's  disease  the  skin  and  mucous  surfaces, 
especially  of  the  face,  mouth,  throat,  hands,  breasts,  and  genitalia,  assume 
a  dark  bronzed  appearance.  Microscopically,  all  that  is  seen  is  an  in- 
crease of  pigment  in  the  corium. 

Hemochromatosis. — A  curious  dull,  brownish,  or  bluish  discoloration 
of  the  skin  is  that  known  as  hemochromatosis,  which  is  a  symptom 
associated  with  some  cases  of  cirrhosis  of  the  liver  and  diabetes  (diabete 
bronze). 

Pigmentation  of  the  skin  also  results  from  a  variety  of  thermic,  chemical, 
and  mechanical  insults,  which,  owing  to  their  nature,  produce  chronic 
irritation.  Such  are  sunburn,  scratching,  parasitic  diseases  and  parasites, 
and  the  application  of  mustard  or  fly-blisters. 

The  exact  nature  of  the  increased  pigmentation  in  these  cases  is  quite 
obscure.  It  seems  generally  accepted  now  that  the  normal  pigment  of 
the  skin  is  produced  through  the  metabolism  of  the  epithelial  cells  (Loeb1), 
and  it  may  be  inferred  that  we  are  dealing  with  increased  or,  at  least, 
disordered  metabolism  in  cases  such  as  have  been  mentioned. 

Icterus. — Icterus  is  a  yellowish,  yellowish-green,  or  olive  discoloration 
of  the  skin  and  external  surfaces,  due  to  the  presence  of  bile  in  the  blood 
and  lymph. 

Pigmentation  is  frequently  found  about  varicose  veins,  chronic  and 
healed  ulcers,  and  is  due  to  hemorrhage,  the  effused  blood  being  trans- 
formed into  hemosiderin  and  in  some  cases  into  hematoidin. 

Pigmentation  may  also  be  due  to  the  deposit  of  silver  in  the  vessel- 
walls  and  fibrous  tissue  (argyriasis)  in  persons  who  have  been  taking 
nitrate  of  silver  for  prolonged  periods,  or,  similarly,  to  arsenic  (arsenical 
melanosis).  Among  the  extraneous  pigments  sometimes  found,  also,  in 
the  skin  may  be  mentioned  Indian  ink  and  carmine  (in  tattooing)  and 
gunpowder. 

PROGRESSIVE  METAMORPHOSES. 

Hyperplasia. — Hyperplasia  of  the  skin  is  met  with  under  widely 
differing  conditions  and  in  a  variety  of  forms.  The  congenital  hyper- 
plasias,  such  as  ichthyosis  congenita,  elephantiasis,  and  nevi  have 
already  been  dealt  with  (p.  907  et  seq.).  The  acquired  forms  are  in 
great  measure  due  to  inflammation  and  irritation,  although  in  some  the 
etiology  is  obscure  or  unknown.  Certain  of  them,  again,  are  on  the 
borderland  between  simple  hyperplasia  and  inflammation. 

Callus. — The  simplest  form  of  hyperplasia  is  callus  (callositas,  tyloma), 
so  commonly  met  with  as  the  result  of  intermittent  pressure.  It  is 
found  usually  on  the  palms  of  the  hands  and  soles  of  the  feet.  The 

1  Transplantation  of  Skin  and  Origin  of  Pigment,  Medicine,  Detroit,  5: 1899: 286; 
also,  Journ.  Amer.  Med.  Assoc.,  31 : 1898: 1362. 


952  THE  SKIN 

outer  layers  of  the  epidermis  are  compressed  into  a  dense  homogeneous 
mass.  As  a  result,  the  middle  layers  are  thickened,  while  the  rete 
and  papillae  are  atrophied.  In  advanced  cases  the  cells  of  the  rete 
extend  deeply  into  the  corium,  where  they  become  fused  together  to 
form  a  sort  of  core,  giving  rise  to  the  well-known  corn.  As  a  rule, 
there  is  more  or  less  secondary  inflammation  in  the  neighboring  tissues, 
which  are  swollen  and  hyperemic. 

Cornu  Cutaneum. — Cornu  cutaneum  is  a  remarkable  outgrowth 
of  the  epidermis,  forming  a  horn-like  excrescence,  which  often  reaches 
a  considerable  size.  The  usual  situation  is  on  the  forehead,  breasts, 
and  hands.  The  horns  may  develop  on  otherwise  healthy  skin,  or 
in  connection  with  scars,  atheroma,  or  tumors.  According  to  Unna, 
the  process  is  a  combination  of  acanthosis  with  hyperkeratosis.  The 
cells  of  the  rete  grow  downward  between  the  papillse,  some  of  which 
become  narrowed  and  elongated,  while  others  undergo  atrophy.  As 
the  process  advances  the  horny  layer  becomes  thickened  and  the  cells 
are  heaped  up  until  a  hard  outgrowth  is  the  result. 

Palmar  and  Plantar  Keratodermia. — Palmar  and  plantar  keratodermia 
(tylosis,  keratosis  palmae  et  plantae)  is  a  local  keratosis,  believed  to  be 
the  result  of  disordered  nerve  function.  It  is  a  rare  disease,  and  is 
congenital  or  acquired  early  in  childhood,  but  has  been  known  to  follow 
hyperidrosis  and  the  prolonged  use  of  arsenic.1  A  somewhat  similar 
condition  is  found  in  eczema,  lichen  planus,  syphilis,  and  other  inflam- 
mations of  the  parts. 

Ichthyosis. — The  so-called  acquired  form  of  ichthyosis  develops 
shortly  after  birth,  and  is  met  with  in  all  degrees  of  severity,  from  a 
simple  thickening  of  the  skin,  scarcely  if  at  all  to  be  distinguished 
from  lichen  pilaris,  to  ichthyosis  simplex,  and  finally  to  ichthyosis 
sauroderma  and  ichthyosis  hystrix,  in  which  the  skin  is  thick,  scaly, 
or  warty. 

In  the  milder  grades  there  is,  according  to  Unna,  a  marked  hyper- 
keratosis of  cells  derived  directly  from  the  rete  without  the  interposition 
of  the  granular  layer.  The  cells  of  the  rete  are  small  and  hypoplastic, 
the  papillse  broad  and  flat.  Excess  of  pigment  is  found,  chiefly  in  the 
palisade  cells.  In  the  cutis  the  collagenous  fibers  are  thickened,  the 
elastic  fibers  and  fat  tend  to  disappear,  and  the  lymph-channels  are 
obliterated.  Secondary  inflammation  is  not  uncommon.  In  the  so- 
called  ichthyotic  eczema,  there  is  more  reaction  on  the  part  of  the  rete, 
as  shown  by  proliferation  and  hyperplasia,  with  reappearance  of  the 
granular  layer.  A  moister  process  with  the  heaping  up  of  crusts  is 
produced.  When  the  hyperplasia  affects  the  papillae  as  well,  a  granular 
or  nodular  surface,  and  even  actual  warts,  may  be  the  result  (ichthyosis 
hystrix).  One  form  is  associated  with  elephantiasis. 

Scleroderma. — Scleroderma  is  an  affection  of  doubtful  etiology.  It  is 
most  common  in  females  and  in  those  of  a  neurotic  disposition. 

1  Romberg,  Klin.  Wahr.  u.  Beobach,  Berlin,  1851 :  228;  Erasmus  Wilson,  Jour, 
of  Cut.  Med.,  1:1868:355. 


ELEPHANTIASIS  953 

Anatomically,  it  may  be  diffuse  and  widespread,  or  give  rise  to  local 
lesions  of  a  peculiar  clinical  type,  known  as  morphoea.  The  diffuse 
form,  less  often  the  circumscribed,  occasionally  comes  on  after  exposure 
to  cold,  erysipelas,  and  acute  rheumatism.  In  some  cases  of  morphcea 
it  is  possible  that  some  disorder  of  the  nerve  centres  is  at  work,  but 
in  the  majority  of  cases  the  nature  of  the  scleroderma  is  quite  obscure. 
In  a  few  cases  the  thyroid  gland  has  been  found  to  be  diminished  in 
size  (Singer  and  Beer).  The  disease  is  met  with  on  the  trunk,  face, 
or  extremities,  and  is  characterized  by  a  peculiar  brawny  induration 
of  the  skin  and  subcutaneous  tissues,  which  feel  much  as  if  they  were 
frozen.  The  disease  may  come  on  quickly  and  extend  rapidly,  it  may 
remain  stationary,  or,  again,  may  finally  retrograde. 

The  histological  changes  are  confined  almost  exclusively  to  the  corium 
and  underlying  parts.  At  most  there  may  be  slight  pigmentation  in 
some  cases  in  the  deeper  parts  of  the  rete.  The  vessels  are  thickened, 
the  lymph-channels  are  narrowed,  and  there  is  hypertrophy  of  the 
elastic  and  fibrous  tissue.  There  is  a  certain  amount  of  cellular  infiltra- 
tion. The  newly-formed  connective  tissue  finally  undergoes  cicatricial 
contraction  and  atrophy  of  the  various  glands  results. 

A  peculiar  form  occurs  in  children,  usually  in  those  of  low  vitality, 
known  as  sclerema  neonatorum.  It  is  congenital  or  begins  in  the  earlier 
months  of  life,  and  usually  affects  the  legs  and  feet.  According  to 
Langer  it  is  due  to  the  solidification  of  the  subcutaneous  fat,  produced 
by  the  lowering  of  the  temperature. 

Elephantiasis. — Elephantiasis  (elephantiasis  Arabum,  pachydermia 
acquisita)  is  a  condition  characterized  by  the  most  extreme  hyper- 
plastic  thickening  of  the  skin  and  subcutaneous  tissues.  The  disease 
is  endemic  in  certain  tropical  and  subtropical  countries,  as  Arabia, 
Egypt,  Central  America,  and  Brazil,  but  sporadic  cases  are  occasion- 
ally met  with  in  the  temperate  climes.  Some  cases  begin  with  the 
clinical  manifestations  of  a  local  inflammatory  process,  as,  for  instance, 
erysipelas  and  lymphangitis,  together  with  fever.  Repeated  attacks 
add  to  the  thickening  of  the  tissues,  which  finally  becomes  extreme. 
Others,  again,  are  more  sluggish  and  insidious. 

The  etiology  is  not  altogether  clear.  Many  cases,  although  not  all,  are 
due  to  the  presence  of  the  Filaria  sanguinis  hominis  and  its  embryos. 
These  accumulate  in  the  lymphatic  channels,  usually  of  the  lower 
extremities,  scrotum,  or  abdomen,  where  they  cause  stasis  of  the  lymph, 
with  consecutive  inflammation  and  fibrous  hyperplasia.  Obstruction  of 
the  lymphatics  from  other  causes,  such  as  the  pressure  of  inflammatory 
exudates  or  tumors,  removal  or  destructive  disease  of  the  lymphatic 
glands,  predispose  to  the  condition,  if  they  do  not  actually  cause  it. 
Sporadic  cases  are  not  infrequently  attributable  to  passive  congestion, 
or  to  chronic  and  relapsing  inflammations,  such  as  erysipelas,  eczema, 
tuberculosis  of  the  skin  or  bones,  varicose  ulcers,  prurigo,  and  syphilitic 
periostitis.  Those  cases  which  develop  insidiously,  without  inflammatory 
manifestations,  are  of  more  doubtful  nature.  Some  are  supposed  to  be 
due  to  inherited  peculiarities  or  intra-uterine  pathological  conditions. 


954 


THE  SKltf 


In  acquired  elephantiasis  the  tissues  involved  present  more  or  less 
thickening,  resulting  in  some  cases  in  enormous  enlargement  of  the  part, 


FIG.  253 


Elephantiasis  of  the  leg.     Enormous  enlargement  of  the  limb,  with  ichthyosis. 
(From  the  Pathological  Museum,  McGill  University.) 


with  obliteration  of  the  normal  contour.  The  skin  and  subcutaneous 
tissues  are  thickened,  firm,  and  indurated.  They  may  be  hard  (elephan- 
tiasis dura),  or  soft  and  grayish-white  (E.  mollis).  In  some  cases  the 


CONDYLOMA  955 

lymphatics  are  dilated  and  on  section  abundant  serous  fluid  exudes 
(E.  lymphangiectatica).  The  skin  surface  is  smooth  (E.  glabrd),  warty 
(E.verrucosa),  nodular  (E.  tuberosa),  or  papillomatous  (E.  papillomatosa). 
In  some  cases  the  horny  layer  is  thickened,  forming  scales  or  plates 
(acquired  ichthyosis,  keratosis). 

Microscopically,  in  the  severer  forms,  the  connective  tissue  of  the 
cutis  is  hyperplastic,  with  some  atrophy  of  the  fat.  The  bloodvessels 
are  dilated  and  thickened,  but  not  invariably  so,  and  there  is  often 
perivascular  leukocytic  infiltration.  In  the  tropical  variety  the  lym- 
phatics are  dilated,  their  walls  thickened,  and  the  subcutaneous  tissues 
are  cedematous.  The  epidermis  presents  varying  degrees  of  keratosis. 

Besides  the  diffuse  form,  local  tumors  of  similar  appearance  are 
described  as  occurring  on  the  scrotum,  prepuce,  vulva,  and,  rarely,  on 
the  breast. 

Keratosis  Pilaris. — Keratosis  pilaris  (lichen  pilaris)  is  characterized 
by  the  formation  of  small  papules  about  the  hair-follicles.  The  process 
may  be  simple  (keratosis  pilaris  alba),  or  complicated  by  inflammation 
(keratosis  pilaris  rubra).  Various  grades  and  modifications  of  the  disease 
exist.  In  well-marked  cases  there  is  hyperkeratosis  in  and  about  the 
follicles,  which  become  occluded  by  horny  plugs  at  their  orifices.  The 
simple  variety  is  usually  found  on  the  extensor  surfaces  of  the  limbs, 
while  the  inflammatory  form  is  apt  to  involve  the  flexor  surfaces  as 
well. 

Keratosis  Follicularis. — Keratosis  follicularis,  or  Darier's  disease,  is 
a  peculiar  affection  of  the  skin,  first  described  by  Darier1  under  the 
name  "  psorospermose  folliculaire,"  since  he  thought  that  certain  cell- 
inclusions  in  the  rete  were  of  the  nature  of  psorosperms.  These  are 
now  generally  thought  to  be  peculiar  forms  of  cell-degeneration.  The 
disease  usually  begins  in  childhood.  The  etiology  is  unknown.  The 
lesion  consists  in  a  primary  keratosis  and  parakeratosis  of  the  sebaceous 
follicles  and  hair-bulbs.  The  process  apparently  begins  in  the  mouths 
of  the  follicles  and,  later,  extends  to  the  interfollicular  tissues.  The 
mouths  of  the  follicles  are  dilated  and  occluded  by  imperfectly  cornified 
cells.  It  is  believed,  also,  that  the  lesions  may  at  times  originate  in  the 
epidermis  and  about  the  orifices  of  the  sweat  ducts.  In  the  rete  are 
to  be  seen  certain  rounded  bodies  that  closely  resemble  psorosperms, 
and  at  the  bottom  of  the  follicle-plugs  are  compressed  homogeneous 
masses,  which  Darier  called  "grains."  Fissures  or  lacunae  are  observed 
between  the  cells  of  the  rete.  The  rete  generally  shows  marked  pro- 
liferation, and  may  extend  deeply  into  the  corium.  The  stratum  granu- 
losum  is  absent.  There  is  slight  cellular  infiltration  in  the  corium, 
and  a  deposit  of  pigment  at  the  periphery  of  the  lesion. 

Condyloma. — Local  outgrowths,  resembling  papillomas,  are  not  infre- 
quently found  in  situations  where  the  skin  is  subjected  to  chronic 
irritation,  as,  for  instance,  the  presence  of  inflammatory  processes,  dis- 
charges, dirt,  and  friction.  Warmth  and  moisture  predispose  to  their 

1  Internal.  Atlas  selt.  Hautkrankh.,  8. 


956 


THE  SKIN 


formation.  We  find,  therefore,  that  they  are  most  commonly  present 
about  the  external  genitals  and  near  the  anus,  where  they  form  what  are 
usually  known  as  condylomata  acuminata.  Gonorrhoea,  venereal  ulcers, 
and  decomposing  secretions  play  the  most  important  part.  They  begin 
as  small  papillary  excrescences,  and  may  increase  until  extensive  warty 
or  cauliflower-like  growths,  of  firm  consistence  and  whitish  color,  are 
produced.  The  process  is  essentially  an  overgrowth  of  the  papillae, 
which,  increase  in  length  and  usually  become  branched.  Histologically 
the  outgrowths  consist  of  vascular  connective  tissue,  containing  collec- 
tions of  round  cells  here  and  there.  The  lymphatics  are  frequently 
packed  with  inflammatory  round  cells,  which  are  also  numerous  in 
the  immediate  neighborhood.  The  epithelium  over  the  hyperplastic 
papilla?  is  markedly  increased  in  thickness. 


FIG.  254 


The  various  grades  of  warts  and  cutaneous  papillomas.     (Perls.) 

Warts. — Warts  (verrucse)  are  of  various  kinds,  hard,  soft,  or  papil- 
lomatous.  The  common  wart  is  found  usually  upon  the  hands.  It 
is  often  multiple,  a  fact  suggesting  a  possible  contagious  nature.  The 
cause  is  unknown,  but  inasmuch  as  warts  frequently  disappear  after 
persisting  for  some  time,  some  local  irritation  is  probably  at  work. 
Warts  form  small  hemispherical  elevations  upon  the  surface  of  the  skin. 

Microscopically  there  is   an  excessive  development  of  the  papillae, 


FIBROMA 


957 


mainly  those  in  the  centre  of  the  nodule.  The  central  vessels  are 
dilated,  and  the  overlying  epithelium  is  hyperplastic. 

Tumors. — Fibromas. — Fibromas  are  not  infrequently  found  in  the 
skin.  They  are  often  multiple  and  may  attain  considerable  size.  The 
hard  fibroma  (fibroma  durum)  is  much  rarer  than  the  soft  form  (fibroma 
molle) . 

Microscopically,  it  is  composed  of  interlacing  bands  of  dense  fibrous 
tissue,  with  relatively  few  nuclei,  and  but  few  elastic  fibrils  and  blood- 
vessels. 

The  soft  fibroma  (fibroma  molle,  fibroma  molluscum,  molluscum  simplex, 
molluscum  pendulum)  is  composed  of  a  loose  meshwork  of  connective 
tissue,  often  cedematous  or  myxomatous.  The  new-growth  begins  in 

FIG.  255 


Soft  fibroma.     Winckel  obj.  No.  6,  without  ocular.     (From  Dr.  A.  G.  Nicholls'  collection.) 

the  subcutaneous  tissues  and  corium,  and  projects  secondarily  into  the 
epidermis.  The  papillae  are  flattened  from  pressure,  and  the  epidermis 
thus  forms  a  uniform  thin  layer  over  the  tumor.  Possibly  of  the  same 
nature  is  the  soft  wart  or  acrochordon. 

According  to  v.  Recklinghausen,1  the  soft  fibromas  arise  from  the 
fibrous  sheaths  of  the  subcutaneous  nerve  filaments,  which  proliferate 
and  form  connective-tissue  growths  of  rather  cellular  type.  He  con- 
sequently terms  them  neuro fibromata.  These  tumors  may  be  restricted 
to  the  area  supplied  by  a  particular  nerve,  or  may  be  disseminated. 
In  the  local  form  a  large  soft  tumor  is  produced  (molluscum  elephant- 

1  Ueber  die  multiplen  Fibrome  der  Haut.,  Berlin,  1882. 


958 


THE  SKIN 


iasticum).  In  other  cases  a  complicated  meshwork  of  connective 
tissue  arises  from  the  endoneurium  (plexiform  neuroma;  "Rankenneu- 
rom,"  q.  v.~).  These  in  the  course  of  their  development  sometimes 
lead  to  diffuse  fibrous  thickening  of  the  skin  and  subcutaneous  tissues 
(elephantiasis  neuromatosa).  This  form  is  often  congenital  and  develop- 
mental in  nature  and  gives  rise  to  curious  lobulated  folds  of  the  skin, 
recalling  the  appearances  in  certain  pachydermata.  In  the  case  of 
dermatolysis  or  cutis  laxa,  found  in  the  so-called  "India-rubber"  men, 
the  connective  tissue  has  been  found  to  be  transformed  into  myxoma- 
tous  material,  while  the  elastic  fibres  were  normal. 

Diffuse  fibromatosis  of  the  skin  (elephantiasis  fibrosa)  is  met  with 
as  well  as  an  abnormal  overgrowth  of  the  fat,  leading  to  thickening 
of  the  skin  (elephantiasis  lipomatosa). 

Gerhardt,  Baerensprung,  and  Simon  have  described  a  multiple  pig- 
mented  papilloma  or  nevus,  which  is  supposed  to  develop  in  connection 
with  the  nerve  trunks  (neuropathic  papilloma,  nervennevus,  ncevus  unius 
lateris).  Little  is  known  about  it. 

FIG.  256 


Keloid. 


A  peculiar  form  of  fibroma  is  the  keloid  (cheloid),  which  forms 
flattened,  round,  or  irregular  masses,  plaques,  and  elevated  streaks 
over  which  the  skin  is  smoothly  stretched.  In  general  it  may  be  said 
that  the  new-growth  resembles  scar  tissue.  The  disease  is  not  common, 
but  is  said  to  be  more  frequent  in  the  negro  race.  It  is  usual  to 
divide  the  cases  into  true  or  spontaneous  and  false  or  cicatricial  keloid 


KELOID  959 

Both  varieties  are  probably  of  the  same  nature,  although  it  is  not 
always  possible  to  get  evidence  of  a  previously  existing  scar.  Keloid 
usually  follows  traumatism,  as  a  scar,  a  vesicle,  a  pustule,  a  burn,  or 
even  a  blow.  Occasionally,  no  etiological  factor  can  be  made  out. 
The  growth  is  apt  to  be  multiple,  and  while  not  malignant,  in  the  usual 
sense,  commonly  recurs  after  removal,  owing  probably  to  a  persistent 
tendency  toward  overgrowth  manifested  in  the  new  scar. 


FIG.  257 


Keloid  in  a  negroj     (Howard  Fox's  case.) 

Histologically,  keloid  in  the  earlier  stages  is  made  up  of  numerous 
spindle  cells,  but  later  of  dense  fibrous  tissue  fibrillse,  which  in  general 
are  arranged  parallel  to  the  long  axis  of  the  tumor.  The  adventitia 
of  the  vessels,  even  beyond  the  limits  of  the  growth,  shows  signs  of 
proliferation,  and  it  is  probable  that  this  is  the  primary  change.  The 
corium  to  some  extent  becomes  involved  in  the  growth. 

Neuroma. — Whether  a  true  neuroma  occurs  in  the  skin  is  doubted 
by  some  observers.  Duhring,  however,  has  described  a  case  in  which 
were  found  newly-formed  non-medullated  nerve  fibers  in  a  connective- 
tissue  matrix,  together  with  elastic  fibrils.  The  vessels  were  thickened 
and  surrounded  by  lymphoid  cells. 

Papilloma. — Papillomas  are  not  infrequent. 


960  THE  SKIN 

Lipoma. — Lipomas,    or   fatty   tumors,    are    of   frequent    occurrence, 
and  arise  from  the  subcutaneous  tissue.     They  form  round,  flattened, 


FIG.  258 


Extensive  papilloma  of  the  foot.     (From  the  Pathological  Museum,  McGill  University.) 

FIG.  259 


Pendulous  lipoma  of  the  shoulder.      (From  the  collection  of  the  Montreal  General  Hospital.) 


OF    THE 

UNIVERSITY 

^ 


XANTHELASMA  901 


and  tabulated  growths,  often  multiple  and  symmetrical.  They  occur 
commonly  on  the  arms  and  about  the  shoulders.  The  exact  cause 
is  not  understood.  Some  are  congenital,  and  in  some  there  appears 
to  be  an  inherited  peculiarity.  They  may  attain  a  considerable  size. 
Rarely,  they  become  inflamed  and  necrose,  giving  rise  to  foul  ulcers. 


FIG.  260 


Lipoma  from  the  subcutaneous  tissues  of  the  gluteal  region,  showing  the  tabulated  structure. 
(From  the  Pathological  Museum  of  McGill  University.) 

Xanthoma  or  Xanthelasma. — Xanthoma  or  xanthelasma  is  a  peculiar 
pigmented  tumor  found  on  the  eyelids  and  other  parts  of  the  body, 
but  occasionally  in  the  internal  organs,  such  as  the  trachea,  pericardium, 
the  capsules  of  the  liver  and  spleen. 

Histologically,  it  is  a  new-growth  of  connective  tissue,  between  the 
fibers  of  which  are  to  be  seen  cells  of  epithelioid  type,  which  vary  con- 
siderably in  size  and  are  multi nucleated,  so  that  they  resemble  the 
giant  cells  in  sarcomas.  These  cells  are  grouped  about  the  vessels  and 
are  often  markedly  infiltrated  with  fat.  They  may  be  regarded  as  im- 
mature fat  cells.  The  growth  forms  tabulated  masses  in  the  deeper 
layers  of  the  corium,  which  occasionally  reach  as  high  as  the  rete.  The 
epidermis  may  be  slightly  thinned.  The  characteristic  yellow  color  of 
the  tumor  is  due  to  a  pigment  called  lipochrome,  found  chiefly  in  the 
fat-containing  cells  or  "xanthoma  bodies,"  but  also  in  the  cells  of  the 
rete  and  corium. 

Xanthomas  form  yellowish  growths  in  the  skin  (xanthoma  planum) 
or  projecting  above  it  (xanthoma  tuberosum),  and  are  commonly  multiple. 
The  etiology  is  obscure.  Cases  are  occasionally  met  with  in  diabetics 
and  in  those  suffering  from  jaundice,  but  are  more  usually  spontaneous,. 


962 


THE  SKIN 


Some  observers  attribute  the  growth  to  a  preceding  inflammation, 
while  others  (Ziegler)  regard  it  as  a  lipomatous  lymphangioma  or 
endothelioma.  Pollitzer1  believes  that  the  xanthomas  found  on  the 
eyelids  are  different  from  those  occurring  in  other  places,  and  are  not 
really  tumors,  but  are  due  to  fatty  degeneration  of  the  fibers  of  the 
prbicularis  muscle  with  proliferation  of  the  nuclei. 

Myoma. — Myomas  occurring  superficially  in  the  skin  are  rare.  They 
form  growths  varying  in  size  from  that  of  a  pin-head  to  that  of  a  walnut. 
They  are  often  multiple,  and  arise  either  from  the  arrectores  pilorum 
or  the  walls  of  the  arterioles.  The  tumors  are  composed  of  interlacing 
smooth-muscle  fibers,  with  a  few  elastic  fibrillse,  and  are  sometimes 


FIG.  261 


Hemangioma  of  the  skin.    The  large  blood  sinuses  are  well  shown.    Zeiss  obj.  DD,  without  ocular. 
(From  the  collection  of  Dr.  A.  G.  Nicholls.) 


definitely  encapsulated.  They  are  generally  found  in  the  skin  of  the 
face  and  arms.  They  are  apt  to  recur.  Herzog2  has  collected  about  a 
dozen  cases  from  the  literature  and  reports  one  of  his  own.  In  some 
instances  the  vessels  are  dilated  (angiomyoma). 

Myxomas,  chondromas,  and  osteomas  are  rare  in  the  skin.  The 
myxomas  are  more  properly  myxosarcomas,  and  are  found  usually  on 
the  external  female  genitalia. 

Angioma. — No  hard  and  fast  line  can  be  drawn  between  certain 
birth-marks  composed  of  dilated  or  newly-formed  vessels  (ncevi  vas- 

1  Trans.  Amer.  Derm.  Assoc.,  1897. 

2  Jour.  Cutan.  and  Genito-urin.  Dis.,  16  :  1898  :  527. 


LYMPHANGIOMA 


963 


culosi)  and  true  tumors.  The  congenital  forms  are  commonly  found 
on  the  face  and  neck,  but  occasionally  also  on  the  extremities,  and  are 
sometimes  related  to  the  branchial  clefts  (fissural  angiomas).  All 
grades  exist  from  a  simple  dilatation  of  thin-walled  vessels  resembling 
capillaries,  and  generally  restricted  to  a  particular  arterial  district 
(teleangiectasis,  angioma  simplex),  or  a  congeries  of  dilated  anastomosing 
vessels,  leading  to  some  enlargement  of  the  part  (cavernoma,  angioma 
cavernosum),  to  a  tumor-like  outgrowth  (angioma  hypertrophicum).  In 
the  last-mentioned  form,  soft  warts  are  produced  containing  thick- 
walled  vessels  and  often  solid  cell-masses.  They  are  pale  or  purplish- 
red  in  color,  and  are  covered  with  smooth  skin.  They  are  commonly 
multiple.  Pigmented  nevi  are  not  infrequently  of  this  type. 

FIG.  262 


Section  from  a  case  of  hemangioma  simplex,  exhibiting  progressive  enlargement  and  extension. 

(Borrmann.) 

Secondary  changes  are  common  in  angiomas.  Thus,  the  intervening 
fibrous  stroma  may  be  noticeably  increased,  and  the  epidermis  or  horny 
layer  may  be  thickened  Qcerato-angiomd). 

Lymphangioma. — Lymphangioma  is,  anatomically  speaking,  strictly 
comparable  to  hemangioma  and  occurs  in  the  form  of  simple  local 
dilatations  of  the  lymph- vessels,  elephantiasis,  or  elevated  nodules. 
Lymphangiomas  are  found  in  almost  any  part  of  the  body  and  may 
arise  either  in  the  skin  or  in  the  subcutaneous  tissues.  The  cause  is 
unknown,  but  from  the  fact  that  many  cases  arise  in  infancy  it  may 


964  THE  SKIN 

be  inferred  that  they  are  often  due  to  some  developmental  anomaly 
of  the  lymphatics.  Certain  cases  of  lymphangiectasis  in  the  genitalia 
or  lower  extremities  are  often  associated  with  proliferation  of  fibrous 
tissue,  and  are  properly  to  be  regarded,  in  the  majority  of  instances  at 
least,  as  due  to  inflammation  and  obstruction  of  the  lymph-channels 
rather  than  as  true  tumors.  In  all  forms  there  is  dilatation  of  the 
lymphatics,  and,  in  the  lymphangioma  proper,  a  new-formation  of 
lymph-vessels.  Frequently  with  this  is  associated  round-celled  infiltra- 
tion about  the  vessels  with  connective-tissue  hyperplasia.  There  may 
also  be  keratosis  of  the  superficial  epidermis.  As  a  rule,  the  blood- 
vessels show  dilatation  and  overgrowth  as  well. 

Some  of  the  warts  of  the  skin  are  pure  lymphangiomas,  while  others 
are  composed  of  solid  masses  of  cells  in  the  corium,  and  have  been 
called  hypertrophic  lymphangiomas,  or  endotheliomas.  Certain  pig- 
mented  nevi  are  also  to  be  included  in  this  group. 

Xeroderma  Pigmentosum. — Under  the  name  xeroderma  pigmentosum 
(atrophoderma  pigmentosum,  melanosis  lenticularis  progressiva,  Pick), 
Kaposi  has  described  a  rare  affection  of  the  skin,  beginning  a  few  months 
after  birth.  It  is  at  first  manifested  by  the  repeated  efflorescence  of 
reddish  spots,  which,  later,  disappear  with  some  scaling  of  the  skin. 
As  the  spots  fade,  pigmented  areas,  not  unlike  freckles,  are  left,  and 
the  vessels  dilate.  The  skin  becomes  smooth  and  atrophic,  and  later, 
warty  outgrowths  are  produced,  which  have  a  striking  tendency  to 
develop  into  carcinoma. 

Multiple  Benign  Cystic  Epithelioma. — Multiple  benign  cystic  epithe- 
lioma  is  a  rather  rare  disorder  of  the  skin,  which  has  led  to  consider- 
able difference  of  opinion.  It  was  first  described  by  Jacquet  and 
Darier1  under  the  name  "hydradenome  eruptif,"  and  somewhat  later 
by  Brooke,2  who  termed  it  "epithelioma  adenoides  cysticum,"  and 
by  Fordyce.3  The  lesion  consists  in  the  formation  of  multiple  small 
papules,  nodules,  or  tubercles  in  the  skin,  usually  on  the  face,  eyelids, 
forehead,  trunk,  or  arms.  In  some  cases  superficial  erosion  takes 
place,  so  that  something  like  "  rodent"  ulcer  is  the  result.  In  a  few 
instances  the  lesions  are  restricted  to  a  small  district,  but,  as  a  rule, 
numerous  widely  disseminated  growths  are  observed.  The  disease 
usually  manifests  itself  in  the  first  two  decades  but  has  been  found  also 
in  advanced  life. 

Histologically,  the  tumors  are  composed  of  irregular,  oval,  or  elongated 
masses  and  strands  of  epithelial  cells,  resembling  those  of  the  deeper 
layers  of  the  epidermis.  In  some  cases  the  epithelial  cells  form  a  uni- 
form diffuse  growth,  while  in  others  there  is  an  intricate  interlacing 
and  anastomosis  of  the  various  bands.  Occasionally  the  growth  presents 
an  alveolar  arrangement.  "Cell-nests,"  identical  with  those  met  with 
in  malignant  epithelioma,  are  often  seen.  Many  of  these  have  under- 

1  Ann.  de  dermat.  et  de  syph.,  8:  1887. 

2  Brit.  Jour,  of  Derm.,  September,   1892. 

8  Jour.  Cutan.  and  Genito-urin.  Dis.,  10  :  1892  :  459  and  501. 


MOLLUSCUM  CONTAGIOSUM 


965 


gone  central  necrosis  and  liquefaction,  forming  globular  cysts.  It  is 
probable  that  the  appearances  described  under  such  a  variety  of  names 
are  due  to  a  number  of  growths  differing  in  their  nature.  Some  have 
their  starting  point  in  the  deeper  layers  of  the  epidermis  and  the  outer 
parts  of  the  hair-sheaths — true  benign  cystic  epithelioma;  others,  again, 
begin  in  the  sebaceous  glands — cystadenoma  sebaceum;  others,  again, 
in  a  proliferation  of  the  endothelium  of  the  bloodvessels — hemangio- 
endothelioma;  while  still  others,  according  to  Kaposi  and  his  school, 
are  due  to  a  new-formation  and  dilatation  of  lymph- vessels  in  the  skin — 
lymphangioma  tuberosum  multiplex.  Some  cases,  like  the  one  reported 
by  Pick,1  are  of  mixed  type,  combining  the  peculiarities  of  the  first 
and  second  forms. 


Molluscum  contagiosum.     Zeiss  obj.  DD,  without  ocular.     (From  the  collection  of 
Dr.  Oskar  Klotz.) 


Molluscum  Contagiosum. — Molluscum  contagiosum  (epithelioma  con- 
tagiosum, molluscum  epitheliale,  endocystic  condyloma — "Sebum- 
wartze"),  like  the  last-mentioned,  is  a  disease  that  has  given  rise  to 
much  difference  of  opinion.  It  appears  in  the  form  of  elevated  nodules 
about  the  size  of  a  pea  or  bean,  which  have  a  peculiar  shiny  and  wax- 
like  appearance  and  present  a  small  central  depression.  The  affection 
is  found  most  commonly  in  children  who  live  under  unhygienic  con- 
ditions. It  occurs  sometimes  in  small  epidemics  and  is  believed  by 

1  Arch.  f.  Derm.  u.  Syphilis,  58:  1902.  For  literature,  see  Hartzell,  Benign  Cystic 
Epithelioma,  Proc.  Path.  Soc.,  Philadelphia,  October,  1902. 


966  THE  SKIN 

many  (Virchow,  Liveing,  Bellinger,  Klebs,  Stellwagon)  to  be  contagious, 
although  this  is  denied  by  others  (Erasmus  Wilson,  Rokitansky,  Hebra, 
Kaposi,  G.  H.  Fox). 

In  general  terms,  the  tumor  may  be  said  to  consist  of  a  series  of  radi- 
ally arranged  masses  of  epithelial  cells,  separated  from  each  other  by 
fibrous  septa  and  converging  toward  a  common  centre.  The  centre 
of  the  new-formation  is  broken  down  and  a  soft  tallowy  substance  can 
be  expressed.  According  to  White  and  Robey,1  the  most  recent  investi- 
gators, the  new-growth  begins  in  the  rete.  The  lowest  layers  of  cells 
resemble  the  normal  prickle-cells,  with  the  addition  that  many  of  them 
contain  one  or  more  nucleoli.  Some  of  the  cells  have  lost  their  nucleus 
and  are  composed  of  a  fine  fibrillary  protoplasm.  The  cells  of  the 
layers  above  become  more  or  less  distorted,  are  often  devoid  of  nuclei, 
and  contain  clear  rounded  spaces  which  give  the  characteristic  appear- 
ance to  the  structure  (molluscum  bodies).  Toward  the  upper  regions 
of  the  growth  the  cells  become  more  or  less  keratinized.  The  signifi- 
cance of  the  changes  has  been  interpreted  variously.  Some  think 
that  the  peculiar  vacuolated  appearance  is  due  to  amyloid  infiltration 
of  the  cells,  while  others  attribute  it  to  the  presence  of  protozoa. 
Repeated  attempts  have  been  made  to  cultivate  the  organism,  but  with- 
out success  so  that  the  majority  of  observers  are  now  agreed  that  the 
so-called  "molluscum  bodies"  are  examples  of  cell-degeneration  and  not 
parasitic.  The  older  views  of  Engel,  Rokitansky,  and  Hebra,  that 
the  growth  originates  in  the  sebaceous  follicles,  and  that  of  Virchow, 
that  it  arises  from  the  hair-follicles,  have  now  practically  been  given 
up,  and  it  is  believed  to  be  more  probable  that  the  new-growth  is  due 
to  a  peculiar  and  characteristic  transformation  of  the  rete  cells  into 
keratin. 

Sarcoma. — Sarcomas  of  the  skin  are  relatively  rare,  and  may  be 
conveniently  divided  into  pigmented  and  non-pigmented  forms.  They 
are  primary  or  secondary. 

Melanotic  sarcoma  (melanoma,  chromatophoroma)  may  arise,  though 
rarely,  from  apparently  normal  skin.  In  about  half  the  cases,  how- 
ever, it  is  secondary  to  sarcoma  of  the  uvea.  The  majority  of  the  remain- 
ing 50  per  cent,  originate  in  pigmented  nevi  or  warts.  The  tumor 
is  recognized  by  its  color,  which  is  brownish  or  brownish-black,  either 
diffuse  or  patchy  in  distribution.  The  primary  tumor  may  be  single 
and  remain  latent  for  years.  It  may  then  form  local  metastases  rapidly. 
This  form  of  new-growth  is  met  with  also  in  horses,  and  curiously 
enough  only  in  white  horses,  in  the  dark  skin  around  the  anus. 

Melanomas  are  tumors  of  connective-tissue  appearance,  originating 
in  the  proliferation  of  certain  cells  which  are  identical  with  the  pigment- 
bearing  cells  (chromatophores)  of  the  normal  skin.  The  newly-formed 
chromatophores  do  not  always  reach  adult  development,  for  many 
are  found  to  possess  shorter  processes  or  are  actually  rounded.  The 
young  cells,  again,  are  often  devoid  of  pigment.  The  growth  com- 

1  Molluscum  Contagiosum,  Jour.  Med.  Research,  (N.  S.  2) :  1902  :  225. 


SARCOMA 


967 


FIG.  264 


monly  begins  in  the  deeper  layers  of  the  skin  and  gradually  insinuates 
itself  through  the  epidermis,  so  that  small  warts,  rarely  larger  than  a 
pea,  are  produced  which  eventually  form  a  fungating  mass.  Unna, 
Gilchrist,  and  others  regard  these  tumors  as  originating  in  the  epithe- 
lium, and  hold,  therefore,  that  they  are  epitheliomatous  rather  than  sar- 
comatous.  The  most  recent  investigations,  however,  do  not  appear 
to  support  this  view,  for  as  Ribbert1  points  out,  melanotic  growths 
frequently  start  in  the  choroid  of  the  eye,  where  the  chromatophores 
are  undoubtedly  of  connective-tissue  origin. 

Morphologically,  the  melanomas  are  spindle-celled  or  alveolar  sar- 
comas. The  latter  is  the  common  form  of  the  primary  melanomas 
of  the  skin.  They  are  often  very 
vascular  and  may  contain  extrava- 
sations of  blood. 

The  pigment — melanin — is  found 
in  fine  particles  or  irregular  clumps 
within  and  about  the  cells.  Its  exact 
composition  is  not  known,  possibly 
varying  according  to  the  nature 
and  position  of  the  growth,  but  it 
is  peculiar  in  that  it  contains  sul- 
phur. Melanomas  are  very  malig- 
nant, frequently  recurring  after 
removal,  and  forming  metastases 
rapidly  in  the  various  viscera.  The 
secondary  growths  may  at  times  be 
devoid  of  pigment. 

Under  the  term  idiopathic  multiple 
pigment  sarcoma,  Kaposi  described 
a  new-growth,  which  is  pigmented 
and  highly  vascular,  owing  to  the 
presence  of  numerous  thin-walled 
capillaries.  It  is  not  now  believed 
to  be  a  true  pigmented  sarcoma,  for 
the  color  is  due  to  the  vascularity 
and  the  deposition  of  altered  blood 
pigment.  The  growth  may  last  for 
years,  and  finally  becomes  malig- 
nant, although  spontaneous  resolu- 
tion sometimes  takes  place.  The 
exact  nature  of  this  tumor  is  not  known.  Some  hold  that  it  belongs 
to  the  infectious  granulomas. 

Non-plgmented  sarcomas  also  occur.  They  are  primary  and  second- 
ary, single  or  multiple,  and  form  nodular  or  papillomatous  masses 
projecting  above  the  skin.  Histologically,  they  are  round-,  spindle-, 
or  mixed-celled. 


Multiple  pigmented  sarcomas  in  the  skin. 
(From  the  collection  of  Dr.  F.  J.  Shepherd.) 


1  Lehrbuch  der  speciellen  Path.,  1902:  784. 


968 


THE  SKIN 


Fibrosarcoma  and  angiosarcoma  are  sometimes  met  with.  Angioma 
serpiginosum  is  a  rare  affection,  which  appears  to  be  a  form  of  angio- 
sarcoma  dependent  on  some  congenital  anomaly  of  the  vessels.1 

Sarcoma-like  new-growths  are  found  in  the  skin  in  certain  cases  of 
leukemia  and  pseudoleukemia. 

Diffuse  sarcomatosis  of  the  skin  (Kaposi),  also  called  mycosis  fun- 
goides,  occurs  under  the  form  of  a  round-celled  sarcoma,  which  produces 
flattened,  knotty  masses  of  new-growth  in  the  skin.  It  extends  slowly 
until  considerable  areas  are  involved.  The  cells  composing  it  resemble 
young  connective-tissue  cells  and  form  diffuse  masses,  or  are  separated 

FIG.  265 


Melanotic  sarcoma.     Winckel  obj.  No.  6,  without  ocular.     (From  Dr.  A.  G.  Nicholls 

collection.) 

into  clumps  by  fibrous  septa.  The  vessels  are  dilated  and  there  is 
03dema  of  the  cutis  and  rete.  This  is  the  stage  of  erythema  and  infiltra- 
tion. Later,  the  cells  are  more  numerous,  more  uniform  in  shape  and 
size,  and  tend  to  be  arranged  in  columns,  while  the  connective  tissue 
is  reduced  to  a  minimum.  Fungating  and  ulcerating  masses  are  ulti- 
mately produced.  The  tumor  finally  takes  on  malignant  action,  although 
occasionally  it  involutes  spontaneously.  It  is  not  yet  settled  whether 
the  new-growth  is  properly  to  be  regarded  as  a  sarcoma  or  whether 
it  is  inflammatory. 

Acanthosis    Nigricans. — Another   rare   condition   of   doubtful    nature 
is   acanthosis   nigricans,   described  by  Pollitzer,  Darier,   Morris,   and 

1  White,  Jour.  Cut.  and  Genito-urin.  Dis.,  12  :  1894  :  468. 


CARCINOMA  969 

others.  Deeply  pigmented,  warty,  and  papillomatous  nodules  are 
found  in  various  parts.  Histologically,  there  is  hyperplasia  of  the  papillae 
and  epidermis,  dilatation  of  the  blood-  and  lymph-vessels,  with  increase 
of  the  pigment  in  the  palisade  cells.  There  is  also  an  imperfect  attempt 
at  the  formation  of  " cell-nests."  The  disease  has  been  found  associated 
with  carcinoma  of  other  structures.  Darier  thinks  that  it  is  due  to  some 
lesion  of  the  sympathetic  nerve. 

Carcinoma. — Carcinoma  is  of  frequent  occurrence  in  the  skin.  It 
is  found  on  the  lip,  nose,  eyelids,  prepuce,  scrotum,  and  vulva,  but 
may  occasionally  develop  in  other  regions.  It  is  in  this  form  of  carci- 
noma that  long-continued  irritation  appears  to  play  an  important  role. 
Thus,  it  is  met  with  on  the  lower  lip  in  smokers,  at  the  margins  of  indolent 
ulcers,  in  patches  of  chronic  dermatitis  caused  by  soot,  tar,  paraffin, 
and  irritating  discharges,  and  about  warts  or  nevi,  which  have  been 
frequently  rubbed  or  excoriated. 

FIG.  266 


Epithelioma  of  the  lip.     (Hyde.) 

Carcinomas  may  develop  from  the  epidermis  or  from  the  epithelium 
of  the  glands  and  hair-follicles,  but  it  is  not  always  possible  to  determine 
the  point  of  origin  in  fairly  advanced  cases.  Certain  of  them,  not 
connected  with  the  epidermal  layer,  may  originate  in  cell-inclusions, 
derived  from  the  epidermis  (dermoids),  or  from  the  branchial  clefts 
(branchiogenic  carcinoma). 

Thiersch  has  divided  skin  carcinomas  into  superficial  and  deep  forms, 
which,  however,  merge  imperceptibly  one  into  the  other. 

Among  the  superficial  forms  may  be  mentioned  rodent  ulcer  and 
Paget's  disease  of  the  nipple.  These  form  shallow  ulcers  with  infiltrated, 


970  THE  SKIN 

slightly  elevated  edges,  which  from  necrosis  may  assume  a  somewhat 
nodular  appearance. 

Rodent  ulcer  occurs  most  frequently  at  the  corner  of  the  nose,  near 
the  eyelids,  or  upon  the  forehead.  The  growth  is  excessively  slow  in 
its  development,  sometimes  lasting  many  years.  Under  surgical  meas- 
ures it  may  heal  up  for  a  time,  but  sooner  or  later,  as  a  rule,  breaks 
out  again.  The  tumor  is  quite  superficial  and  spreads  slowly  at  its 
margin,  but,  owing  to  the  relatively  large  amount  of  necrosis,  presents 
the  clinical  picture  of  an  ulcer  rather  than  a  neoplasm.  Histologically, 
it  is  an  epithelioma. 

Paget's  disease  begins  at  the  nipple  and  apparently  originates  in  a 
very  chronic  form  of  inflammation.  A  shallow,  reddened  ulcer  is 
produced,  which  slowly  extends  its  borders.  When  well-developed, 
the  microscopic  appearances  are  those  of  a  squamous-celled  epithe- 
lioma. The  tumor  is  further  interesting,  inasmuch  as  several  observers 
have  thought  that  certain  peculiar  appearances  within  the  cells  are 
parasites.  It  is,  however,  more  likely  that  these  are  forms  of  cell 
degeneration.  Occasionally  as  ulceration  progresses,  healing  takes 
place  toward  the  centre  with  the  formation  of  a  scar  (cicatrizing  epithe- 
lioma). 

The  deeply-penetrating  carcinomas  originate  in  nodules  of  new- 
growth,  which,  when  necrosis  has  taken  place,  present  the  appearance 
of  ulcers  with  nodular  bases  and  irregular  edges.  When  growth  is 
active,  fungating  papillomatous  masses  are  produced.  This  form  is  very 
malignant,  growing  rapidly,  and  producing  metastases  more  quickly 
than  the  superficial  varieties.  All  sorts  of  intermediate  grades  exist 
between  the  superficial  and  the  deeply-penetrating  carcinomas,  and 
epithelial  proliferation,  connective-tissue  overgrowth,  and  necrosis  may 
be  combined  in  a  variety  of  ways. 

The  microscopic  appearances  of  epithelioma  of  the  skin  vary  accord- 
ing to  circumstances.  In  a  well-marked  example  of  a  not  too  rapidly 
growing  epithelioma,  the  rete  sends  out  downgrowths  into  the  under- 
lying tissues  in  the  form  of  processes  which  divide,  subdivide,  and 
anastomose.  The  outermost  cells  of  these  downgrowths  correspond 
in  structure  with  the  lowermost  cell  of  the  normal  rete.  As  they 
approach  the  central  portion  they  become  more  cuboidal,  being  practi- 
cally identical  with  the  prickle-cells,  while  at  the  centre  they  are  more 
or  less  flattened  and  cornified.  In  many  cases  the  cornified  areas 
present  the  appearance  of  flattened,  concentrically  arranged  spindles 
or  plates,  which  may  be  compared  to  the  stratification  of  an  onion, 
the  so-called  "cell-nests"  (Perlkugeln).  The  central  portion  occa- 
sionally becomes  fatty,  colloid,  granular,  or  in  rare  instances  calcified. 
The  connective  tissue  enveloping  the  growth  is  normal,  or,  as  in  most 
cases,  thickened,  and  shows  infiltration  with  small  round  cells,  either 
the  result  of  cell-proliferation,  or,  possibly,  inflammation.  In  the  more 
rapidly  growing  varieties  the  cells  are  of  the  more  primitive  type 
and  present  little  or  no  tendency  to  cornification,  so  that  cell-nests  are 
lacking.  In  cases  where  pressure  has  been  exerted  upon  the  processes, 


ALOPECIA  971 

the  cells  become  flattened  and  spindle-shaped,  resembling  somewhat 
those  of  a  sarcoma. 

Certain  rare  forms  of  carcinoma  deserve  a  passing  notice.  One 
of  these  is  the  so-called  "lenticular"  carcinoma.  It  is  usually  found 
in  the  mamma,  where  it  is  secondary  to  a  deeper  seated  carcinoma  of 
the  gland,  but  is  occasionally  met  with  as  a  primary  growth  in  other 
parts  of  the  body.  The  affected  skin  and  subcutaneous  tissues  are 
diffusely  infiltrated  with  new-growth,  and  are  swollen,  hard,  and  resist- 
ant, resembling  washleather.  On  section  the  structure  is  tough  and 
gristly,  and  may  contain  considerable  fluid,  due  to  pressure  upon  the 
lymphatics.  Thus  a  form  of  elephantiasis,  "cancer  en  cuirasse," 
is  the  result. 

Alveolar  carcinoma  may  be  found  in  the  deeper  layers  of  the  skin 
and  subcutaneous  tissues. 

Apart  from  the  local  metastases  that  form  in  the  skin  about  an 
epithelioma,  secondary  carcinoma  is  not  common.  Both  connective 
tissue  and  epithelial  tumors  may  form  metastases  in  the  skin.  Carcinoma 
of  the  breast  is  perhaps  the  most  frequent  cause. 


THE  ACCESSORY  STRUCTURES  AND  APPENDAGES  OF  THE  SKIN. 

The  Hair. 

The  congenital  anomalies  of  the  hair,  hypertrichosis,  alopecia  adnata, 
canities,  have  already  been  referred  to  (see  p.  909). 

Alopecia. — The  acquired  form  of  alopecia,  or  falling  of  the  hair 
(alopecia  acquisita,  clavities  acquisita),  may  be  a  senile  manifestation 
(alopecia  senilis),  usually  associated  with  atrophy  of  the  skin  and  absorp- 
tion of  the  subcutaneous  fat,  or  may  occur  early  in  life  (alopecia  pre- 
matura). 

Alopecia  Prematura. — Alopecia  prematura  may  come  on  without 
any  obvious  cause  (idiopathic),  but  is  often  associated  with  disease  of 
the  scalp  or  of  the  general  system.  Thus,  eczema,  erysipelas,  syphi- 
litic eruptions,  seborrhoea,  favus,  and  ring-worm  are  common  causes 
of  loss  of  hair.  The  hair  also  not  infrequently  falls  out  after  acute 
and  other  infective  diseases,  such  as  typhoid,  scarlatina,  syphilis,  and 
tuberculosis,  and  during  lactation  in  weakly  individuals. 

Alopecia  Areata. — Alopecia  areata  is  characterized  by  the  formation 
of  one  or  more  areas  of  baldness,  which  may  be  found  on  any  region  of 
the  body  normally  covered  with  hair.  The  scalp  and  the  beard  are 
the  parts  usually  involved.  The  patches  are  round  or  oval,"  sharply 
defined,  and  become  quite  devoid  of  hair.  The  skin  of  the  affected 
part  is  soft,  smooth,  depressed,  at  first  slightly  reddened,  later  white 
and  glistening.  The  hair  at  the  periphery  shows  no  obvious  change, 
but  gradually  falls  out  in  turn.  The  sharp  line  of  demarcation  between 
the  round  bald  spot  and  the  normally  covered  scalp  is  a  characteristic 
feature.  The  spots  may  gradually  increase  until  the  hair  of  the  scalp, 


972'  THE  NAILS 

beard,  and  eyebrows,  and  even  of  the  whole  of  the  body,  has  entirely 
disappeared. 

Microscopically,  the  hairs  and  hair-follicles  are  atrophied,  there  is 
diapedesis  of  lymphocytes  with  accumulation  of  mast-cells,  and  absence 
of  pigment  in  the  rete. 

The  exact  cause  is  not  altogether  understood.  Perhaps  the  majority 
of  observers  consider  the  disease  to  be  a  trophoneurosis,  inasmuch  as 
it  in  many  cases  follows  injury  to  the  nerves,  shock,  or  other  disturb- 
ance of  the  nervous  system.  On  the  other  hand,  the  occasional  occur- 
rence of  the  disease  in  epidemic  form,  together  with  some  evidence  of 
contagion,  suggests  a  parasitic  cause  in  some,  at  least,  of  the  cases. 
Sabouraud  regards  as  the  specific  cause  a  bacillus,  which  is  present 
in  the  hair-follicles,  and  identical  with  a  bacillus  which  he  finds  in 
seborrhoea.  Rarely  ringworm  gives  rise  to  similar  appearances,  and 
has  been  mistaken  for  alopecia  areata  by  competent  observers. 

Atrophy. — Atrophy  of  the  hair  is  due  to  disease  of  the  scalp,  such 
as  seborrhoea,  eczema,  and  psoriasis,  or  to  some  systemic  disorder  asso- 
ciated with  poverty  of  nutrition.  The  hair  is  dry,  brittle,  and  lacks 
lustre. 

Trichorrhexis  nodosa  is  a  rare  form  of  atrophy,  in  which  the  shafts 
of  the  hairs,  usually  of  the  beard,  present  nodular  swellings,  through 
which  the  hair  readily  breaks.  In  monilethrix,  an  affection  which 
is  generally  congenital,  the  shaft  of  the  hair  presents  ring-like  con- 
strictions through  which  the  hair  is  apt  to  fracture. 

Hypertrichosis. — Hypertrichosis,  apart  from  the  congenital  and 
developmental  forms,  occasionally  results  in  areas  that  have  been  sub- 
jected to  irritation,  pressure,  inflammation,  or  other  cause  which  leads 
to  congestion.  In  women,  hair  upon  the  upper  lip,  or  other  parts  of 
the  face,  is  occasionally  due  to  an  hereditary  peculiarity  or  to  some 
abnormality  of  the  sexual  functions.  It  is  not  uncommon  after  the 
menopause. 

Ganities. — Canities,  or  blanching  of  the  hair,  occurs  physiologic- 
ally in  old  age,  but  is  not  infrequently  met  with  in  younger  people. 
It  is  sometimes  an  inherited  peculiarity,  or  may  follow  mental  over- 
work, nervous  shock,  or  prolonged  disease.  Local  blanching  has  been 
noticed  in  migraine  and  neuralgia.  The  condition  is  due  not  only 
to  loss  of  pigment,  but  to  the  presence  of  air  in  the  substance  of  the 
hair. 

The  Nails. 

Congenital  absence  and  developmental  anomalies  of  the  nails  are  rare 
and  usually  associated  with  defects  of  the  hair  and  teeth. 

White  spots  upon  the  nails  (leukopathia  unguis)  are  not  infrequent, 
due  to  the  presence  of  air  between  the  layers  of  the  keratin.  They 
are  the  result  of  slight  injuries  or  of  impairment  of  nutrition.  Trans- 
verse furrows  are  often  found  upon  the  nails  of  those  who  have  suffered 
from  acute  disease  or  other  cause  which  has  lowered  their  vitality. 


BROMIDROSIS  973 

The  nail  may  be  dislocated  from  its  bed  by  traumatism  or  the  col- 
lection of  pus  or  blood  beneath  it.  This  may  lead  to  complete  exfolia- 
tion. The  nails  may  also  be  shed  in  alopecia  areata,  syphilis,  diabetes 
mellitus,  and  hysteria. 

Inflammation  of  the  matrix  of  the  nail  (onychia)  and  of  the  surround- 
ing soft  parts  (paronychia)  may  be  the  result  of  traumatism  or  local 
infection,  or,  again,  may  be  occasionally  seen  in  syphilis  and  tuberculosis. 
Favus  and  ring-worm  sometimes  attack  the  nail  (onychomycosis)  and 
give  rise  to  onychia  and  paronychia. 

In  atrophy  the  nail  is  thinned  and  softened,  becomes  brittle,  and  is 
often  traversed  by  ridges  or  furrows. 

Simple  overgrowth  of  the  nails,  which  may  properly  be  regarded  as 
a  physiological  condition,  since  it  is  due  to  the  omission  of  the  customary 
cutting  of  the  nails,  is  met  with  among  certain  races,  such  as  the  Chinese, 
and  among  some  of  the  fakirs  of  India.  In  the  forms  of  overgrowth 
found  in  other  nations,  however,  there  is  evidence  of  disease  as  well. 
The  nail  substance  is  thickened  (hyperonychia),  irregular,  roughened, 
brittle,  and  discolored.  The  nails  may  in  time  become  twisted,  bent, 
and  project  like  talons  beyond  the  finger-tips  (onychogryphosis).  It  is 
not  uncommon  to  find  inflammation  and  even  suppuration  going  on 
in  these  cases  beneath  the  nail.  The  condition  is  met  with  in  old 
persons  who  are  dirty,  debilitated,  and  uncared  for,  in  syphilis,  eczema, 
psoriasis,  and,  occasionally,  in  tuberculosis,  leprosy,  myxcedema,  acro- 
megaly,  neuritis,  and  neurotrophic  disorders.  A  common  form  of 
simple  enlargement  is  the  so-called  "ingrowing  toe-nail,"  in  which  the 
nail  increases  in  breadth  and  penetrates  the  flesh  of  the  toe,  giving  rise 
to  inflammation  and  suppuration  (paronychia). 


The  Sweat-glands. 

Hyperidrosis  and  Anidrosis. — These  are  functional  disorders  of  the 
sweat-glands,  and  are  either  an  evidence  of  some  inherited  peculiarity 
or  the  result  of  systemic  or  local  disease.  No  gross  changes  are  found 
in  the  glands,  but  it  is  probable  that  in  most  cases  the  conditions  are 
due  to  some  disturbance  of  the  innervation  of  the  part,  or  to  the  direct 
action  of  a  toxin  upon  the  secreting  cells  in  the  process  of  elimination. 
Complete  absence  of  sweat  secretion  (anidrosis)  probably  does  not 
occur,  except  locally  in  cases  where  the  glands  have  been  destroyed  by 
disease.  Diminution  in  the  amount  of  sweat  (hyphidrosis)  is  found  in 
many  acute  fevers,  in  myxcedema,  and  in  such  conditions  as  eczema, 
psoriasis,  and  pityriasis.  Hyperidrosis  is  most  common  in  the  palms, 
soles,  axillary  and  pubic  regions.  Unilateral  or  local  hyperidrosis  is  met 
with  in  some  nervous  diseases  and  in  neurotic  individuals. 

Bromidrosis. — Bromidrosis,  or  offensive  perspiration,  is  usually 
associated  with  hyperidrosis,  and  generally  affects  the  same  parts. 
There  is  a  certain  amount  of  odor  connected  with  ordinary  perspiration, 
and  this  is  more  evident  in  some  individuals  and  races  than  in  others. 


974  THE  SWEAT-GLANDS 

In  stout  persons  or  those  who  perspire  freely  the  sweat  often  emits 
a  sour  smell  shortly  after  it  is  excreted.  In  true  bromidrosis,  the  odor 
is  penetrating  and  disgusting.  The  condition  may,  of  course,  be  assisted 
by  uncleanly  habits,  but  is  often  met  with  in  those  who  are  scrupulously 
clean.  In  such  we  have  to  look  for  the  cause  in  some  neuropathic 
disturbance.  The  foetid  odor  is  by  many  attributed  to  the  decomposition 
of  the  sweat  by  microorganisms. 

Uridrosis. — Uridrosis,  or  the  excretion  of  urinary  salts  through  the 
sweat,  is  met  with  occasionally  in  Bright's  disease,  cholera,  and  some 
serious  constitutional  disorders.  It  has  been  recorded  also  after  the 
administration  of  jaborandi.  In  marked  cases  scales  of  urea  may  be 
deposited  on  the  skin. 

Chromidrosis. — Chromidrosis,  or  colored  sweat,  is  an  excessively  rare 
condition.  It  is  possibly  in  some  cases  neurotrophic  in  nature.  More 
often,  coloration  of  the  sweat  is  due  to  the  elimination  through  the  skin 
of  such  substances  as  copper,  or  the  application  of  dyes  to  the  skin. 
One  form,  red  sweat,  which  affects  the  axillary  and  genital  regions,  is 
due  to  concretions  upon  the  shaft  of  the  hairs  (leptathrix),  resulting 
from  bacterial  activity. 

Hematidrosis. — Bloody  sweat  (hematidrosis)  is  a  rare  condition  due 
to  hemorrhage  into  the  sweat-glands.  It  has  occurred  in  neurotic 
individuals. 

Phosphorescent  Sweat. — Phosphorescent  sweat  has  been  described. 

Sudamina. — Sudamina  (miliaria  crystallina)  is  not  infrequently  met 
with  in  febrile  conditions,  especially  those  associated  with  profuse 
sweating.  Small,  clear  vesicles,  often  abundant,  form  in  the  horny 
layer  of  the  skin,  which  disappear  after  a  few  days.  The  condition 
is  due  either  to  blocking  of  the  duct  and  consequent  retention  of  the 
sweat,  or,  according  to  some,  rupture  of  the  duct  and  escape  of  the 
sweat  into  the  horny  layer. 

Hydrocystoma. — Hydrocystoma  is  a  rather  rare  condition,  usually 
affecting  the  face.  One  or  more  clear,  deep-seated  vesicles  are  formed, 
apparently  the  result  of  dilatation  of  the  sweat-ducts  in  their  course 
through  the  corium. 

Hydradenitis  Suppurativa. — Hydradenitis  suppurativa  is,  as  its  name 
implies,  a  suppurative  inflammation  of  the  sweat-glands.  It  is  most 
often  found  in  the  axillary  and  pubic  regions.  The  process  begins 
deep  in,  involves  the  surrounding  structures,  and  eventually  leads  to 
destruction  of  the  glands. 


The  Sebaceous  Glands. 

Seborrhoea. — The  sebaceous  glands  normally  secrete  an  oily 
substance,  which  is  elaborated  in  the  glandular  epithelium  and  dis- 
charged upon  the  surface  of  the  skin,  where  it  acts  as  a  sort  of  lubricant. 
Excess  of  this  secretion  gives  rise  to  the  condition  known  as  seborrhcea. 
In  one  form,  seborrhcea  sicca,  minute,  dry,  filmy  scales  are  produced, 


COMEDO  975 

composed  in  part  of  dried  sebum,  but  also  of  desquamated  horny  epi- 
thelium and  dirt  or  dust.  In  the  other,  there  is  excessive  secretion  of 
the  sebum,  so  that  the  skin  is  kept  constantly  oily  (seborrhcea  oleosa). 

Seborrhoea  may  be  a  local  or  a  general  condition.  The  former  is 
usually  met  with  on  the  scalp  or  in  the  genital  region.  Generalized 
seborrhoea  is  rather  rare,  and  is  most  frequently  found  in  young  children 
where  the  greasy  secretion  which  normally  covers  the  skin  during  intra- 
uterine  life  (smegma,  vernix  caseosa)  persists  for  some  time  after  birth. 
It  is  not  uncommon  to  find  in  infants  dirty  crusts  upon  the  scalp,  com- 
posed of  sebum,  dirt,  epithelial  scales,  and  hairs.  In  mild  cases  of 
seborrhoea  sicca  there  is  only  a  trifling  exfoliation  of  the  epithelial 
scales  (dandruff},  with  slight  increase  of  secretion,  but  in  others  there  may 
be  considerable  heaping  up  of  material  into  larger  scales  and  crusts 
(pityriasis  furfuracea  capillitii).  Seborrhoea,  if  long-continued,  leads 
to  atrophy  of  the  hair-follicles  and  loss  of  the  hair.  It  is  not  infre- 
quently, also,  accompanied  by  inflammatory  phenomena  (seborrhoeal 
eczema,  seborrhoeal  dermatitis). 

The  cause  is  obscure,  since  many  cases  occur  in  apparently  healthy 
people,  but  some  are  connected  with  digestive  disturbances  or  poor 
nutrition.  Anything  which  tends  to  keep  the  scalp  warm  and  moist 
would  predispose  to  the  affection. 

Asteatosis. — Asteatosis,  absence  or  lack  of  the  sebaceous  secretion 
(xerodermia) ,  is  rare  as  a  primary  disease,  but  is  usually  an  accom- 
paniment of  other  disease,  notably  where  the  skin  is  dry  and  scaly. 
Thus,  it  is  found  in  psoriasis,  prurigo,  pityriasis  rubra  pilaris,  leprosy, 
and  ichthyosis.  The  skin  becomes  dry,  fissured,  and  the  epithelium 
scales  off. 

There  are  several  allied  conditions  that  are  due  to  the  accumulation 
of  the  sebum  within  the  sebaceous  glands  or  their  ducts.  This  accu- 
mulation and  retention  of  secretion  is  brought  about  by  obstruction 
to  the  ducts  from  dirt  or  overgrowth  of  the  lining  epithelium,  or,  in 
some  cases,  by  alteration  in  the  character  of  the  secretion  itself. 

Comedo. — Comedo  is  a  condition  in  which  the  excretory  duct  of  a 
sebaceous  gland  and  sometimes  that  of  a  hair-follicle  are  obstructed. 
This  results  in  the  formation  of  a  minute  nodule  in  the  skin,  which 
presents  externally  as  a  blackish  point,  the  size  of  a  pinhead  or  less. 
On  squeezing  this,  a  fine  worm-like  or  thread-like  substance  is  extruded, 
having  a  blackish  or  brownish  spot  at  the  end.  This  substance  is  com- 
posed of  inspissated  sebum,  fattily  degenerated  and  keratinized  epithe- 
lial cells,  and  not  infrequently  lanugo  hairs.  The  dark  spot  at  the 
end  of  the  plug  is  largely  composed  of  dirt  and  dust,  and,  according 
to  Unna,  a  particular  pigment — ultramarine. 

The  anatomical  condition  appears  to  be  overgrowth  of  the  epithelial 
cells  lining  the  duct,  with,  possibly,  parakeratosis  of  the  external  cells,  in 
this  way  leading  to  obstruction  to  the  free  discharge  of  the  sebum,  which 
consequently  becomes  altered  in  character.  It  is  also  possible  that  the 
irritation  of  fine  hairs  or  inspissation  of  the  secretion  may  be  the  exciting 
cause  of  the  cellular  proliferation.  Not  infrequently,  the  obstructed 


976  THE  SEBACEOUS  GLANDS 

glands  become  inflamed,  forming  small  red,  elevated  papules  and 
pustules  (one  form  of  acne).  Comedones  are  found  usually  upon  the 
face  near  the  nose,  on  the  forehead,  and  occasionally  upon  the  shoulders. 
They  may  be  very  numerous.  The  affection  has  been  regarded  as 
having  some  connection  with  gastro-intestinal  disturbances,  but  is  often 
enough  met  with  in  healthy  people.  It  is,  however,  most  common 
about  the  age  of  puberty,  when  the  skin  and  its  associated  glands  are 
particularly  active. 

Milium. — Milium  is  a  form  of  obstruction  of  the  sebaceous  glands 
in  which  the  lumina  become  somewhat  dilated,  forming  small  nodules 
the  size  of  a  pin-head  or  smaller,  of  a  whitish  or  yellowish  color,  which 
project  slightly  above  the  general  surface  of  the  skin.  On  incising 
one  of  these,  a  smooth  or  warty  lobulated  mass  can  be  expressed  con- 
sisting of  fat  and  epidermal  cells  surrounded  by  concentric  layers  of 
keratinized  cells.  Milia  are  found  usually  upon  the  eyelids.  Where  a 
group  of  sebaceous  glands  are  enlarged  and-  distended  with  secretion 
and  proliferated  cells  the  condition  is  known  as  acrochordon.  This 
is  met  with  most  frequently  in  elderly  people,  usually  on  the  eyelids, 
throat,  and  neck. 

Inflammations.— Acne  Vulgaris. — Acne  vulgaris  is  an  inflammation 
of  the  sebaceous  glands,  which  is  liable  to  involve  the  hair-follicles 
and  surrounding  tissues  as  well.  Not  infrequently  the  trouble  is  to  be 
traced  to  comedones.  In  the  early  stages  small,  elevated,  reddened 
papules  are  formed,  usually  upon  the  face,  but  occasionally  also  on  the 
neck,  shoulders,  trunk,  and  extremities  (acne  simplex).  Where  there  is 
a  central  blackish  point  the  condition  is  called  acne  punctata.  The 
process  often  goes  on  to  suppuration,  so  that  elevated,  reddish  pustules 
are  produced,  varying  in  size  from  that  of  a  pin-head,  or  smaller,  to  that 
of  a  pea,  presenting  a  yellowish  centre  (acne  pustulosa).  On  incising 
this  a  small  drop  of  pus  can  be  evacuated.  In  the  so-called  acne 
indurata  comparatively  large  purplish-red  and  hard  nodules  are  pro- 
duced which  are  much  indurated  and  persist  for  a  considerable  time. 
They  leave  considerable  staining  of  the  skin  and  more  or  less  scarring. 
Occasionally  acne  spots  present  central  necrosis  and  resemble  variola 
pocks.  Acne  is  most  common  in  persons  about  the  age  of  puberty  or 
in  early  adult  life.  Those  who  have  poor  circulation  or  who  are  the 
subjects  of  gastro-intestinal  disorders  are  supposed  to  be  more  liable. 
Acne  may  also  be  caused  by  external  irritation. 

A  mycotic  folliculitis  and  perifolliculitis,  involving  the  hair-follicles 
and  sebaceous  glands,  due  to  the  trichophyton,  is  well  known  (tinea 
sycosis)  (see  p.  929). 

Cysts. — Atheroma,  or  sebaceous  cyst,  is  a  cystic  dilatation  of  a 
sebaceous  gland  or  hair-follicle,  due  to  the  accumulation  of  secretion 
within  its  cavity.  There  is  usually  a  smooth,  connective-tissue  capsule, 
lined  by  stratified  pavement  cells.  The  contents  are  liquid,  semi- 
solid,  or  cheesy,  and  are  composed  of  fat,  epithelial  debris,  cholesterin, 
and  fine  hairs.  The  cysts  may  be  all  sizes  up  to  that  of  a  fist.  They 
are  usually  found  upon  the  scalp,  but  occasionally  also  in  the  skin  of 


TUMORS  977 

the  neck  and  face,  of  the  trunk  and  extremities.  The  contents  of 
sebaceous  cysts  may  undergo  extensive  horny  transformation  or  even 
calcification,  so  that  bone-like  nodules  result. 

Tumors. — Somewhat  similar  cysts,  but  more  deeply  seated,  present  a 
different  structure,  being  lined  by  modified  skin,  and  therefore  are  of 
the  nature  of  dermoids.  Others,  again,  are  more  or  less  completely 
filled  with  papillary  outgrowths,  so  that  they  may  be  properly  regarded 
as  papillary  cystadenomas.  Occasionally  they  become  malignant. 

Adenoma  of  the  sebaceous  glands  is  a  rather  rare  affection,  met  with 
usually  in  persons  mentally  defective.  Small,  round  or  oval,  sessile 
tumors  are  found  usually  on  the  chin,  forehead,  or  about  the  nose. 

Carcinoma  may  develop  from  the  sweat-glands. 


SECTION   IX. 
THE  MUSCULAR  SYSTEM. 


CHAPTEE  XLIL 

THE  SKELETAL  MUSCLES. 
THE  MUSCLES. 

THE  skeletal  muscles  belong  to  the  voluntary  group,  and  are  there- 
fore of  the  striated  order.  They  are  composed  of  fibers  averaging 
about  4  to  5  cm.  long  and  from  10  to  55  /*  broad.  On  finer  analysis, 
these  fibers  consist  of  a  soft  protoplasmic  ground-substance,  presenting 
a  distinct  transverse  striation  and  a  fainter  longitudinal  fibrillation, 
inclosed  in  a  clear,  structureless,  and  elastic  sheath  (the  sarcolemma). 
The  nuclei  of  the  muscle-fibers  are  situated  upon  the  surface  of  the 
muscle-cylinder,  are  ellipsoidal  in  shape,  with  their  long  axes  parallel 
to  that  of  the  fiber.  The  number  of  the  nuclei  varies  considerably, 
being  more  numerous  in  the  young  and  smaller  muscles.  Increase  in 
size  of  the  fiber  is  not  necessarily  associated  with  numerical  increase  of 
the  nuclei. 

The  muscle-fibers  are  grouped  into  fasciculae,  and  these  again  into 
larger  bundles,  which  together  constitute  the  complete  anatomical 
muscle.  The  various  components  are  held  together  and  supported 
by  a  connective-tissue  stroma,  that  inclosing  the  larger  bundles  being 
called  the  perimysium,  while  the  delicate  interstitial  substance  envelop- 
ing the  individual  fibers  is  termed  the  endomysium. 

The  muscles  are  highly  vascular,  the  larger  vessels  being  found  in 
the  perimysium,  and  these  break  up  into  a  freely  anastomosing  network 
of  capillaries  within  the  endomysium.  The  terminations  of  the  nerves 
supplying  them  are  highly  specialized  arborizations  or  end  plates. 

Under  normal  conditions,  the  length  and  breadth  of  the  muscles 
vary  within  wide  limits,  according  to  the  degree  of  relaxation  or  contrac- 
tion incident  to  their  functioning  for  the  time  being.  When  at  rest, 
the  muscles  are  held  in  a  condition  short  of  complete  relaxation,  called 
tonus,  which  may  be  defined  in  a  sense  as  passive  or  inherent  contrac- 
tion. Under  pathological  conditions  the  degree  of  contraction  and 
of  tonus  may  be  greatly  altered. 


980  THE  MUSCLES 

Muscles  are  liable  to  a  great  variety  of  disease  processes.  Owing 
to  their  exposed  position  and  their  close  association  with  the  skeleton 
in  regard  to  the  function  of  locomotion,  they  are  particularly  liable  to 
direct  and  indirect  traumatism.  Again,  owing  to  their  abundant  blood 
supply  they  are  readily  brought  under  the  influence  of  various  circulating 
toxins  and  infective  microorganisms.  And,  further,  they  may  present 
grave  changes  as  a  result  of  defective  innervation. 

The  pathological  lesions  are  manifested  not  only  by  qualitative 
changes  in  the  fibers  themselves,  but  also  quantitatively  by  an  increase 
or  diminution  of  the  muscle-substance,  either  absolutely  or  relatively  to 
the  amount  of  the  connective-tissue  stroma. 


CONGENITAL  ANOMALIES. 

These  are  so  numerous  that  it  is  impossible  to  enter  adequately  into 
the  subject  here.  Many  of  the  anomalies  are  interesting,  not  only 
from  the  point  of  view  of  development,  but  also  because  they  have  a 
practical  bearing  in  regard  to  surgery  and  medicine. 

In  general  terms  it  may  be  said  that  there  is  no  anomaly  of  the 
muscles  in  man  which  does  not  have  its  prototype  in  one  or  other  of 
the  lower  animals.  Briefly,  these  consist  in  irregular  origin  and  insertion 
of  the  muscles;  complete  or  partial  defect  of  certain  muscles  or  groups 
of  muscles;  reduplication;  while  again,  certain  muscles  may  be  present 
in  man  which,  though  normally  present  in  other  species,  are  not  regu- 
larly found  in  the  human  subject.1  One  of  the  most  important  of  these 
peculiarities  about  which  a  word  or  two  may  be  spoken  is  defect  of  the 
diaphragm,  which  sometimes  gives  rise  to  serious  clinical  manifestations. 
The  diaphragm  may  be  defective  to  an  extent  varying  from  a  small 
opening  to  one  involving  half  the  structure.  The  deficiency  is  usually 
on  the  left  side  and  in  the  muscular  portion  somewhat  posteriorly.  The 
condition  is  often  associated  with  other  grave  developmental  errors,  such 
as  anencephaly,  hemicrania,  and  anomalies  of  the  fingers  and  toes.  In 
many  such  cases  prolonged  continuation  of  life  is  of  course  impossible, 
but  where  the  diaphragm  alone  is  involved  persons  so  affected  have 
been  known  to  reach  a  fairly  advanced  age.  Owing  to  the  deficiency  in 
this  structure,  it  is  usual  at  some  time  or  other,  to  find  certain  of  the 
abdominal  viscera,  such  as  the  stomach,  omentum,  intestines,  liver, 
spleen,  and  kidney  in  the  thoracic  cavity.  In  such  cases  there  is 
usually  marked  dyspnoea  and  embarrassment  of  the  heart's  action,  with 
physical  signs  of  displacement  of  the  heart  and  lungs,  and  the  presence 
of  a  solid  organ  or  hollow  viscus  containing  air  in  the  chest  cavity  (see 
p.  404). 

1  Those  desiring  more  detailed  information  are  referred  to  Dr.  F.  J.  Shepherd's 
article  on  The  Anomalies  of  Muscles,  in  the  Reference  Handbook  of  the  Medical 
Sciences,  6:  1903:  42,  second  edition.  Wm.  Wood  &  Co.,  New  York. 


HEMORRHAGE  981 


CIRCULATORY  DISTURBANCES. 

Owing  to  the  free  anastomosis  of  the  vessels,  local  disorders  of  the 
circulation  are  not  readily  brought  about.  Any  such  changes  are  caused 
only  by  extensive  disease  of  the  bloodvessels  or  systemic  affections  of 
the  blood  and  circulatory  apparatus.  Except  in  the  case  of  the  grosser 
lesions,  it  is  difficult  to  recognize  circulatory  disturbances  post  mortem. 

Anemia. — Anemia  may  be  local  or  general.  The  local .  form  is 
brought  about  by  obstruction  of  a  main  arterial  trunk  or  compression 
of  the  muscle.  Toxic  substances  acting  upon  the  blood,  various  blood 
diseases,  and  weak  driving  power  of  the  heart  lead  to  general  anemia. 
Muscles  so  affected  are  pale,  soft,  dryer  than  normal,  and  deficient  in 
coloring  matter,  although  in  some  cases  this  is  increased. 

Hyperemia. — Active  hyperemia  is  found  in  the  neighborhood  of 
inflammatory  processes.  Passive  hyperemia  is  present  in  all  conditions 
of  general  vascular  stasis. 

(Edema. — Microscopically,  here,  the  muscle-fibers  are  vacuolated, 
owing  to  hydropic  infiltration,  while  the  connective  tissue  appears  to 
be  looser  than  normal.  In  the  severer  forms  the  fibers  degenerate  or 
even  liquefy. 

Hemorrhage. — Hemorrhage  into  the  muscles  is  not  infrequent.  It 
may  be  extensive,  minute  or  petechial.  The  larger  extravasations 
(hematoma)  are  the  result  of  trauma;  rupture  of  the  muscle  from 
excessive  contraction,  as,  for  example,  in  tetanus;  increased  blood 
pressure,  and  certain  degenerative  changes  in  the  vessel  walls.  Petechice 
are  most  probably  due  to  defective  nutrition  of  the  walls  of  the  capil- 
laries and  smaller  bloodvessels,  leading  to  fatty  changes  which  pre- 
dispose to  rupture,  or,  as  some  think,  to  diapedesis.  These  small 
hemorrhages  are  met  with  in  certain  of  the  infectious  fevers,  the  hemor- 
rhagic  diatheses,  pernicious  anemia  and  leukemia,  poisoning  from 
phosphorus  and  various  drugs,  and  multiple  sarcomatosis.1  When 
the  extravasation  is  extensive  the  muscle-fibers  are  pushed  apart  and 
compressed,  and  eventually  undergo  coagulation  necrosis  or  even 
disintegrate.  Should  the  process  go  on  to  healing,  the  blood  is  absorbed, 
or  in  part  absorbed  and  in  part  organized,  with  the  formation  of  a  pig- 
mented  scar  in  which  there  is  an  imperfect  attempt  at  restoration  of 
the  fibers.  In  rare  instances,  as  in  traumatic  myositis  ossificans,  the 
connective  tissue  may  undergo  metaplasia  into  cartilage  and  bone. 

Infarction. — Infarction  is  occasionally  brought  about  by  obstruc- 
tion to  the  blood  supply,  as,  for  instance,  in  arteriosclerosis,  endar- 
teritis,  local  compression,  weak  circulation,  thrombosis,  and  embolism. 
For  the  reason  mentioned,  the  condition  is  not  common.  It  is  most 
likely  to  be  met  with  in  those  weakened  by  prolonged  disease.  When 

1  For  a  critical  examination  of  the  forms  of  hemorrhage  here  referred  to,  see 
Nicholls  and  Learmonth,  The  Hemorrhagic  Diathesis  in  Typhoid  Fever,  and  its 
Relationships  to  Purpuric  Conditions  in  General,  Lancet,  London,  1 :  1901 :  305. 


982  THE  MUSCLES 

extensive,  large  areas  of  muscle  may  present  what  is  known  as  Zenker's 
necrosis,  but  more  commonly  multiple  minute  hemorrhages  into  the 
connective  tissue  are  produced.  The  larger  areas  of  anemic  necrosis 
are  often  bounded  by  a  zone  of  secondary  hemorrhagic  exudation. 
Should  such  an  area  become  infected,  abscess  may  result.  Certain 
cases  of  bedsores  and  senile  gangrene  are  of  the  nature  of  infarcts.  Total 
or  partial  ischemic  necrosis  of  the  psoas,  usually  the  left,  has  been 
noted  in  patients  who  for  a  long  time  have  kept  the  recumbent  position. 


INFLAMMATIONS. 

Myositis. — Myositis,  or  inflammation  of  muscle,  usually  arises 
by  the  direct  extension  of  an  inflammatory  process  from  some  adjacent 
part,  or  from  trauma.  Affections  of  the  bones,  joints,  skin,  and  mucous 
surfaces  play  an  important  role,  as  do  also  pleurisy,  peritonitis,  peri- 
and  paranephritis. 

In  other  cases,  myositis  is  hematogenous,  the  result  of  bacterial 
invasion  or  circulating  toxins.  This  is  met  with  in  septic  infection  of 
wounds,  puerperal  septicemia,  osteomyelitis,  malignant  endocarditis, 
acute  rheumatism,  typhoid  fever,  and  glanders. 

In  general  terms  it  may  be  stated  that  the  inflammation  largely 
affects  the  connective  tissue  and  bloodvessels,  while  the  changes  in  the 
muscle-fibers  are  mainly  degenerative  and  secondary. 

Acute  Myositis. — Acute  myositis  assumes  several  forms.  The  simplest 
type  is  characterized  by  a  slight  exudation  of  inflammatory  products 
into  the  perimysium  with  diapedesis  of  leukocytes.  The  muscle-fibers 
may  be  practically  normal  (acute  interstitial  myositis)  or  may  show 
various  grades  of  degeneration,  such  as  cloudy  swelling,  fatty  degenera- 
tion, and  coagulation  necrosis  (acute  diffuse  myositis).  The  condition 
is  met  with  in  typhoid  fever,  after  slight  trauma,  about  intramuscular 
hemorrhages,  in  the  neighborhood  of  local  inflammatory  foci,  and  is 
usually  a  temporary  condition  of  trifling  import.  The  process  may 
heal,  and,  provided  that  the  organization  of  the  muscle  be  not  destroyed, 
restitutio  ad  integrum  may  be  complete. 

In  more  severe  cases  the  muscle-fibers  may  to  some  extent  be 
destroyed  and  be  replaced  by  fibrous  tissue. 

In  still  more  severe  forms,  where  there  is,  for  instance,  infection  with 
pyogenic  microorganisms,  diffuse  suppuration  may  occur  in  the  muscle, 
or  abscesses  may  form  (acute  purulent  myositis).  This  is  seen  in  such 
conditions  as  infected  wounds,  erysipelas,  septic  arthritis,  ulcerative 
colitis,  septicemia,  and  glanders. 

In  the  early  stages  the  muscle  is  greatly  reddened  and  swollen,  gradually 
becoming  grayish,  grayish-yellow,  or,  if  hemorrhage  occur,  brownish  or 
grayish-green  in  appearance.  It  is  soft,  friable,  and  quickly  breaks 
down,  so  that  numerous  cavities  of  varying  size,  containing  pus  and 
shreddy  debris,  are  produced.  The  abscesses  may  remain  localized 
or  the  pus  may  burrow  widely  along  the  fascia  and  intermuscular  septa. 


MYOSITIS  983 

In  very  severe  infection,  or  where  the  resisting  power  of  the  tissues 
is  slight,  gangrene  may  occur,  the  muscle  turning  greenish-black  or 
black,  and  becoming  converted  into  a  dirty,  evil-smelling  mass,  which 
quickly  undergoes  liquefaction  and  disintegration.  In  the  neighbor- 
hood of  the  abscesses  the  muscle-fibers  are  found  to  be  in  various  stages 
of  degeneration,  and  where  there  is  a  tendency  to  heal,  there  is  a  zone 
of  granulation  tissue.  If  the  patient  survive,  small  abscesses  may 
disappear  after  the  pus  has  been  absorbed,  while  larger  ones  may  heal 
when  the  contents  have  been  discharged,  either  externally,  or  into  some 
cavity.  Others  become  encapsulated  by  active  granulation  and  the 
formation  of  connective  tissue.  In  time  the  abscess  contracts,  the 
contents  gradually  disappear,  become  inspissated,  or  infiltrated  with 
lime  salts.  When  large  portions  of  the  muscle  are  destroyed,  repair 
by  proliferation  of  the  muscle  cells  is  very  imperfect.  Where  the 
irritation  is  comparatively  slight,  though  continuous,  or  where  repeated 
attacks  of  acute  myositis  have  taken  place,  there  may  be  marked  prolifera- 
tion of  the  connective  tissue,  so  that  the  muscle  looks  as  if  traversed 
by  whitish  bands  or  membranes. 

A  curious  affection,  the  etiology  of  which  is  still  somewhat  obscure, 
is  the  so-called  primary  acute  polymyositis ,  described  by  Wagner,1 
Unverricht,  Hepp,  v.  Striimpell,2  and  Levy.  It  is  characterized  clini- 
cally by  fever,  pain,  and  swelling  of  the  muscles  of  the  tongue,  back, 
and  extremities.  Voluntary  movement  is  usually  completely  lost. 
There  is  generally  some  redness  of  the  skin,  with  the  production  of  rashes 
of  various  sorts,  so  that  the  disease  has  been  called  dermatomyositis. 
The  resemblance  to  trichinosis  is  close,  and  at  times  a  microscopic 
examination  is  necessary  to  make  the  diagnosis. 

Post  mortem,  the  muscles  are  brownish-red  in  color,  with  areas  of 
waxy  yellow  appearance,  and  present  punctate  and  linear  pigmenta- 
tion. The  fibers  are  separated  by  hemorrhagic  and  purulent  exudation. 
Marked  extravasation  of  blood  between  the  muscle  bundles  may  be  found. 

Microscopically,  one  finds  granular  degeneration  of  the  muscle-fibers, 
with  vacuolation,  loss  of  striation,  proliferation  of  the  muscle-nuclei,  and 
round-celled  infiltration  in  the  intramuscular  connective  tissue. 

The  disease  may  be  of  mild  intensity,  but  is  apt  to  become  chronic, 
and  usually  terminates  fatally  after  weeks  or  months.  It  is  almost 
certainly  of  an  infective  nature.  Senator  was  probably  the  first  to 
advance  the  view  that  polymyositis  is  in  the  main  due  to  auto-intoxica- 
tion. Since  the  affection  has  been  observed  after  the  use  of  improper 
food,  it  has  been  suggested  that  the  toxin  is  derived  from  the  gastro- 
intestinal tract.  Von  Striimpell  regards  it  as  due  to  bacterial  toxins 
circulating  in  the  blood,  derived  from  some  local  focus  of  infection. 

A  condition  of  great  interest  is  the  so-called  myositis  ossificans,  which, 
as  its  name  implies,  is  an  inflammation  of  the  muscles  accompanied 

1  Acute  Polymyositis,  Deutsches  Archiv  f.  klin.  Med.,  40: 1887:  241. 

2  Zur  Kenntniss  der  primaren  akuten  Polymyositis  Deutsch.  Zeitschr.  f.  Nerven- 
heilk.,  1:1891:479. 


984  THE  MUSCLES 

by  the  formation  of  bone.  The  disease  is  a  rare  one,  although  it  has 
been  known  since  1740,  when  the  first  case  was  reported  by  Freke  in 
the  Philosophical  Transactions.  Since  this  date  only  about  85  examples 
have  been  recorded.  The  etiology  is  still  quite  obscure.  Following 
Cahen,1  it  is  usual  to  divide  the  disease  into  two  forms,  a  localized  or 
stationary  and  a  progressive  form. 

In  the  first-mentioned  class  of  cases  the  disease  appears  to  be 
dependent  upon  trauma  or  irritation,  and  is  found  particularly  in 
muscles  which  are  overexercised  (exercise  bones).  The  muscles  affected 
are  usually  the  deltoids  and  pectorals  in  soldiers,  the  adductors  of  the 
thighs  in  riders,  occasionally  the  arms  in  gymnasts,  and  the  legs  in 
dancers.  The  bone  is  present  in  the  form  of  splinters,  plates,  or  bosses, 
either  attached  to  the  bones  and  tendons,  or  forming  movable  masses 
in  the  intramuscular  connective  tissue. 

More  obscure  still  is  myositis  ossificans  progressiva,  which  is  dis- 
tinguished from  the  first-mentioned  form  by  the  fact  that  it  begins 
in  early  life  and  successively  involves  one  muscle-group  after  another. 
The  disease  is  most  commonly  found  in  males,  according  to  Miinch- 
meyer,  9  out  of  12  cases,  and  Roth,  30  out  of  39.  The  affection  comes 
on  idiopathically  or  follows  slight  trauma.  After  a  somewhat  acute 
onset  with  local  swelling,  pain,  and  slight  febrile  reaction,  it  subsides 
in  intensity,  but  usually  advances  steadily  by  a  series  of  relapses.  The 
disease  generally  begins  in  the  muscles  or  fasciae  of  the  neck,  back,  and 
thorax,  gradually  spreading  to  other  parts  of  the  body.  It  invariably 
ends  fatally. 

In  the  first  stage  the  muscles  contain  areas  which  are  swollen,  painful, 
and  doughy,  due  apparently  to  inflammatory  infiltration  within  the 
intramuscular  connective  tissue.  In  the  next  stage  there  is  an  over- 
growth of  the  intramuscular  connective  tissue  with  leukocytic  infiltra- 
tion. The  muscle-fibers  lose  their  striation  and  show  fatty  or  other 
degeneration,  the  muscle-nuclei  are  increased  in  many  cases,  suggesting 
giant  cells,  and  the  fibers  finally  disintegrate.  In  the  third  stage, 
ossification  takes  place  in  the  affected  areas.  The  bone  is  found  in 
the  form  of  spicules,  plates,  nodules,  or  arborescent  masses  in  the  con- 
nective tissue  of  the  muscles,  the  fascia,  and  tendons.  In  some  cases 
the  muscle-bundles  are  chiefly  involved;  in  others,  the  tendons  and 
fasciae;  and  in  a  third  class  the  newly-formed  bone  is  so  associated  with 
the  old  that  the  disease  presents  the  picture  of  multiple  exostoses,  or, 
in  parts,  hyperostoses.  In  course  of  time  large  areas  of  muscle  are 
replaced  by  bone,  leading  to  marked  deformity  and  immobility  of  the 
joints.  Ultimately  the  patient  becomes  perfectly  helpless.  The  only 
muscles  which  escape  are  those  of  the  hand  and  the  muscles  which  are 
not  attached  to  bone  at  both  ends,  although  even  in  the  latter  case 
immunity  is  not  absolute.  The  "ossified  man"  seen  in  circuses  is 
usually  an  example  of  this  terrible  disease. 

The  newly-formed  bone  is  formed  directly  from  connective  or  granu- 

1  Ueber  Myositis  Ossificans,  Deut.  Zeit.  f .  Chir.,  31 :  1890 :  372. 


TUBERCULOSIS  985 

lation  tissue  after  the  fashion  of  periosteal  bone-formation,  or  indirectly 
from  newly-formed  cartilage  cells.  The  muscle-bundles  remain  passive 
throughout  the  process  and  the  degenerative  changes  present  in  them 
are  usually  regarded  as  secondary  to  the  pressure  of  the  enlarging  bony 
masses  and  to  ankylosis. 

Many  different  opinions  have  been  expressed  as  to  the  true  nature 
of  myositis  ossificans.  Virchow  placed  it  on  the  border-line  between 
tumor-formation  and  inflammation.  Mays  and  Cahen,  among  others, 
regard  it  as  a  true  tumor.  Nicoladoni  advances  the  hypothesis  that 
the  disease  is  a  trophoneurosis.  Eichhorst  thinks  that  it  is  secondary 
to  some  disease  of  the  spinal  cord. 

Among  the  predisposing  causes  may  be  mentioned  exposure  to 
cold,  unhygienic  surroundings,  and  rheumatism.  The  most  important 
exciting  cause  is  trauma,  either  a  single  injury  or  repeated  irritation. 
This  plays  the  most  important  part  in  the  local  form  of  the  disease. 
We  have  analogous  formation  of  bone  in  the  choroid  of  the  eye  in 
chronic  inflammation,  and  it  is  supposed  that  there  is  here  a  metaplasia 
of  the  newly-formed  granulation  and  connective  tissue  due  to  a  constant 
mild  stimulation.  Warthin  is  inclined  to  favor  the  view  that  in  the 
localized  form,  in  which  he  includes  inflammatory  ossification  and  the 
"exercise  bone,"  there  is  an  atavistic  reversion  to  the  splint-bones  of 
the  lower  animals. 

With  regard  to  the  progressive  form  of  the  disease,  the  fact  that  it 
is  so  commonly  found  in  early  childhood  and  that  it  is  usually  sym- 
metrical has  suggested  that  there  is  some  congenital  anomaly  at  work. 
This  view  is  supported  by  the  observation  of  Florschiitz,  who  noted 
the  frequent  association  of  microdactyly  with  ankylosis  of  the  phalanges 
of  the  thumb  and  the  absence  of  one  phalanx  of  both  great  toes.  This 
malformation  has  since  been  found  in  about  75  per  cent,  of  the  cases. 
Hallux  valgus,  malposition  and  hypoplasia  of  certain  organs  of  genera- 
tion, have  also  been  reported.  If  we  take  this  view,  we  may  assume 
that  portions  of  the  osteogenic  layer  of  the  periosteum  or  its  forerunners, 
or  possibly  bone-corpuscles,  have  become  isolated  in  the  course  of  growth 
from  their  proper  environment,  forming  "rests"  within  the  muscles, 
which  have  subsequently  taken  on  abnormal  activity.  Or,  if  may  be 
that  undifferentiated  mesenchymal  cells  have  remained  dormant  until, 
under  some  sufficient  stimulus,  they  have  developed  into  masses  of 
connective  tissue,  cartilage,  or  bone  in  abnormal  situations.1 

Tuberculosis. — Tuberculosis  of  the  muscles  is  usually  secondary 
to  tuberculosis  of  neighboring  parts,  such  as  the  joints,  bones,  skin, 
mucous  and  serous  membranes.  It'  is  met  with  commonly  in  the 
muscles  of  the  back,  pelvis,  and  hip-joint,  as  a  result  of  tuberculosis 
of  the  spinal  column,  pelvic  bones,  and  hip-joints.  The  iliopsoas 
and  gluteal  muscles  are  commonly  involved  in  tuberculosis  of  the 
lumbar  vertebrae  and  the  sacro-iliac  synchondrosis. 

1  For  a  discussion  of  the  nature  of  myositis  ossificans  and  a  full  bibliography, 
see  Lydia  M.  De  Witt,  Amer.  Jour.  Med.  Sci.,  120:  1900:  295 


986  THE  MUSCLES 

The  intermuscular  connective  tissue  in  such  cases  presents  chronic 
thickening,  and  there  may  be  the  formation  of  numerous  granulomas, 
which  by  their  confluence  lead  to  the  production  of  large  caseous 
nodules  and,  when  they  soften,  to  the  so-called  "cold"  abscesses.  The 
abscess  cavities  are  lined  by  tuberculous  granulation  tissue.  The  puri- 
form  material  often  burrows  extensively  through  the  muscle  and  along 
the  fascia,  and  may  burst  externally,  leaving  discharging  fistula?  or 
sinuses.  Large  areas  of  muscle  and  connective  tissue  may  be  destroyed 
in  this  way.  The  process  spreads  through  the  formation  of  new  tuber- 
cular foci  in  the  neighborhood  of  the  abscesses,  which  in  their  turn 
enlarge,  become  confluent,  and  break  down.  In  the  more  chronic 
forms  there  is  a  considerable  proliferation  of  fibrous  tissue,  which 
invades  and  replaces  the  muscle-bundles  and  in  its  turn  becomes 
caseous. 

Microscopically,  the  tuberculous  nodules  consist  of  a  caseous  necrotic 
centre,  bounded  by  a  zone  of  lymphocytes  and  epithelioid  cells,  with 
possibly  occasional  giant-cells.  The  smaller  bloodvessels  may  be 
obliterated  by  proliferative  endarteritis.  The  muscle-fibers  in  the  neigh- 
borhood of  the  destructive  process  are  usually  atrophied,  while  the 
nuclei  are  increased  in  number.  In  many  places  all  that  remains  is 
the  sarcolemma  sheath  with  nuclei.  Where  the  muscle  has  disappeared, 
its  place  is  taken  by  connective  tissue,  which  may  be  seen  in  places  to 
be  infiltrated  by  tuberculous  granulation  tissue. 

Primary  tuberculosis,  so  far  as  is  known  at  present,  is  rare.  It  is 
always  hematogenous.  The  rarity  of  the  affection  is  believed  by  some 
to  be  due  to  the  bactericidal  action  of  the  muscle  fluids,  which  Trya 
states  is  more  powerful  than  that  of  other  tissue  juices.  Primary  tuber- 
culosis may  be  a  manifestation  of  general  miliary  infection,  or  may  be 
purely  local.  Multiple  miliary  foci  of  tuberculous  granulation  tissue 
may  be  found,  or  larger  single  or  multiple  nodules,  which  in  time  may 
soften  and  break  down  into  abscesses.  In  certain  cases  these  abscesses 
become  delimited  by  connective  tissue.  In  some  instances  there  is  a 
more  infiltrating  or  diffuse  process  at  work,  which  at  first,  or  until 
caseation  sets  in,  bears  a  general  resemblance  to  sarcoma. 

Syphilis. — Syphilis  of  the  muscles  takes  the  form  of  chronic  pro- 
liferative inflammation  of  the  connective  tissue  (myositis  fibrosa  syphi- 
litica)  or  of  gummas.  The  muscles  usually  affected  are  the  biceps 
and  those  of  the  neck,  back,  throat,  tongue,  and  sphincter  ani.  The 
gummas  are  often  of  large  size,  and  are  surrounded  by  dense  connective 
tissue.  In  the  early  stages  the  vascular  granulation  tissue  may  easily 
be  mistaken  for  sarcoma,  but  the  course  of  the  case  and  the  therapeutic 
test  will  generally  differentiate.  The  muscle-fibers  in  the  neighbor- 
hood undergo  secondary  atrophy  and  the  extensive  fibrosis  frequently 
leads  to  marked  contractures. 

Gonorrhoeal  Myositis. — Gonorrhosal  myositis  is  occasionally  hema- 
togenic,  but  usually  arises  by  extension  from  the  joints  or  bones.  In 
this  form  interstitial  proliferation  of  the  connective  tissue  is  a  marked 
feature. 


PARASITES 


987 


Glanders. — Glanders  may  be  acute  or  chronic.  It  produces  multiple 
abscesses  throughout  the  muscle.  The  infection  is  hematogenic  or 
lymphogenic.  The  muscles  of  the  calves  are  the  ones  chiefly  involved. 
The  abscesses  contain  a  thin,  greasy,  puriform  fluid  of  grayish  color, 
in  which  the  specific  microorganisms  can  usually  be  demonstrated. 

Actinomycosis. — Actinomycosis  is  metastatic  or  prbduced  by  direct 
extension  from  some  neighboring  part.  Sluggish  granulomas  are  formed, 
showing  fatty  degeneration  and  liquefaction.  In  this  way  abscesses  are 
produced  which  may  heal  with  the  formation  of  fibrous  nodules. 

Parasites. — The  chief  parasites  are  the  Trichina  spiralis,  the  Cysti- 
cercus  celluloses,  and  the  Echinococcus. 

The  most  common  is  the  Trichina  spiralis,  which  enters  the  human 
organism  through  the  ingestion  of  imperfectly  cooked,  infected,  or 
"measly"  pork.  The  parasite  is  found  encysted  in  great  numbers  in 


FIG.  267 


TrichinsD  encysted  in  muscle.     Zeiss  obj.  DD,  without  ocular. 
University,  Pathological  Department.) 


(Collection  of  McGill 


the  diaphragm,  tongue,  muscles  of  the  neck,  larynx,  thighs,  the  inter- 
costals,  and  to  some  extent  in  other  parts.  The  embryos  of  the  Trichina 
are  produced  in  the  intestine  and  make  their  way  along  the  lymphatics 
to  the  various  muscles.  At  first  the  organism  is  found  in  the  sarcolemma 
sheath,  but  soon  invades  the  muscle-fiber,  which  in  time  degenerates 
and  disappears.  When  the  condition  is  fully  developed  there  is  found 
a  clear,  chitinous  cyst  in  which  the  parasite  lies  coiled  up  amid  a  granular 
detritus.  These  cysts  are  large  enough  to  be  easily  recognized  by  the 
naked  eye.  In  the  early  stage  there  is  more  or  less  acute  inflammation 


THE  MUSCLES 


with  round-celled  infiltration  of  the  intermuscular  connective  tissue, 
the  formation  of  fibroblasts,  and  multiplication  of  the  muscle-nuclei. 
Later,  the  acute  manifestations  pass  off  and  the  cyst  becomes  infiltrated 
with  lime  salts. 


RETROGRESSIVE  METAMORPHOSES. 

The  physical  state  of  a  muscle  is  conditioned  by  the  degree  of  its 
functional  activity,  the  nature  of  the  nervous  impulses  reaching  it, 
and  the  character  of  the  metabolism  going  on  within  it.  In  cases 
where  the  functional  demands  are  diminished  or  altogether  lacking 
the  muscle  undergoes  atrophy  and  degeneration.  Where  there  is  an 
increased  call  upon  its  activity,  hypertrophy  and  hyperplasia  result.  If, 
however,  the  demand  be  excessive  or  too  prolonged,  hypertrophy  in 
time  gives  way  to  atrophy.  Again,  so  long  as  the  normal  relationship 
between  the  muscle  and  the  nervous  system  is  maintained,  the  muscle 
retains  its  natural  tonus.  Where  this  relationship  is  disturbed,  as  in 
certain  disorders  of  the  central  nervous  system  and  peripheral  nerves, 
the  tonus  is  gradually  impaired,  nutrition  is  defective,  and  the  muscle 
eventually  undergoes  wasting  and  degeneration. 

FIG.  268 


Generalized  marantic  atrophy,  from  "  summer  diarrhoea."     (Dr.  A.  E.  Vipond's  case.) 

Atrophy. — Atrophy  of  muscle  may  be  divided  into  four  main 
varieties,  simple,  degenerative,  neuropathic,  and  primary  myopathic 
or  dystrophic  atrophy. 

Simple  Atrophy. — Simple  atrophy  is  found  typically  in  old  age  (senile 
atrophy)  and  in  athrepsia  and  inanition  from  any  cause  (marantic 
atrophy).  In  many  cases  the  condition  is  a  transient  one,  provided 
that  the  exciting  cause  be  removed,  and  the  muscle  returns  to  its 
normal  condition.  In  other  instances,  however,  when  the  condition  is 
long-continued,  degenerative  changes  are  superadded.  Atrophy  from 
disuse  may  be  brought  about  by  fracture  of  a  muscle,  tendon,  or  bone, 
ankylosis  of  joints,  fixation  by  splints,  or  even  by  voluntary  inactivity. 
The  last-mentioned  form  is  met  with  in  certain  fakirs  in  India,  who 
retain  a  limb  in  some  fixed  position  for  prolonged  periods.  Over- 
work, by  excessive  contraction  of  the  fibers  and  exhaustion  of  nutrition, 
will,  after  a  preliminary  stage  of  compensatory  hypertrophy,  also  give 


NEUROPATHIC  ATROPHY  989 

rise  to  atrophy.  Perhaps  more  common  are  degenerative  atrophies, 
such  as  those  met  with  in  infections  and  intoxications,  chronic  cachexias, 
anemia,  arteriosclerosis,  and  pressure. 

In  general,  atrophic  muscles  are  small,  pale,  and  flabby,  or  some- 
times brownish  from  a  deposit  of  pigment.  In  simple  atrophy  the 
changes  are  not  striking.  The  fibers  are  thinner  than  normal,  are 
losing  their  striation,  and  occasionally  are  fragmented.  The  connective 
tissue  between  the  bundles  may  be  relatively  or  absolutely  increased, 
and  may  present  a  deposit  of  fat — lipomatous  atrophy.  In  many  cases, 
however,  and  possibly  the  majority,  these  changes  are  combined  with 
others  of  a  degenerative  nature,  such  as  Zenker's  degeneration,  cloudy 
swelling,  lacunar  erosion,  hydropic  and  fatty  changes.  In  both  the 
simple  and  degenerative  forms  of  atrophy,  rows  or  clusters  of  nuclei 
are  met  with  along  the  atrophied  fibers,  upon  which  they  abut,  due 
possibly  to  a  more  or  less  abortive  attempt  to  reproduce  the  damaged 
structures.  When  the  atrophy  is  complete,  sarcolemma  sheaths  are 
found  containing  pigment,  nuclei,  and  multinucleated  cells. 

Neuropathic  Atrophy. — Of  great  practical  importance  are  the  neuro- 
pathic atrophies.  Here  the  process  is  confined  to  certain  muscles  or 
groups  of  muscles,  and  is  due  to  a  lesion  in  the  central  or  peripheral 
nervous  system.  In  the  first  case  one  finds  a  degeneration  of  the  gan- 
glion cells  of  the  anterior  horns  of  the  spinal  column,  in  the  pyramidal 
tracts,  the  medulla,  and  the  motor  areas  of  the  cerebral  cortex.  This 
results  in  what  are  called  respectively  progressive  spinal,  bulbar,  and 
cerebral  atrophies.  The  extent  of  the  atrophy  resulting  is  dependent 
upon  the  degree  of  the  disease  present  in  the  trophic  centres,  and  its 
localization  is  governed  by  the  particular  centres  involved.  In  certain 
diseases  with  a  comparatively  localized  lesion,  such  as  anterior  polio- 
myelitis, myelomalacia,  certain  forms  of  sclerosis,  tumors,  and  com- 
pression myelitis,  the  atrophy  is  confined  to  those  small  groups  of 
muscles  deriving  their  nutrition  from  the  affected  cells,  but  some  of 
the  affections  are  progressive,  leading  to  extensive  muscular  wasting 
and  in  some  instances  to  the  involvement  of  nearly  all  the  principal 
muscles  of  the  body.  In  some  cases,  as  in  amyotrophic  lateral  sclerosis, 
the  primary  degeneration  is  not  confined  to  the  trophic  ganglion  cells 
but  extends  to  the  pyramidal  tracts  and  other  regions  near  by. 

The  neuropathic  atrophies  usually  attack  persons  who  have  previously 
been  well  and  strong,  and  usually  affect  those  muscles  which  are  most 
used.  In  manual  laborers  the  muscles  of  the  thenar  and  hypothenar 
eminences,  the  lumbricales  and  the  interossei,  are  liable  to  be  first 
involved  (Aran-Duchenne  type).  In  other  cases  the  disease  begins  in 
the  muscles  of  the  shoulder  and  arm.  The  disease  is  often,  although 
not  invariably,  symmetrical,  and  gradually  extends  from  one  group 
of  muscles  to  another,  until,  unless  the  disease  be  arrested,  most  of  the 
muscles  are  involved  and  the  patient  becomes  almost,  if  not  entirely, 
helpless.  In  cases  where  the  medulla  is  first  involved  there  is  diffi- 
culty in  articulation  and  deglutition,  with  drooling  of  saliva  and  feeble- 
ness of  the  voice  (progressive  bulbar  paralysis).  The  muscles  of  the 


990  THE  MUSCLES 

lower  extremities  are  involved  late,  if  at  all.  Irregular  forms  are  also 
met  with  in  which  the  wasting  begins  in  the  lower  extremities  and 
extends  gradually  upward. 

Secondary  muscular  atrophy  is  also  met  with  in  certain  affections 
of  the  peripheral  nerves,  such  as  multiple  neuritis,  chronic  lead  and 
arsenical  poisoning,  injuries  to  the  motor  nerves. 


FIG.  269 


Progressive  muscular  dystrophy  (family  type,  showing  the  characteristic  pose).     (From 
the  clinic  of  the  Royal  Victoria  Hospital.) 

Bearing  a  somewhat  close  resemblance  to  the  progressive  spinal 
atrophies  is  the  so-called  primary  myopathy  or  progressive  muscular 
dystrophy.  This  affection  frequently  begins  in  childhood,  but  is  also 
met  with  in  adults.  The  cause  is  quite  obscure,  but  it  is  character- 
istically a  familial  disease,  so  that  it  has  been  suggested  that  some 
congenital  anomaly  of  development  is  at  work. 

Three  clinical  types  are  recognized:  the  infantile,  juvenile,  and  adult. 
The  first-mentioned  form  usually  begins  in  the  muscles  of  the  face, 
giving  rise  to  a  peculiar  expressionless  appearance  pathognomonic  of 
the  disease — the  myopathic  facies.  The  juvenile  form  involves  the 
muscles  of  the  calves,  thighs,  back,  shoulder-girdle,  and  arms.  The 


PRIMARY  MYOPATHY 

adult  variety  begins  either  in  the  lower  extremities  or  in  the  upper 
extremities  and  face. 

In  this  affection  no  constant  changes  have  been  found  in  the  cord 
or  peripheral  nerves,  although  some  observers  have  described  atrophy 
of  the  ganglion-cells  of  the  posterior  roots  and  cytoplasmic  changes  in 
the  ganglion-cells  of  the  spinal  cord.  Kollaritz  has  described  atrophy 
of  the  motor  cells  and  fibers  of  the  central  gray  matter  of  the  cervical 
and  dorsal  cord.  The  majority  of  observers  seem  to  think  that  the 
disease  originates  in  the  voluntary  muscles.  It  is  quite  possible,  how- 
ever, that  the  changes  in  the  central  nervous  system  and  the  muscle 
are  related,  and  that  both  are  dependent  on  errors  of  development.  For 
it  is  obvious  that  developmental  errors  affecting  the  ganglion  cells  and 
motor  fibers  would  necessarily  be  associated  with  imperfect  develop- 
ment of  the  corresponding  muscles.  Others  again  would  regard  the 
nerve  changes  as  secondary  to  the  degeneration  of  the  muscle-fibers. 
Clinically  and  anatomically  there  are  two  forms,  the  atrophic  and  the 
pseudohypertrophic. 

In  the  former  the  changes  in  the  muscle  do  not  differ  materially 
from  those  found  in  the  spinal  form.  There  are  vacuolation,  fragmen- 
tation, atrophy,  and  finally  disintegration  of  the  muscle-fibers.  The  trans- 
verse striation  is  lost  and  the  fibers  appear  to  be  either  homogeneous 
or  finely  granulated.  Numerous  cells  may  be  met  with  containing 
many  nuclei,  thus  resembling  giant  cells.  There  is  also  a  certain 
amount  of  proliferation  of  the  connective  tissue  forming  the  endomysium. 
These  appearances  have  been  variously  interpreted.  Hoffmann  and 
Waldeyer  hold  that  the  striated  muscle  cells  are  a  syncytium  pro- 
duced by  the  fusion  of  spindle-shaped  mesodermic  cells.  Some,  like 
Durante  and  Kroesing,  regard  the  longitudinal  fibrillation  of  the  muscle- 
fiber  as  a  reversionary  degeneration.  They  believe  that  these  fibrillae 
are  competent  to  form  new  muscle-fibers,  but  usually  degenerate  into 
connective  tissue.  The  increase  of  the  connective  tissue  may,  therefore, 
be  due  to  this  degeneration  rather  than  to  proliferation  of  the  old 
connective  tissue.  It  is  more  generally  believed,  however,  that  the 
increase  of  the  connective  tissue  is  owing  to  the  well-known  tendency 
of  connective  tissue  to  replace  destroyed  structures.  The  large  multi- 
nucleated  cells  referred  to  are  usually  regarded  as  evidence  of  an 
attempt  at  regeneration,  but  Fujinami  holds  that  they  are  identical  with 
the  myoblasts  of  growing  muscle  and  that  therefore  they  indicate  a 
return  to  an  embryonic  condition. 

The  pseudohypertrophic  form  presents,  in  addition  to  the  changes 
just  described,  a  formation  of  fatty  tissue  resulting  in  a  notable  increase 
in  the  bulk  of  the  affected  muscle.  The  muscle-fibers  here  again  are 
more  or  less  atrophied.  A  true  hypertrophy  of  the  fibers,  accompanying 
the  increase  of  connective  and  adipose  tissue,  has  been  observed  by 
Durante.  In  most  cases  the  fatty  transformation  follows  the  atrophy 
and  is  compensatory,  in  a  manner  somewhat  analogous  to  the  produc- 
tion of  fat  about  a  contracted  or  fibroid  kidney,  although  in  some  few 
cases  it  is  possibly  the  precursor  '  le  cause  of  the  muscular  atrophy 


992  THE  MUSCLES 

from  simple  pressure.  The  fat  is  produced  probably  from  the  pro- 
liferating cells  of  the  endomysium,  although  Kroesing  believes  that  there 
is  a  direct  metaplasia  of  a  muscle  into  fat. 

Degenerations.— Cloudy  Swelling. — Cloudy  swelling  (parenchymatous 
degeneration,  albuminous  degeneration,  parenchymatous  myositis)  is  a 
common  accompaniment  of  acute  febrile  diseases,  and  is  also  brought 
about  by  inflammation,  circulating  toxins,  and  disturbances  of  circulation. 
The  affected  muscle  is  pale,  swollen,  and  doughy. 

Microscopically,  the  fibers  are  swollen,  the  striation  indistinct,  and 
the  cytoplasm  contains  innumerable  minute  globules  of  an  albuminous 
nature,  which  render  it  somewhat  opaque.  These  are  readily  dissolved 
out  by  the  application  of  dilute  alkali  or  acetic  acid. 

Fatty  Degeneration. — In  severe  cases  the  condition  passes  by  easy 
stages  into  fatty  degeneration.  This  is  also  met  with  in  phosphorus 
poisoning.  In  this  condition  the  muscle  is  soft,  friable,  and  of  a  pale 
yellow,  streaky  appearance. 

Microscopically,  the  fibers  are  swollen,  paler  than  usual,  somewhat 
reticulated,  and  the  nuclei  show  degenerative  changes.  When  the  tissue 
is  treated  with  osmic  acid  the  fatty  droplets  within  the  fibers  stain 
brown  or  black,  and  with  Sudan  III,  yellow  or  carmine. 

Fatty  Infiltration. — In  fatty  infiltration  there  is  a  deposit  of  fat  in 
the  connective  tissue  of  the  endomysium.  In  the  earlier  stages  there 
is  sometimes  a  proliferation  of  the  cells  of  the  endomysium.  In  many 
instances  the  infiltration,  or  metaplasia,  whichever  it  may  be,  is  only 
microscopic  in  amount,  but  in  some  cases,  as  in  pseudohypertrophic 
muscular  paralysis,  the  amount  of  fat  is  excessive  and  leads  to  enlarge- 
ment of  the  muscle  bundles. 

Hydropic  or  Vacuolar  Degeneration. — Hydropic  or  vacuolar  degenera- 
tion is  met  with  in  oedema  of  the  muscle  and  some  forms  of  inflamma- 
tion. The  muscle  appears  to  be  pale  and  watery. 

Colliquative  Necrosis. — Colliquative  necrosis  occurs  in  chronic  oedema 
and  suppurative  inflammation.  The  fibers  at  first  are  enlarged,  granular, 
or  vacuolated,  and  finally  melt  away. 

Lacunar  Erosion. — Lacunar  erosion  is  a  process  analogous  to  the 
lacunar  erosion  of  bone.  Here  certain  cells,  derived  possibly  from  the 
internal  perimysium  or  the  sarcolemma  sheath,  attack  the  fibers  and 
lead  to  atrophy.  This  is  seen  most  frequently  in  the  neighborhood 
of  metastatic  cancerous  nodules. 

Vitreous,  Waxy,  or  Hyaline  Degeneration. — Vitreous,  waxy,  or  hyaline 
degeneration  is  a  form  of  coagulation  necrosis  affecting  the  muscle 
protoplasm,  first  described  by  v.  Zenker1  as  occurring  in  typhoid  fever, 
and  hence  called  Zenker's  degeneration.  It  is,  however,  met  with  in 
a  great  many  other  conditions,  such  as  sepsis,  variola,  intoxications, 
inflammation,  traumatism,  burns,  freezing,  bedsores,  tetanus,  and  in 
the  neighborhood  of  carcinomas.  The  muscles  usually  affected  are  the 
recti  abdominis,  the  adductors  of  the  thighs,  and  psoas. 

1  Ueber  d.  Veranderung.  d.  willkurl.  Musk,  bei  Typhus  abdom.,  Leipzig,  1864. 


GANGRENE 


993 


FIG.  270 


When  the  condition  is  extensive,  the  affected  muscle  is  soft,  doughy, 
pale,  and  semitranslucent  like  raw  fish. 

Microscopically,  the  fibers  are  swollen,  the  striations  have  disappeared, 
and  the  protoplasm  has  a  structureless,  waxy,  somewhat  opaque  appear- 
ance. In  the  milder  forms  the 
nuclei  are  preserved  and  regener- 
ation is  believed  to  be  possible, 
but  in  severe  cases  the  nuclei  dis- 
appear. There  is  usually  to  be 
seen  a  small-celled  infiltration  of 
the  endomysium.  In  the  most 
advanced  cases  the  swollen  fibers 
fragment  and  coalesce  into  irreg- 
ular hyaline  masses.  The  hyaline 
substance,  as  a  rule,  stains  badly, 
although  at  times  it  presents  the 
microchemical  reactions  of  fibrin 
or  colloid.  Disintegration  is  oc- 
casionally so  extreme  that  hemor- 
rhages, often  of  large  size,  may 
take  place  into  the  muscle. 

Amyloid  Transformation. — Amy- 
loid transformation  is  rare  and 
apparently  always  a  local  condi- 
tion, being  found  in  situations 
where  there  has  been  previous  in- 
flammation. According  to  Ziegler, 
amyloid  disease  is  met  with  in  the 
tongue  and  larynx.  The  process 
begins  in  the  perimysium  internum  and  the  sarcolemma  sheaths,  which 
are  converted  into  a  thick  translucent  material  encroaching  upon  the 
muscle-fibers  and  eventually  leading  to  their  atrophy.  In  time,  neigh- 
boring deposits  become  confluent  and  form  nodular  masses. 

Calcification. — Calcification  has  been  observed  in  the  muscles  in  the 
neighborhood  of  old  encapsulated  abscesses,  in  certain  other  chronic 
inflammatory  processes,  trauma,  and  as  a  sequel  of  marked  atrophy. 
The  condition  may  follow,  for  example,  suture  of  a  wound  of  the 
muscle.  According  to  Schujeninoff,  calcification  takes  place  after  the 
fibers  have  undergone  colloid  degeneration. 

Gangrene.  —  Gangrene  occurs  in  severe  infective  processes,  decu- 
bitus,  burns,  freezing,  sclerosis  of  the  nutrient  arteries,  or  results  from 
pressure  exerted  on  tissues  of  low  vitality.  The  affected  muscles  become 
brownish,  grayish-  or  greenish-black,  break  up  into  shreds  or  liquefy 
(moist  gangrene).  In  cases  where  the  blood  supply  to  the  part  is  scanty 
and  the  tissue  is  exposed  to  the  air,  the  part  dries  up  (mummification). 
Owing  to  the  presence  of  putrefactive  germs,  noxious  gases  are  pro- 
duced and  the  tissues  become  very  foul  and  offensive.  A  very  rapid 
and  malignant  form  of  gangrene,  associated  with  the  production  of  gas 
63 


V.  Zenker's,  or  wax-like,  degeneration  of 
muscle  fibers  (a,  6)  seventeen  hours  after  tem- 
porary ligation  of  the  same.  In  b  there  is 
already  some  accumulation  of  leukocytes. 
(Oberndorffer.) 


994  THE  MUSCLES 

in  the  subcutaneous  tissues,  is  the  so-called  emphysematous  gangrene, 
or  gangrene  foudroyante,  due  to  the  B.  Welchii  or  the  bacillus  of  malig- 
nant oedema. 

Microscopically,  in  the  moist  form,  the  muscle-fibers  show  simple 
or  coagulation  necrosis,  vacuolation,  and  liquefaction,  while  there  is  a 
deposit  of  blood-pigment,  cholesterin,  and  triple  phosphates.  In  dry 
gangrene  the  cells  shrink  and  dry  up  into  a  keratinoid  substance.  As  a 
rule,  the  dead  tissue  is  delimited  from  the  healthy  part  by  a  zone  of 
reactive  inflammation  in  which  the  vessels  are  congested,  and  there  is 
an  exudation  of  inflammatory  leukocytes. 

FIG.  271 


Senile  gangrene  of  the  great  toe,  from  a  case  of  arterial  thrombosis.  The  toe  is  shrunken  and 
its  epidermis  is  being  exfoliated.  At  the  line  of  demarcation  the  skin  has  retracted  (a)  and  the 
deeper  parts  are  separating  (6). 

Pigmentation. — Pigmentation  is  a  constant  accompaniment  of  that 
form  of  atrophy  known  as  brown  atrophy.  The  pigment,  called  hemo- 
fuscin,  is  found  in  the  form  of  minute,  yellowish-brown  granules  in 
the  cytoplasm  about  the  poles  of  the  nuclei,  but  in  advanced  cases 
is  scattered  throughout  the  greater  part  of  the  fiber.  In  some  cases 
the  increase  in  pigment  is  possibly  only  a  relative  matter,  due  to  atrophy 
of  the  cytoplasm  with  retention  of  the  normal  muscle  pigment.  In 
other  cases  there  seems  to  be  a  transformation  of  the  myohematin 
into  granular  material,  leading  to  an  absolute  increase. 

In  cases  of  hemorrhage,  hematoidin  and  hemosiderin  may  be  deposited 
in  the  muscle-bundles  and  connective  tissue. 


PROGRESSIVE  METAMORPHOSES. 

The  repair  of  injury  to  muscles,  such  as  that  produced  by  disease 
or  mechanical  trauma,  may  be  perfect  or  imperfect  according  to  the 
character  and  extent  of  the  lesion.  Provided  that  the  muscle-nuclei 


HYPERTROPHY  995 

and  the  sarcolemma  be  not  destroyed,  slight  injuries  to  the  contractile 
substance  are  repaired  by  the  production  of  new  muscle-fibers  exactly 
as  in  the  cases  of  the  embryonal  formation  of  muscle.  Volkmann 
states  that  regeneration  is  functionally  important  only  after  typhoid 
fever  and  freezing,  while  in  injuries  function  is  not  restored  unless  the 
wound  is  small.  Where  large  areas  of  muscular  tissue  are  destroyed, 
as  by  inflammation  or  severe  mechanical  trauma,  regeneration  of  muscle 
is  incomplete  and  a  large  part  of  the  deficit  is  made  good  by  the  forma- 
tion of  granulation  and  ultimately  connective  tissue.  In  all  cases  the 
repair  is  brought  about  by  the  proliferation  of  the  preexisting  cells 
of  the  part.  Where  a  fibrous  scar  has  been  produced,  after  a  more  or 
less  prolonged  period,  it  is  replaced  to  a  limited  extent  by  the  invasion 
of  new  muscle-fibers  derived  from  the  uninjured  muscle  in  the  neighbor- 
hood. The  muscle-nuclei  divide  either  directly  or  by  mitosis,  while 
at  the  same  time  there  is  a  local  increase  of  the  sarcolemma  substance. 
At  the  end,  or  at  some  point  along  the  course  of  the  fiber,  multinucleated 
buds  of  protoplasm  are  produced  which  grow  out  into  the  granulation 
tissue.  At  first  these  present  no  striation,  but  soon  become  fibrillated, 
and  the  bud,  or  myoblast,  as  it  is  called,  becomes  striated.  Besides  the 
myoblasts,  free  multinucleated  cells  may  be  seen  which  are  known 
as  sarcolytes.  The  majority  of  these  probably  undergo  fatty  degenera- 
tion or  necrosis  and  ultimately  disappear,  although  some  of  them  may 
form  new  fibers  or  fuse  with  preexisting  ones.  The  sarcolytes  are 
probably  derived  from  free  muscle-corpuscles,  and,  where  there  has 
been  an  actual  loss  of  substance  in  the  muscle,  are  apt  to  be  rather 
numerous.  They  are  phagocytic  and  may  be  seen  to  contain  pigment, 
bits  of  necrotic  muscle,  and  detritus. 

Hypertrophy. — True  hypertrophy  of  muscle  probably  does  not 
occur  except  in  the  case  of  individual  fibers.  It  is  true  that  under 
conditions  of  increased  work  the  muscle-fibers  increase  both  in  length 
and  breadth,  but  there  is  practically  always  a  numerical  increase  as 
well,  so  that  the  condition,  more  correctly  speaking,  is  a  combination 
of  hypertrophy  and  hyperplasia.  Hypertrophy,  so-called,  may  be 
congenital  as  well  as  acquired,  as  in  the  cases  of  general  and  local 
gigantism  and  hemihypertrophy. 

In  myotonia  congenita,  or  Thomsen's  disease,  Erb1  has  described  a 
marked  increase  in  the  size  of  the  affected  muscle-fibers,  with  prolifera- 
tion of  nuclei,  loss  of  striation,  and  vacuolation.  Koch  describes 
longitudinal  cleavage  of  the  fibers,  producing  numerical  increase.  He 
observed  amitotic  division  of  the  nuclei  resulting  in  the  production  of 
narrow  elongated  cells,  which  contained  rows  of  nuclei  similar  to  those 
found  in  degenerative  and  regenerative  processes.  It  is  doubtful 
whether  the  disease  is  not  a  degenerative  process  rather  than  true 
hypertrophy.  In  certain  cases  of  muscular  atrophy,  the  myopathies, 
local  trauma,  and  certain  infectious  diseases,  like  typhoid,  isolated 
fibers  may  show  hypertrophy,  apparently  compensatory. 

1  Die  Thomsen'sche  Krankheit,  Leipzig,  1886. 


996  THE  MUSCLES 

Tumors. — Primary  tumors  of  the  muscles  are  quite  rare  and  gen- 
erally arise  from  the  fascia  or  intermuscular  connective  tissue.  Among 
the  benign  growths  may  be  mentioned  the  fibroma,  lipoma,  myxoma, 
chondroma,  and  angioma.1  Hard  fibromas  have  been  observed  in  the 
fascia  of  the  recti  abdominis,  and  myxomas  in  the  muscles  of  the  thigh. 
Myomas  may  originate  from  the  arrectores  pilorum. 

Sarcoma. — The  most  frequent  growths  are  the  various  forms  of  sarcoma, 
fibrosarcoma,  myxosarcoma,  and  myxoliposarcoma.  These  form  large 
tumors  and  are  soft  and  cellular,  being  composed  of  round  or  spindle  cells. 

Various  forms  of  degeneration  are  common,  and  there  may  be  meta- 
plasia into  bone  or  cartilage. 

FIG.  272 


Myoma  from  the  arm.     Winckel  obj.  No.  3,  without  ocular.     (From  the  collection  of 
Dr.  A.  G.  Nicholls.) 

Myosarcoma,  that  is  to  say,  a  sarcoma  originating  from  embryonic 
muscle  cells,  is  theoretically  possible,  but  little  is  known  as  to  its  actual 
occurrence. 

Rhabdomyoma  and  rhabdomyosarcoma  have  been  described  by  Billroth2 
and  Buhl,3  but  are  excessively  rare.  We  have  met  with  one  case  (Fig.  273) 
of  a  rhabdomyosarcoma,  in  a  trout.4  It  is  thought  that  such  tumors  arise 
from  preexisting  muscle-cells  or  possibly  from  embryonic  cell  inclusions. 

Secondary  sarcoma  of  the  muscle  is  common. 

1  Porcile,  II  Policlinico,  Rome,  15:  1908:  289. 

2  Rhabdomyom,  Virch.  Arch.,  9:  1856:  172. 

3  Rhabdomyom,  Zeit.  f .  Biol,  1 : 1865. 

4  Adami,  Montreal  Med.  Journ.,  37: 1908: 163, 


TUMORS  997 

Carcinoma. — Carcinoma  is  always  secondary  and  due  to  direct  exten- 
sion or  lymphogenic  invasion  from  a  growth  in  some  neighboring  part, 
as,  for  example,  in  the  breast,  lips,  skin,  or  stomach.  Hematogenic 
metastasis  is  rare. 

The  growth  is  usually  diffuse  or  in  the  form  of  minute  miliary 
nodules,  arranged  in  clusters  or  rows  in  the  interstices  of  the  tissues. 
The  sarcolemma  sheath  may  be  invaded  by  the  cancer  cells,  so  that 
absorption  of  the  fibers  takes  place  with  the  formation  of  indentations 

FIG.  273 


Rhabdomyosarcoma  in  a  brook  trout.     Reichert  obj.  No.  7,  without  ocular.     (From  the 
collection  of  Dr.  J.  George  Adami.) 

similar  to  Howship's  lacunae.  Owing  to  pressure,  the  eroding  action  of 
the  cancer  cells,  and  the  influence  of  the  soluble  toxins,  the  muscle-cells 
undergo  atrophy,  coagulation  necrosis,  liquefaction,  and  ultimately  dis- 
appear. There  is  usually  a  certain  amount  of  interstitial  inflammation, 
and  the  endothelial  lining  of  the  neighboring  vessels  proliferates,  leading 
gradually  to  their  obliteration. 

Small  teratomas  and  dermoid  cysts  have  been  found  in  the  muscles 
of  the  cheek,  tongue,  neck,  abdominal  wall,  and  lumbar  region. 


THE  TENDONS,  TENDON-SHEATHS,  AND  BURS^l. 

The  tendons  are  composed  of  a  peculiar  dense,  avascular  connective 
tissue,  arranged  as  an  aggregation  of  small  bundles  which  are  embedded 
in  a  looser  connective  tissue  provided  with  bloodvessels.  They  are 


998  THE  TENDONS  AND  BURS& 

inclosed  in  a  sheath  containing  a  small  amount  of  synovial  secretion, 
thus  allowing  movement  of  the  tendon  backward  and  forward.  Owing 
to  the  peculiarly  dense  and  resistant  character  of  these  structures  and 
the  fact  that  the  bloodvessels  are  scanty,  primary  affections,  particularly 
the  infective  and  inflammatory  ones,  are  infrequent.  Much  more  com- 
monly, the  tendons  and  their  sheaths  are  secondarily  involved  from 
disease  processes  in  the  neighborhood. 

CIRCULATORY  DISTURBANCES. 

Hemorrhage  into  the  sheath  of  a  tendon  may  follow  trauma  and 
result  in  the  formation  of  a  hematoma. 


INFLAMMATIONS. 

Tendinitis.  —  Inflammation  of  a  tendon  is  called  tendinitis;  of  a 
tendon  sheath,  tendovaginitis  or  tenosynovitis.  The  two  conditions  are 
more  often  than  not  associated  one  with  the  other. 

In  general,  tendinitis  and  tenosynovitis  bear  a  close  resemblance  to 
inflammation  of  the  joints.  The  condition  usually  follows  trauma  or 
arises  by  extension  from  neighboring  structures.  It  is  rarely  primary. 
Wounds,  fractures,  and  strains  may  set  up  inflammation  and  also  cir- 
culating toxins  and  bacteria,  such  as  the  pyogenic  cocci,  Gonococci,  and 
Pneumococci.  Tendinitis  and  tenosynovitis  may  be  serous,  fibrinous,  or 
purulent  in  character. 

Simple  Tendinitis. — Simple  tendinitis  and  tenosynovitis  may  occur 
in  those  of  a  rheumatic  or  gouty  disposition  after  strain  of  the  tendons, 
exposure  to  cold,  and  rarely  in  certain  infective  diseases,  such  as 
syphilis,  typhoid,  and  scarlatina.  There  is  usually  little  exudate,  but 
owing  to  the  deposit  of  fibrin  upon  the  surfaces  of  the  tendon  and  sheath, 
a  rough,  grating  sensation  or  crepitus  is  felt  when  the  tendon  is  put 
in  action  (tenosynovitis  sicca  acuta).  It  is,  perhaps,  most  frequently 
met  with  in  the  tendo  Achillis,  and  next  in  the  extensor  tendons  of  the 
forearm. 

Acute  Purulent  Tendinitis. — Acute  purulent  tendinitis  and  tenosynovitis 
arise  after  traumatism  where  a  wound  has  become  infected,  or  by 
extension  from  neighboring  parts,  as,  for  instance,  in  erysipelas,  infected 
wounds  and  abscesses.  It  may  also  be  hematogenic,  due  to  the  action 
of  Gonococci  or  other  pyogenic  microorganisms. 

The  tendon  and  its  sheath  become  cedematous,  the  interfascicular 
connective  tissue  is  congested  and  infiltrated  with  round  cells,  while 
there  is  an  accumulation  of  pus  within  the  cavity.  In  mild  cases 
recovery  may  take  place  without  much  or  any  interference  with  func- 
tion. In  severe  cases  there  is  liable  to  be  adhesion  between  the  tendon 
and  its  sheath,  or  the  tendon  may  become  cloudy,  soften,  and  finally 
necrose.  Where  the  tendon  has  been  destroyed  regeneration  and  repair 
may  take  place. 


TENDINITIS  999 

Chronic  Tendinitis. — Chronic  tendinitis  may  occur  alone,  but  is  usually 
associated  with  tenosynovitis.  It  may  follow  the  acute  forms  or  repeated 
acute  attacks,  or,  again,  may  come  on  insidiously. 

In  this  form  degeneration  of  the  tendon  is  common.  Mucinous  and 
hyaline  changes  take  place.  The  interfascicular  connective  tissue  is 
increased  in  amount,  and  calcification  or  metaplasia  into  bone  or  carti- 
lage is  sometimes  met  with.  In  certain  diseases,  as  senile  arthritis  and 
rheumatoid  arthritis,  small  nodosities,  varying  in  size  from  that  of  a  pin- 
head  to  that  of  a  bean,  may  form  in  the  interfascicular  connective  tissue, 
arranged  often  like  a  string  of  beads.1  They  usually  disappear  sponta- 
neously in  a  few  months,  but  may  undergo  hyaline  change  or  calcification. 

In  chronic  tenosynovitis  there  may  be  a  serous  exudate,  or,  again, 
but  little  fluid  may  be  present.  The  exudate,  when  present,  contains 
mucin  and  pseudomucin.  It  may  be  so  great  in  amount  as  to  cause 
marked  distension  of  the  tendon  sheath  (hygroma  of  the  tendon  sheath, 
hy drops  tendo-vaginalis).  This  usually  occurs  in  the  palms  and  in 
the  sheath  of  the  flexors  of  the  carpus.  At  times  the  accumulation 
is  so  great  that  cyst-like  swellings  are  produced.  Owing  to  the  fact 
that  the  sheath  is  contracted  at  the  annular  ligament  of  the  wrist,  fusi- 
form or  hour-glass-shaped  swellings  result.  More  rarely,  the  tendon 
sheaths  of  the  finger,  the  back  of  the  hand,  or  other  parts  may  be  involved. 
The  sheath  in  time  becomes  thickened  and  there  is  a  deposit  of  fibrin 
within  its  cavity.  This  finally  may  become  organized,  so  that  papillary 
or  polypoid  excrescences  are  formed,  at  first  hyaline,  but  later  fibrous, 
or  even  cartilaginous  or  bony.  These,  when  in  the  partially  organized 
condition,  may  break  loose,  forming  free  bodies  in  the  sac,  the  so-called 
"rice-bodies,"  or  "corpuscula  oryzoidea." 

Microscopically,  they  consist  of  homogeneous  or  laminated  masses  of 
hyaline  appearance,  in  which  but  few  cells  are  seen.  Giant  cells  and 
leukocytes,  however,  are  at  times  in  evidence.  They  are  particularly 
common  in  tuberculous  tenosynovitis.  In  fact,  hygroma  of  the  type 
just  described  is  held  by  some  to  be  commonly  tuberculous. 

Gout.  —  In  gout,  the  tendons  and  their  sheaths  are  frequently 
involved.  There  is  a  deposit  of  uratic  salts  in  the  stroma  of  the  tendon, 
the  peri-  and  interfascicular  connective  tissue  proliferates  and  becomes 
cellular,  the  tendon  bundles  are  dissociated,  and  the  various  tissues 
may  finally  undergo  degeneration  and  necrose.  The  salts  are  sur- 
rounded by  granulation  tissue  which  sometimes  contains  giant  cells. 

Tuberculosis. — Tuberculosis  of  the  tendons  and  their  sheaths  is 
rarely  primary,  but  usually  follows  tuberculosis  of  the  bones  or  joints. 
Primary  tuberculosis  occurs  most  frequently  in  the  forearm  (Schu- 
chardt2).  The  tubercles  are  formed  chiefly  in  the  tendon  sheaths  and 
are  accompanied  by  an  exudative  process.  The  sheath  in  time  becomes 
thickened  from  the  production  of  numerous  tubercles  within  its  sub- 
stance, as  well  as  by  the  formation  of  new  fibrous  tissue.  In  the  advanced 

1  See  Hirschsprung,  Jahrb.  f.  Kinder.,  16:  1881. 

2  Virch.  Archiv,  135:  1894:  394. 


1000  THE  BURS& 

stages  the  tendons  and  sheaths  may  be  incrusted  with  fungous  granula- 
tions covered  with  fibrin  or  puriform  exudation.  The  amount  of  the 
fluid  is  often  large,  and  "  rice-bodies"  are  common. 


RETROGRESSIVE  METAMORPHOSES. 

Degenerative  disturbances  are  apt  to  be  slight  and  unimportant. 

Atrophy. — Atrophy  may  occur  where  the  muscle  belonging  to  the 
tendon  has  previously  undergone  atrophy,  but  this  result  is  usually 
long  delayed.  The  structure  peculiar  to  the  tendon  is  lost  and  it  is 
converted  into  scar-like  connective  tissue.  The  supporting  stroma 
is  increased  through  proliferation  of  its  cells,  and  may  be  converted 
into  fat. 

Hyaline  and  mutinous  degeneration  have  been  observed  in  some  cases 
of  inflammation. 

After  injuries  or  severe  inflammation  the  tendons  may  necrose. 

Calcification  has  been  observed  in  chronic  inflammations,  and  uratic 
deposits  in  the  tendons  and  tendon  sheaths  are  frequently  met  with 
in  gout. 

PROGRESSIVE  METAMORPHOSES. 

When  a  tendon  is  severed,  provided  that  suppuration  does  not  occur, 
function  is  restored  by  the  union  of  the  divided  ends.  This  takes 
place,  not  by  the  formation  of  new  tendon,  but  by  the  production  of 
dense  scar-tissue,  more  grayish  and  less  glistening  than  tendon,  derived 
mainly  from  the  proliferation  of  the  connective-tissue  matrix. 

Metaplasia. — Metaplasia  of  the  tendinous  material  into  mucin,  fat, 
cartilage,  or  bone  is  met  with  in  many  forms  of  chronic  inflammation. 

Tumors. — Tumors  are  rare.  Sarcoma  may,  possibly,  develop  from  the 
tendon  or  its  sheath.  A  very  rare  growth  is  the  lipoma  arborescens,  in 
which  branching  papillary  excrescences  of  fatty  tissue  are  formed  within 
the  tendon  sheath. 

According  to  Ledderhose,1  the  so-called  ganglion  of  the  wrist,  back 
of  hand,  and  foot,  which  used  to  be  considered  a  form  of  hydrops  of 
the  sheath,  is  a  new-formation  of  a  myxomatous,  gelatinous,  or  colloid 
nature. 

The  Bursae. 

The  bursse  are  connective-tissue  sacs  containing  clear  synovial  fluid. 
The  structures  forming  the  wall  resemble  in  general  the  tendon  sheaths. 
Bursse  are  usually  found  in  well-defined  situations,  wherever  muscles 
or  tendons  play  over  bones,  or  where  the  tissues  are  subjected  to  press- 
ure. They  are  not  constant  in  number  or  in  situation,  but  may  be 

1  Zeitsch.  f.  Chir.,  37  :  1893. 


INFLAMMATIONS  1001 

developed  by  friction  or  pressure,  in  the  course  of  certain  occupations, 
in  places  where  they  ordinarily  are  not  present.  The  pathological 
processes  affecting  them  are  analogous  to  those  occurring  in  the  tendon 
sheaths. 

Hemorrhage  into  bursal  sacs  occurs  from  trauma  or  circulatory  dis- 
turbances. Large  blood  tumors  or  hematomata  may  be  produced. 

Inflammations. — Acute  bursitis  or  acute  hygroma  is  due  most  com- 
monly to  wounds,  bruises,  or  contusions,  or,  more  rarely,  to  hema- 
togenic  infection.  It  may  give  rise  to  a  serous,  serofibrinous,  or  puru- 
lent exudation.  A  painful  fluctuating  swelling  is  produced.  Common 
examples  of  this  are  the  so-called  "housemaid's  knee"  and  "miner's 
elbow."  Suppurative  bursitis  may  extend  to  the  adjacent  tissues. 

Chronic  bursitis  takes  the  form  of  an  accumulation  of  fluid  in  the 
cavity — hydrops  or  hygroma  bursos.  The  exudation  is  at  first  viscid 
and  mucinous,  but  later  becomes  thinner  and  more  watery.  The 
swelling  may  be  as  big  as  an  apple  or  larger.  The  wall  of  the  bursa 
is  not  at  first  much  altered,  but  sooner  or  later  becomes  thickened,  or 
may  be  covered  with  shaggy  fibrous  outgrowth  or  even  cartilaginous  or 
bony  plates  and  excrescences.  Frequently  "rice-bodies"  are  observed. 
In  some  cases  these  may  be  so  large  and  numerous  that  the  sac  is  filled, 
and  on  palpation  gives  rise  to  a  peculiar  crepitus — ganglion  crepitans. 

Tuberculous  bursitis  is  primary  or  secondary.  The  wall  of  the  sac 
becomes  thickened  and  infiltrated  with  tuberculous  granulomas,  while 
the  surface  is  covered  with  fungous  granulations.  There  is  usually 
considerable  exudate  (hygroma  tuber  culosum). 

Tumors  of  the  bursse  are  rare.  Sarcoma  and  fibroma  have  been 
described. 


SECTION  X. 
THE  OSSEOUS  SYSTEM. 


CHAPTEE    XLIII. 

THE  BONES,  JOINTS,  AND  CARTILAGES. 

THE  BONES. 

INTRODUCTORY. 

THE  bony  skeleton  subserves  two  important  purposes  in  the  organism. 
It  affords  a  more  or  less  rigid  scaffolding  for  the  support  of  the  organs 
and  soft  tissues  generally,  and  contributes  to  the  important  function  of 
locomotion,  with  the  secondary  circulatory  and  respiratory  functions 
which  this  mechanical  act  connotes.  Bone  is,  perhaps,  the  most  resistant 
and  indestructible  tissue  in  the  body,  but,  in  spite  of  its  hardness  and 
apparent  solidity,  it  is  not  the  permanent,  unchangeable  structure  that  one 
would  at  first  sight  suppose.  Like  other  tissues  it  exhibits  the  various 
anabolic  and  katabolic  processes  that  are  characteristic  of  all  vital 
forces.  Breaking  down  and  building  up  are  continually  going  on 
even  in  health,  and  may  be  much  exaggerated  and  perverted  under 
various  pathological  conditions. 

Bone  is  normally  produced  in  two  ways.  It  is  derived  from  osteo- 
genetic  centres  within  a  but  slightly  differentiated  connective-tissue 
matrix  (intramembranous  formation)  or  from  cartilage  (endochondral 
formation).  The  first  mode  of  origin  is  well  exemplified  in  the  case  of 
the  calvarium,  and  the  second,  in  the  spinal  column  and  long  bones 
generally. 

In  the  intramembranous  form  of  osteogenesis,  bony  spicules  con- 
taining lime  salts,  together  with  bone-corpuscles  and  cells,  are  formed 
within  a  proliferation-tissue  consisting  partly  of  cells  and  partly  of  a 
more  or  less  perfectly  developed  homogeneous  or  fibrillar  ground  sub- 
stance. These  bony  masses  gradually  increase  in  size,  and,  when  they 
finally  coalesce  to  form  plates,  gain  in  thickness  through  the  activity  of 
the  external  connective-tissue  layer,  which  is  henceforth  known  as  the 
periosteum.  Much  in  the  same  way,  the  earliest  manifestations  of 
ossification  begin  in  the  preexisting  cartilage  that  is  eventually  to 
become  the  bony  skeleton.  From  certain  regions  in  the  surrounding 
fibrous  investment  or  perichondrium  bony  processes  are  formed  which 


1004  THE  BONES 

gradually  extend  inward.  This  method  of  growth  from  the  peri- 
chondrium,  or  from  what  later  becomes  the  periosteum,  is  found  to 
some  extent  throughout  life,  and  accounts  more  especially  for  the 
increase  in  thickness  of  bone. 

Besides  this,  there  is  what  is  called  the  endochondral  ossification,  which 
is  brought  about  by  the  marrow  tissue  of  the  primitive  bone  proliferating 
and  invading  the  calcifying  cartilage,  which  it  extensively  destroys.  As 
soon  as  the  marrow  spaces  begin  to  appear,  endochondral  bone  forma- 
tion proper  commences.  In  the  neighborhood  of  the  medullary  spaces, 
where  they  are  bounded  by  the  solid  cartilage,  the  cartilage  cells  enlarge 
and  proliferate,  forming  small  clusters,  which  ultimately  become  arranged 
in  rows  parallel  to  the  long  axis  of  the  bone  that  is  to  be.  It  is  through 
the  proliferation  of  these  cells  that  the  increase  in  the  length  of  bone  is 
brought  about.  When  the  cartilage  cells  have  attained  their  full  size, 
there  occurs  a  deposit  of  lime  salts  in  the  ground  substance  and  the 
capsules  of  the  cartilage  cells.  In  this  way  is  formed  a  narrow  line  of 
demarcation  in  the  deeper  layers  of  the  intermediate  cartilage,  which, 
however,  does  not  remain  perfect,  inasmuch  as  the  vascularized  marrow 
gradually  dissolves  it  away  in  places  and  penetrates  through  it,  leaving 
only  islets  of  cartilaginous  ground  substance  and  calcified  tissue.  These 
are,  in  time,  converted  into  bone  proper  through  the  agency  of  certain 
cells  derived  from  the  bone-marrow,  called  osteoblasts.  The  cartilage 
in  this  way  is  gradually  replaced  by  bone. 

If  one  examines  a  growing  bone  after  it  is  completely  blocked  out, 
the  following  condition  of  things  will  be  found.  The  shaft,  or  diaphysis, 
is  completely  ossified.  At  the  end  is  the  cartilaginous  plate  called  the 
epiphysis.  Between  the  diaphysis  and  the  epiphysis  is  the  so-called 
intermediate  cartilage.  The  epiphysis  contains  within  it  more  or  less 
complete  centres  of  ossification.  The  intermediate  cartilage  is  com- 
posed of  two  layers,  a  bluish,  translucent  one,  the  zone  of  proliferation, 
and  another  of  a  thin,  opaque,  yellowish-white  appearance,  the  zone  of 
calcification.  The  centre  of  ossification  in  the  epiphyses  gradually 
enlarges  until  the  structure  is  completely  transformed  into  bone.  As 
soon  as  the  shaft  and  the  epiphysis  are  firmly  united  into  a  bony  mass 
growth  in  the  length  of  the  bone  ceases.  This  normally  occurs  between 
the  ages  of  twenty  and  twenty-seven,  but  under  certain  pathological 
conditions  may  take  place  prematurely,  or,  again,  may  be  delayed. 
Similarly,  the  synostosis  of  a  synchondrosis  or  suture  arrests  further 
growth  at  that  point.  Synostosis  may  be  delayed  or  may  occur  in 
places  where  ossification  does  not  normally  take  place.  In  the  case  of 
the  skull  premature  ossification  of  one  or  more  sutures  may  be  found  and 
leads  to  various  forms  of  asymmetry  and,  in  extreme  conditions,  to  micro- 
cephaly. Premature  synostosis  of  the  intersphenoid  and  sphenobasilar 
synchondroses  causes  shortening  of  the  base  of  the  skull  and  a  deeply 
set  nose.  This  is  to  be  observed  in  cretinism  and  some  forms  of  chondro- 
dystrophia.  Premature  synostosis  of  both  sacro-iliac  synchondroses 
leads  to  a  uniform  contraction  of  the  pelvis ;  of  one,  to  an  obliquely  con- 
tracted pelvis. 


DWARFISH  1005 


ANOMALIES  OF  GROWTH  AND  DEVELOPMENT. 

These  are  numerous  and  important,  and  are  manifested  in  the  forms 
of  deficiency  or  excess  in  growth,  or  in  some  peculiarity  in  the  quality 
of  the  bone.  The  causes  that  bring  these  about  may  be  inherited,  or 
acquired  during  intra-uterine  or  extra-uterine  life.  As  a  rule,  imperfec- 
tions of  the  bones  are  associated  with  analogous  abnormalities  in  the 
soft  parts  connected  with  them,  although  exceptions  occur.  The  term 
applied  to  complete  defect  of  a  bony  or  other  structure  is  agenesy  or 
aplasia,  but  these  names  are  applied  sometimes,  though  incorrectly,  to 
a  less  extreme  degree  of  the  condition,  a  partial  deficiency.  This  is 
more  properly  styled  hypoplasia. 

Agenesy,  in  the  stricter  sense,  is  always  local,  and  is  found  usually  in 
the  calvarium  and  vertebral  arches,  less  frequently  in  the  extremities 
and  bodies  of  the  vertebrae.  The  skull,  as  a  whole,  may  be  practically 
absent,  as  in  acephaly,  or  partially  defective,  as  in  anencephaly,  cyclops, 
and  other  grave  malformations.  The  spinal  column  may  be  rudi- 
mentary, as  in  iniencephaly  and  spina  bifida.  or  one  or  more  of  the 
vertebrae  may  be  lacking.  The  sternum  may  be  cleft  into  two  longi- 
tudinally. The  distal  ends  of  the  radius,  tibia,  and  fibula  may  be 
absent  in  malformations  of  the  hands  and  feet.  Occasionally  the 
clavicles  are  absent,  or  the  fibula. 

In  another  class  of  cases  the  primitive  "anlage"  have  been  laid  down, 
so  far  as  we  know,  in  a  normal  manner,  but  the  structures  arising  from 
them  have  failed  to  reach  their  complete  development — hypoplasia. 
This  condition  may  be  local  or  general.  In  the  local  form  the  head  may 
be  abnormally  small  (one  form  of  microcephaly),  an  arm  or  a  leg  may  be 
deficient  in  size  (microbrachius,  micropus),  or,  again,  all  four  extremities 
may  be  affected  (micro melus).  In  such  cases  the  most  important  etio- 
logical  factor  seems  to  be  pressure  upon  the  foetus  from  a  contracted 
amnion  or  from  bands  or  adhesions,  although  it  is  not  impossible  that 
in  some  cases  the  primitive  "anlage"  may  be  at  fault. 

Dwarfism. — When  the  body  as  a  whole  is  diminutive  we  may  speak 
of  dwarfism  (microsomia,  nanosomia).  An  allied  condition  is  infantilism, 
with  which  dwarfism  is  usually,  but  not  invariably,  associated.  Dwarf- 
ism implies  a  diminutive  structure,  while  infantilism  connotes  delayed 
or  imperfect  function,  whereby  an  individual  retains  the  characteristics 
of  the  child  long  after  these  should  have  disappeared.  In  other  words, 
dwarfism  bears  the  same  relation  to  vegetative  growth  that  infantilism 
does  to  function. 

There  are  two  forms  of  dwarfism:  essential  dwarfism,  in  which  the 
individual  is  to  all  intents  and  purposes  normal  save  in  the  one  particular 
of  size,  and  symptomatic  dwarfism,  where,  in  addition  to  diminutive  size, 
there  are  evidences  of  more  serious  disturbance,  such  as  the  stigmata 
of  infantilism,  cretinism,  rickets,  syphilis,  cardiovascular  anomalies,  or 
actual  malformations  and  deformities.  In  the  combination  of  dwarfism 
and  infantilism,  to  which  the  name  ateleiosis  has  been  applied,  the 


1006  THE  BONES 

process  of  ossification  is  greatly  delayed,  and  the  genital  organs  are  im- 
perfect in  structure  and  function.  Perfection  may  in  time  be  reached, 
but  the  size  remains  small. 

The  most  important  etiological  factors  in  the  production  of  essential 
dwarfism  are  peculiarities  of  the  germinal  cells,  which  may  be  inherited 
or  acquired  de  novo.  In  the  latter  case,  tuberculosis,  alcoholism,  and 
syphilis  appear  to  play  a  part.  Intra-uterine  malnutrition  of  the  foetus, 
due  to  hardship  or  ill-health  on  the  part  of  the  mother,  and  anomalies 
of  placentation  should  also  be  mentioned  in  this  connection.  Circula- 
tory disturbances  and  disorders  of  internal  secretion  in  the  foetus  itself 
are  of  importance.  Virchow  long  ago  pointed  out  the  close  connection 
between  infantilism  and  cardiovascular  hypoplasia,  and  more  recently, 
Gilbert  and  Rathery1  have  noted  a  tendency  to  dwarfism  in  certain  cases 
of  mitral  stenosis.  The  dependence  of  some  forms  of  dwarfing  on 
athyroidism  is  also  well  recognized.  According  to  Kiister,  Eiselsberg,2 
and  others,  removal  of  the  thyroid  in  growing  animals  is  followed  by 
inhibition  of  growth,  and  defect  of  the  thyroid  secretion  is  now  generally 
admitted  to  be  the  cause  of  that  interesting  congenital  affection  called 
cretinism,  in  which  stunting  of  the  growth  is  a  conspicuous  feature. 
Inferentially,  athyroidea  may  be  a  factor  in  the  production  of  the  true 
dwarfs. 

In  secondary  dwarfism,  in  addition  to  general  hypoplasia,  there  are 
evidences  of  disease  or  malformation,  and  the  changes  in  the  tissues  are 
qualitative  as  well  as  quantitative.  Many  cases  of  this  type  have  been 
regarded  as  syphilis,  rickets,  or  cretinism,  and  the  lines  of  demarcation 
have  not  always  been  closely  drawn.  In  fact,  the  exact  nature  of 
these  conditions  is  one  of  the  most  difficult  problems  in  etiology,  and 
further  study  is  still  needed  to  finally  clear  up  the  subject.  We  have  to 
consider  in  this  connection  the  affections  known  as  cretinism,  rachitis, 
chondrodystrophia  fcetalis,  osteogenesis  imperfecta,  and  osteopsathy- 
rosis. 

Cretinism. — In  cretinism  the  head  is  usually  large,  the  vertex  flattened, 
and  the  occiput  prominent.  The  fontanelles  and  sutures  remain  open 
for  a  long  time.  The  nose  is  retracted  at  the  root  and  is  short  and  thick, 
with  large,  wide  nostrils.  The  lips  and  tongue  are  enlarged.  The  teeth 
appear  late,  and  the  first  dentition  usually  persists  throughout  life.  The 
limbs  and  trunk  are  disproportionate  and  the  stature  is  stunted.  Maffei, 
in  22  cases  out  of  25,  found  the  height  to  be  less  than  140  cm.,  while 
several  were  under  95.  The  hair  on  the  pubes  and  in  the  axillae  is  scanty 
or  absent,  and  the  sexual  organs  are  poorly  developed.  Puberty,  if  it 
occur  at  all,  is  late. 

The  disturbance  is  associated  with  delayed  ossification  of  the  cartilages. 
Hofmeister,3  studying  a  case  with  the  oxrays,  found  that  the  epiphyseal 
ends  of  the  bones  grew  slowly,  while  the  epiphyseal  plates  persisted  for  a 
long  time.  Periosteal  ossification  may  be  normal  or  in  excess. 

1  Presse  m<§d.,  May  7,  1900.  2  Langenbeck's  Archiv,  49:  1895:  207. 

3  Fortschr.  auf  dem  Gebiete  der  Rontgen-Strahlen,  1 ;  1897, 


RACHITIS 


1007 


Microscopically,  Langhans1  found  that  at  the  ends  of  the  bones  the 
cartilage  cells  were  small,  spindle-shaped,  and  anomalously  arranged, 
being  longitudinal  to  the  long  axis  of  the  columns.  The  rows  were  inter- 
rupted and  irregular.  The  bony  trabeculae  were  shortened  and  the 
marrow  spaces  in  the  youngest  portions  of  the  bones  were  large  and 
widely  separated. 

Cretins  may  continue  to  grow  until  they  are  thirty  to  forty  years  of 
age,  and  ossification  may,  in  time,  be  completed.  The  cause  of  the 
disorder,  namely,  defect  of  the  thyroid  secretion,  may  be  due  to  a  variety 
of  pathological  conditions,  such  as  atrophy,  fibrosis,  or  cystic  degenera- 
tion of  the  gland. 


FIG.  274 


FIG.  275 


Sporadic  cretinism. 


Rickets.  Fractured  clavicles :  prominence 
of  frontal  eminences;  Harrison's  groove; 
and  pot-belly.  %  (Dr.  A.  E.  Vipond's^  case.)  * 


Rachitis. — The  exact  place  which  rachitis  or  rickets  should  occupy 
in  the  scheme  of  pathology  is  still  somewhat  debatable.  The  condition 
is  thought  by  some  to  be  due  to  infection  or  auto-intoxication,  and,  there- 
fore, to  some  extent  is  possibly  inflammatory  (Kassowitz2).  This  view 
is  somewhat  supported  by  the  character  of  the  lesions  in  the  bones,  and 


1  Virch.  Archiv,  128:  1892:  318. 


2  Zeit.  f.  klin.  Med.,  7: 1884:  36. 


1008  THE  BONES 

by  the  experimental  work  of  Morpurgo,1  who  showed  that  rachitic  changes 
could  be  produced  in  young  white  rats  by  the  injection  of  a  certain  dip- 
lococcus.  The  condition  is,  however,  so  closely  dependent  on  malnu- 
trition, brought  about  by  improper  diet,  overcrowding,  and  general 
unhygienic  surroundings,  that  in  default  of  more  information  we  may 
perhaps  be  justified  in  regarding  it,  for  the  present,  simply  as  a  dis- 
order of  growth.  Rickets  probably  is  not  hereditary,  although  it 
cannot  be  denied  that  intra-uterine  influences  may  play  a  part  to  a 
limited  extent. 

The  disease  usually  makes  its  appearance  after  the  sixth  month,  during 
the  first  or  second  year.  The  lesions  are  characteristic,  and,  if  severe, 
lead  to  serious  deformity  and  stunting  of  the  growth.  The  skull  is  large. 

FIG.  276 


Craniotabes  in  the  newborn  child.     Supposed  cause,  rickets.     (From  the  Pathological 
Museum,  McGill  University.) 

although  the  face  is  relatively  small.  The  forehead  is  square  and  promi- 
nent, owing  to  the  presence  of  flat  hyperostoses  on  the  frontal  eminences. 
The  sternum  projects,  while  the  sides  of  the  thorax  are  drawn  in  (pectus 
carinatum).  The  abdomen  is  protuberant.  The  spine  is  often  curved 
and  the  extremities  greatly  deformed,  owing  to  the  weight  of  the  body 
and  muscular  traction  acting  on  the  imperfectly  calcified  bone.  The 
pelvis  is  deformed  and  contracted.  Dentition  is  delayed  and  the  teeth 
are  small  and  badly  formed.  In  the  milder  cases  deformities  are  not  so 
extensive,  but  swellings  at  the  ends  of  the  long  bones  and  at  the  costo- 
chondral  junctions  (rachitic  rosary}  are  a  noteworthy  feature.  Periosteal 

1  Centralbl.  f.  Path.,  13:  1902:  113. 


RACHITIS  1009 

as  well  as  epiphyseal  bone-formation  is  interfered  with.  The  periosteum 
is  readily  stripped  off  and  the  underlying  bone  is  softer  and  more  spongy 
than  normal.  The  epiphyseal  zone  of  proliferation  is  thicker  than 
usual,  irregular  in  outline,  soft,  and  hyperemic. 

The  pathological  changes  may  be  summed  up  in  the  statement  that 
there  is  excessive  absorption  of  the  bone  with  impairment  of  the  process 
of  calcification.  The  normal  process  of  lacunar  bone-absorption  is 
much  exaggerated,  so  that  the  solidity  of  the  greater  part  of  the  skeleton 
is  more  or  less  destroyed.  The  external  denser  layer  of  the  bones  be- 
comes osteoporotic  and  the  trabeculse  of  the  spongiosa  are  attenuated  and 
may  even  disappear.  This  is  well  seen  in  the  bones  of  the  skull,  which 

FIG.  277 


Rickets.  The  section  is  taken  longitudinally  at  the  junction  of  a  rib  with  a  costal  cartilage. 
The  irregularity  of  the  columns  of  cartilage  cells  and  the  zone  of  congestion  are  easily  seen. 
Zeiss  obj.  A,  without  ocular.  (From  the  collection  of  Dr.  Oskar  Klotz.) 

sometimes  become  soft  and  give  way  under  the  pressure  of  'the  finger 
(craniotabes).  Deformities  and  even  fractures  may  thus  be  brought 
about.  Not  only  this;  there  is  quite  early  a  formation  of  new  bone  which 
is  poor  in  or  quite  devoid  of  lime  salts  (osteoid  tissue).  This  is  laid  down 
in  the  neighborhood  of  the  remains  of  the  trabeculse,  and  may  also  form 
new  trabeculae.  The  newly-formed  osteoid  tissue  is  derived  from  the 
periosteum  and  the  bone-marrow,  which  are  extremely  vascular.  The 
proliferating  tissue  is  composed  not  only  of  epithelioid  osteoblastic  cells, 
but  also  of  spindle  and  stellate  cells  within  a  fibrillar  matrix.  When 
arising  from  the  marrow  the  new  substance  is  strictly  comparable  to 
the  internal  callus  found  in  recent  fractures.  The  trabeculse  derived 
from  the  periosteum  are  formed  in  much  the  same  way  also  as  the 
64 


1010  THE  BONES 

outer  callus,  and  are  composed  of  a  cellular  or  cellulofibrous  material, 
In  the  periosteum  of  the  long  bones  cartilage  is  often  laid  down,  which 
in  turn  undergoes  the  characteristic  transformation.  In  this  way  it 
comes  about  that  the  external  surface  of  the  bones  is  covered  with  a 
vascular  spongy  layer,  which  offers  some  resistance  to  the  finger,  but  is 
readily  cut  with  the  knife.  In  extreme  cases,  the  original  compact  bone 
becomes  porotic. 

*  The  endochondral  ossification  is  also  seriously  interfered  with.  In 
the  more  marked  cases  there  may  be  no  deposit  of  lime  salts  in  the  zone 
of  calcification,  and  in  the  milder  forms  there  are  merely  spicules  scattered 
here  and  there.  At  the  same  time  there  never  fails  to  be  an  enlargement 
of  the  proliferation  zone  of  the  cartilage,  manifested  principally  in  the 
long  columns  of  hypertrophic  cartilage  cells.  With  this,  vascular  marrow 
spaces  are  formed  here  and  there  which  gradually  encroach  upon  the 
solid  cartilage.  The  more  severe  the  disease,  the  more  marked  is  the 
absorption  of  the  cartilage.  Somewhat  behind  the  zone  of  hypertrophic 
and  vascularized  cartilage  a  zone  of  osteoid  tissue  is  formed,  which  may 
be  from  5  to  15  mm.  in  thickness.  As  the  disease  heals,  lime  salts  are 
deposited,  beginning  in  the  central  portions  of  the  trabeculae  of  osteoid 
substance  and  gradually  extending,  until  the  bone  becomes  solid  again, 
and  even,  in  fact,  much  more  dense  and  ivory-like  than  normal  bone. 

Chondrodystrophia  Foetalis. — Somewhat  allied  to  cretinism  and  rickets, 
at  least  in  outward  appearance,  is  the  affection  known  as  chondrodys- 
trophia  fcetalis  (Kaufmann)  or  achondroplasia  (Parrot).  We  owe  our 
knowledge  of  this  disease  chiefly  to  the  researches  of  Kaufmann,1 
whose  work  has  enabled  us  to  differentiate  it  from  a  variety  of  other 
conditions  with  which  it  was  formerly  confused.  The  obscurity  involv- 
ing the  condition  even  yet  is  well  indicated  by  the  numerous  names  that 
have  been  proposed  for  it:  namely,  rachitis  foetalis,  pseudorachitism, 
pseudorachitis  foetalis  micromelica,  cretinoid  dysplasia,  chondrodys- 
trophia  fcetalis,  achondroplasia,  chondritis  foetalis. 

The  disease  begins  in  fcetal  life,  running  its  course,  as  it  is  believed, 
from  the  third  to  the  sixth  week.  Consequently,  the  parts  chiefly  affected 
are  the  base  of  the  skull,  the  long  bones,  ribs,  and  pelvis.  The  bones 
that  are  formed  in  membrane  and  those  that  in  late  fcetal  life  are  mainly 
cartilaginous  commonly  escape.  The  individuals  thus  affected  are  usually 
stillborn  but  may  survive  to  adult  life. 

In  a  typical  case  the  body  as  a  whole  is  dwarfed,  the  type  being  micro- 
melic,  and  the  lesions  are  generally  symmetrical.  The  head  is  large  and 
the  trunk  plump,  approximating  normal  size.  The  micromelia  is  rhizo- 
melic,  and  the  hands  exhibit  what  is  called  the  "trident"  deformity. 
There  are,  however,  notable  variations  in  the  configuration  of  the  skull, 
and  in  the  length,  curvature,  and  consistence  of  the  bones  of  the  extremi- 
ties. In  regard  to  the  first-mentioned  particular,  Kaufmann  recognizes 
two  main  groups :  one  in  which  there  is  a  cretinoid  conformation  of  the 

1  Untersuchungen  iiber  die  sogennante  foetale  Rachitis,  Berlin,  1892,  and  Ziegler's 
Betirage,  13:  1893:  32. 


PLATE   XIV 


Osteogenesis  Imperfeeta,  with  Multiple  Antenatal  Fractures  of 
Ribs,  Long  Bones,  etc. 


The  fractures  show  themselves  as  nodosities  with   abnormal 
curvatures  of  the   bones. 


OSTEOGENESIS  IMPERFECT  A  1011 

head,  that  is  to  say,  a  deeply  sunken  nose,  prominent  eyelids  and  lips, 
thick  cheeks,  and  a  large  mouth;  and  a  second,  in  which  the  nose  is  flat- 
tened and  retracted  as  a  whole.  In  the  former  group  the  bone  is  of  good 
consistence,  although  somewhat  more  vascular  than  normal,  while  in  the 
latter  its  texture  is  soft.  Some  cases  are  associated  with  craniotabes 
(Klein),  and  there  may  be  beading  at  the  costochondral  sutures.  Defec- 
tive development  of  the  pelvis,  cotyloid  and  glenoid  cavities,  has  also  been 
noted.  The  vertebrae  may  be  involved  (Regnault),  so  that  lordosis 
results.  Growth  in  the  length  of  the  bones  is  seriously  interfered  with, 
owing  to  faulty  ossification  at  the  epiphyseal  lines,  but  periosteal  ossifi- 
cation is  practically  normal,  with  the  result  that  the  bones  become  short, 
plump,  and  thick.  An  ingrowth  of  the  periosteum  into  the  epiphyseal 
sutures  has  been  observed  in  many  cases. 

According  to  the  character  of  the  changes  in  the  long  bones,  Kauf- 
mann  differentiates  three  forms — chondrodystrophia  malacica,  c.  hypo- 
plastica,  and  c.  hyperplastica.  In  the  first  variety  the  cartilages  at  the 
ends  of  the  long  bones  are  to  some  extent  increased  in  size,  but  no 
columns  of  cartilage  cells  are  formed.  The  cartilage  in  places  is  softened 
and  in  others  is  irregularly  calcified  and  ossified.  In  the  hypoplastic 
form  the  ossification  of  the  cartilage  is  appreciably  behind  the  normal  in 
extent,  the  columns  of  cartilage  cells  are  small,  the  cells  themselves  are 
deficient  in  growth,  being  spindle-shaped,  irregularly  arranged,  and  hav- 
ing the  appearance  of  being  compressed.  The  hyaline  matrix  is  more  or 
less  soft  and  homogeneous.  The  third  type  presents  a  marked  over- 
growth of  the  cartilage  leading  to  notable  thickening  at  the  epiphyseal 
ends  of  the  bones.  Ossification  is  extremely  irregular. 

The  etiology  of  this  disease  is  quite  obscure,  and  it  is  by  no  means 
certain  that  Kaufmann's  three  types  are  different  phases  of  the  one 
affection.  Heredity  seems  to  play  a  part  in  some  cases.  There  is  some 
evidence  for  believing  that  the  cretinoid  variety  is  really  a  form  of  cretin- 
ism and  due  to  athyroidea.  With  regard  to  the  other  types,  it  is  possible 
that  defects  in  the  primitive  "anlage"  are  at  fault,  or,  again,  intra-uterine 
pressure  (Klebs1).  In  view  of  the  fact  that  obvious  errors  in  develop- 
ment are  sometimes  associated  with  chondrodystrophia,  as,  for  instance, 
polydactylism  and  cleft  palate,  and  that  the  condition  imperceptibly 
shades  off  into  a  pronounced  developmental  anomaly  (Missbildung), 
which  is  finally  represented  by  phocomelia,  Virchow  has  objected  to  the 
term  chondrodystrophia,  which  is  certainly  open  to  criticism  in  that  it 
localizes  the  disturbance,  and,  moreover,  attributes  it  to  a  nutritional 
defect. 

Osteogenesis  Imperfecta. — The  exact  relationship  of  the  disease,  called 
by  Vrolik  and  Stilling,  osteogenesis  imperfecta,  to  chondrodystrophia 
is  still  sub  judice.  The  studies  of  Stilling,2  Hildebrandt,3  and  Harbitz4 
go  to  show  that  it  is  a  definite  intra-uterine  process.  Although  the 
affection  has  been  described  only  in  newborn  or  very  young  infants,  it 

1  Lehrbuch  d.  allg.  Path.,  Jena,  2.  2  Virch.  Archiv,  115: 1899: 357. 

3  Virch.  Archiv,  158:  1899:  426.  4  Ziegler's  Beitrage,  30:  1901:  605. 


1012 


THE  BONES 


is  not  necessarily  fatal,  and  Harbitz  suggests  that  certain  of  the  cases  of 
dwarfism  that  have  been  regarded  as  foetal  rickets  may  have  been 
osteogenesis  imperfecta.  There  is,  undoubtedly,  some  defect  in  the 
process  of  ossification,  inasmuch  as  the  bones  are  soft  and  brittle,  so 
that  deformities  and  fractures  readily  occur.  Like  chondrodystrophia, 
porosity  of  the  bones  is  occasionally  inherited,  and,  as  Bircher1  has 
shown,  the  two  conditions  may  be  combined.  It  is  interesting,  also,  that 
borderland  cases,  presenting  in  one  and  the  same  individual  some  of  the 
features  of  chondrodystrophia,  osteogenesis  imperfecta,  and  rickets,  have 
been  recorded  (Hektoen2). 


FIG.  278 


Sir 


<"?V 


Fragilitas  ossium.  Section  is  taken  transversely  through  the  femur  of  a  newborn  infant  with 
chondrodystrophia  and  osseous  fragility.  To  the  right  is  periosteum  with  a  layer  of  cartilage; 
to  the  left  a  portion  of  the  shaft.  Bone-formation  is  deficient,  as  evidenced  by  the  scanty  and 
attenuated  trabeculse.  Zeiss  obj.  DD,  without  ocular.  (Case  of  Dr.  Oskar  Klotz.) 

In  osteogenesis  imperfecta  a  cardinal  feature  is  the  extraordinary 
manner  of  ossification  of  the  skull.  The  calvarium  is  not  formed  of 
continuous  bony  plates  with  regular  sutures,  but  of  a  multitude  of  small 
mosaics,  sometimes  touching  one  another,  but  more  often  united  by 
bridges  of  membrane.  In  a  remarkable  case,  described  by  Stilling,  the 
vault  of  the  skull  consisted  of  a  membranous  sac  in  which  were  scattered 
spicules  of  bone.  So  far  as  is  known,  synostosis  of  the  os  tribasilare  does 
not  occur  in  this  disease. 

Microscopic  study  shows  that  the  trabeculse  are  few  in  number, 
irregular,  and  imperfectly  formed.  There  is  no  continuous  system  of 


1  Lubarsch  u.  Ostertag's  Allg.  Aetiol.,  Wiesbaden,  1896:  53. 

2  Amer.  Jour.  Med.  Sci.,  125:  1903:  751. 


GIG  ANT  ISM  1013 

trabeculse  with  Haversian  canals,  and  lamellae  as  in  normal  bone.  The 
cause  is  absolutely  unknown.  In  one  case  hydramnios  was  present  in 
the  mother. 

As  has  been  mentioned,  the  condition  of  brittleness  of  the  bones, 
osteopsathyrosis  or  fragilitas  ossium,  is  an  occasional  feature  in  the 
curious  affections  just  described,  but  is  sometimes  found  as  a  distinct 
entity.  The  exact  connection  between  osteopsathyrosis  and  osteogenesis 
imperfecta  is  by  no  means  clearly  made  out.  As  Gurlt1  has  observed, 
the  condition  is  occasionally  inherited.  A  remarkable  instance  is  on 
record  also  of  dwarfism  and  osseous  fragility  occurring  in  the  same 
family  throughout  three  generations  (Ekmann2). 

The  sum  total  of  these  collective  studies  serves  to  show  that  there  are 
a  number  of  anomalies  of  growth  and  development,  more  or  less  distinct, 
but  shading  off  imperceptibly  one  into  the  other.  That  there  is  such  a 
thing  as  "fcetal  rickets,"  in  the  sense  of  rickets  that  has  run  its  complete 
course  during  intra-uterine  life,  may  well  be  doubted.3 

Gigantism. — In  many  respects  the  antithesis  of  dwarfism  is  the 
remarkable  condition  known  as  gigantism,  or  more  correctly  macro- 
genesy,  in  which  there  is  a  notable  increase  in  the  length  and  thickness 
of  the  bones,  with  concomitant  changes  in  the  soft  tissues.  We  have 
here  evidences  of  increased  proliferation  of  the  cartilages  in  the  process 
of  endochondral  ossification,  together  with  excessive  deposit  of  bone 
by  apposition.  The  condition  may  be  congenital  or  may  develop  in 
later  life. 

Partial  or  Local  Gigantism. — Local  forms,  affecting  chiefly  the  bones  of 
the  face  (leontiasis  ossea,  Virchow),  the  fingers,  and  toes,  have  been 
observed.  The  exact  nature  of  leontiasis  ossea  is  obscure,  but  it  appears 
to  be  a  diffuse  hyperostosis,  somewhat  analogous  to  the  local  exostoses 
and  hyperostoses  found  in  certain  degenerative  disturbances  and  chronic 
inflammations.  The  local  gigantism  of  childhood  affects  usually  the 
upper  limbs,  and  may  be  unilateral  or  bilateral.  Other  developmental 
anomalies  may  be  associated  with  the  overgrowth.  Thoma,4  for  example, 
has  observed  defective  formation  of  the  genital  organs  in  a  youth  the 
subject  of  hemihypertrophy. 

General  Gigantism. — When  the  body  as  a  whole  is  hypertrophic  we 
speak  of  general  gigantism  or  macrosomia. 

This  is  excessively  rare  as  a  congenital  anomaly.  According  to  the 
law  of  "deviation"  formulated  by  Thoma,5  we  can  assume  the  existence 
of  giant  growth  in  cases  where  the  length  of  the  body  exceeds  57  cm.  and 

1  Handb.  der  Lehre  von  den  Knochenbruchen,  1  :  1862  :  147. 

2  Dissertatio  inedica  descriptionem  et  casus  aliquot  osteomalacise  sistens,  Upsalae, 
1788. 

3  Those  interested  will  find  the  subject  more  fully  discussed  in  an  article  by  one 
of  us  (A.  G.  N.)  in  the  Reference    Handbook  of  the   Medical  Sciences  (2d  edit.) 
8:1904:419,  N.  Y.,  Wm.  Wood  &  Co. 

4  Text-book  of  General  Pathology,  London,  1 : 198,  A.  &  C.  Black. 

5  Untersuchungen  liber  die  Grosse  u.  das  Gewicht  der  anatomischen  Bestandtheile 
des  menschlichen  Korpers  im  gesunden  u.  im  kranken  Zustande,  Leipzig,  1882. 


1014  THE  BONES 

the  weight  is  above  4f  kg.  The  largest  newborn  infant  on  record  is  one 
reported  by  Dubois,1  which  weighed  11.3  kilos  (twenty-four  pounds 
thirteen  and  one-half  ounces).  As  a  rule,  giant  children  are  stillborn. 
When  they  survive,  the  excessive  size  may  be  compensated  by  slow 
growth  subsequently,  but  occasionally  such  infants  grow  very  fast  and 
attain  puberty  early. 

More  often,  however,  general  macrosomia  first  makes  its  appearance 
between  the  tenth  and  the  twentieth  year,  usually  with  the  onset  of 
puberty.  The  abnormality  manifests  itself  chiefly  by  an  increase  in 
the  length  of  the  long  bones,  mainly  of  the  lower  extremities,  but  to 
some  extent  in  the  trunk,  so  that  the  height  is  notably  increased.  The 
increase  in  weight  is  less  apparent,  being  never  more,  and  usually  less, 
than  the  increase  in  height  would  warrant.  In  giants  the  head  is  rela- 
tively small  and  the  overgrowth  is  disproportionate.  Stigmata  of  infantil- 
ism, such  as  knock-knee  and  genital  hypoplasia,  may  be  present. 
Some  cases  are  associated  with  other  developmental  defects,  facial  hemi- 
hypertrophy,  local  exostoses,  or  curved  bones.  These  may  be  regarded 
as  examples  of  symptomatic  gigantism. 

Much  rarer  is  essential  gigantism,  in  which  the  overgrowth  is  uniform 
and  symmetrical,  and  the  affected  individuals  are  strong  and  in  every 
way  perfect. 

The  causes  of  gigantism  are  obscure.  It  has  been  attributed  by  some 
to  peculiarities  in  the  germ  cells.  This  view  is  supported  by  the  fact 
that  the  condition  is  sometimes  inherited,  and,  also,  by  the  observations 
of  Engel-Reimers,2  who  has  emphasized  the  view  that  excessive  muscular 
development,  the  so-called  "  athletic"  habit,  is  often  due  to  an  abnormal 
predisposition  and  not  to  functional  overactivity.  In  this  connection 
increased  intra-uterine  nutrition  appears  to  play  a  leading  role. 

Several  facts,  also,  would  indicate  that  irritation  or  excessive  stimulation 
of  the  epiphyseal  ends  of  the  bones  tends  to  produce  overgrowth.  This 
can  be  brought  about  experimentally  by  driving  ivory  pegs  into  the  ends 
of  the  bones  or  by  feeding  young  animals  with  phosphorus  or  arsenic 
(Wegner3).  Elongation  of  the  bones  has  also  been  noted  in  connection 
with  osteomyelitis,  fractures  of  the  shaft,  tuberculosis  of  the  joints, 
superficial  ulcers,  and  dilated  veins.  Local  hyperostoses  of  the  head 
have  been  known  to  follow  trauma  and  erysipelas. 

Giant  growth  also  has  been  met  with  after  the  acute  infective  fevers 
in  childhood,  which  were  apparently  the  exciting  cause. 

Other  cases,  again,  appear  to  be  dependent  on  disorders  of  internal 
secretion.  A  tendency  to  exaggerated  growth,  particularly  of  the  lower 
extremities,  has  been  observed  in  eunuchs  and  castrated  animals. 

There  is  considerable  ground  for  believing,  further,  that  a  large  pro- 
portion of  the  cases  of  gigantism  (42  per  cent,  according  to  Sternberg4) 

1  Les  gros  enfants  au  point  de  vue  obstetrical,  These  de  Paris,  1897. 

3  Jahrb.  der  Hamburg  Staatskrankenanstalten,  3:  1894. 

1  Ueber  den  Einfluss  des  Phosphors  au  den  Organismus,  Virch.  Archiv,  55: 1872: 11. 

4  Beitrage  zur  Kenntniss  der  Akromegalie,  Zeit.  f.  klin.  Med.,  27:  1894:  85;  ibid., 
1895:86. 


GIGANTISM  1015 

are  really  acromegaly.  Brissaud  and  Meige1  have  upheld  the  view  that 
the  two  affections  are  essentially  the  same  thing.  The  same  pathological 
process  at  work  during  the  growing  period  of  life  will  produce  gigantism ; 
at  a  later  period,  when  epiphyseal  ossification  is  completed,  acromegaly. 
It  is  possible  that  some  at  least  of  the  cases  of  gigantism  which  do  not 

FIG.  279 


Lateral  scoliosis.     (From  the  Surgical  Clinic  of  the  Montreal  General  Hospital.) 

present  the  characteristics  of  acromegaly  are  nevertheless  dependent  on 
abnormal  function  of  the  pituitary  body  (acrome'galie  fruste2). 

Besides  the  peculiarities  of  growth  and  development  just  considered, 

1  Jour,  de  me'd.  et  chir.  prat.,  January  25,  1895. 

2  For  a  full  discussion  of  the  general  subject  of  gigantism,  and  references  to  the 
literature,   see  Nicholls,   article  Gigantism,  Reference  Handb.  of  the  Med.  Sci., 
second  edition,  N.  Y.,  Wm.  Wood  &  Co.,  8  :  1904  :  457. 


1016  THE  BONES 

there  are  others  more  localized  which  are  brought  about"  by  abnormal 
static  and  mechanical  influences  exerted  upon  the  organism  during  the 
developmental  period  of  life.  These  may  arise  both  before  and  after 
birth  and  frequently  cause  most  pronounced  deformities. 

While  many  of  these  congenital  anomalies  are  probably  to  be  attributed 
to  peculiarities  of  the  germinal  cells,  others  are,  at  least  in  many  instances, 
more  closely  connected  with  intra-uterine  pressure.  Among  these  condi- 
tions may  be  mentioned  microcephaly,  micromelia,  the  fusion  of  bones, 

FIG.  280 


Scoliosis  with  marked  posterior  deformity  (kyphosis). 

polydactylism,  and  the  amputation  of  limbs.  The  factors  usually  at  work 
are  a  contracted  amniotic  sac,  bands  or  adhesions  traversing  the  sac,  the 
weight  of  superimposed  limbs,  or  a  knotted  umbilical  cord. 

To  a  large  extent,  also,  the  development  of  the  bony  cavities,  such  as, 
for  instance,  the  cranium,  the  orbits,  and  the  thorax,  is  dependent  upon 
the  state  of  development  of  the  contained  organs.  Should  these  be  small 
or  otherwise  defective,  the  corresponding  bony  parts  are  hypoplastic  or 
defective.  In  this  category  may  be  placed  such  conditions  as  cranioschisis, 
craniorachischisis,  anencephaly,  spina  bifida,  and  the  like.  Increased 


SCOLIOSIS 


1017 


pressure  within  the  bony  cavities  will  also  lead  to  enlargement  of  the 
same.  Thus,  hydrocephalus  is  an  enlargement  of  the  cranium,  often  with 
separation  of  the  bones  and  flattening  of  the  brain  substance,  caused  by 
excess  of  the  cerebrospinal  fluid. 

In  later  life,  too,  owing  to  external  influences,  bones  that  were  originally 
well-formed  and  developed  may  become  deformed,  and  this  the  more 
readily  occurs  should  the  bone  from  any  cause  become  soft  and  yielding. 

Scoliosis. — One  of  the  most  common  of  these  deformities  is  scoliosis,  or 
lateral  deviation  of  a  portion  of  the  vertebral  column.  As  a  rule,  there  is 
curvature  of  the  dorsal  spine  to  the  right,  with  a  compensatory  deviation  of 

FIG.  281 


Knock-knee.     (From  the  Surgical  Clinic  of  the  Montreal  General  Hospital.) 

the  lumbar,  and  sometimes  the  cervical,  portion  to  the  left.  This  may  be 
brought  about  by  excessive  distension  of  one  thoracic  cavity,  as  from  an 
exudate  or  new-growth,  or  by  contraction  of  the  cavity  from  fibroid  indu- 
ration of  the  lung,  the  inspissation  of  a  pleuritic  exudate,  or,  again,  by 
fixation  of  the  pelvis  in  an  oblique  position.  It  is  also  met  with  in  Fried- 
reich's  ataxia  and  the  progressive  myopathies.  Perhaps  the  commonest 
causes  are  faulty  methods  of  standing  or  sitting  in  childhood.  In  the  more 
severe  cases  the  deformity  comes  on  quickly  and  becomes  extreme,  being 
often  combined  with  posterior  curvature— kyphosis.  There  is  often  with 
this  more  or  less  rotation  of  the  vertebrae  on  their  vertical  axes.  The  liga- 
ments become  stretched  and  may  calcify,  owing  to  the  production  of  osteo- 
phytes.  Curvature  forward,  or  lordosis,  is  also  frequently  met  with. 


1018  THE  BONES 

Coxa  Vara. — Coxa  vara  is  a  condition  of  the  hip-joint  brought  about  by 
imperfect  development  of  the  acetabulum,  or  changes  in  the  head  or  neck 
of  the  femur.  It  is  met  with  where  there  is  abnormal  yielding  of  the 
bone,  as  in  rickets,  and  leads  to  dislocation. 

Genu  Valgum. — Genu  valgum  is  met  with  in  children  from  the  second 
to  the  fourth  year,  or  at  puberty.  In  this  condition  the  leg  forms  an 
obtuse  angle  with  the  thigh  at  the  knee-joint.  It  may  be  unilateral  or 
bilateral,  and  is  often  met  with  also  in  those  who  are  a  great  deal  on  their 
feet  and  who  do  heavy  manual  labor.  It  may  occasionally  be  due  to 
traumatic  separation  of  the  epiphyseal  cartilages  with  dislocation  of  the 
fragments  and  subsequent  union  in  the  faulty  position,  to  carious  processes 
in  the  external  condyle  of  the  femur,  or,  again,  to  arthritis  deformans. 

Talipes  Valgus. — Talipes  valgus  (pes  planus),  or  flat-foot,  is  a  condition 
in  which,  owing  to  the  stretching  of  the  internal  lateral  and  plantar  liga- 
ments, the  arch  of  the  foot  is  more  or  less  completely  destroyed.  In  severe 
cases  the  normal  concavity  on  the  under  side  of  the  foot  may  give  place 
to  actual  convexity.  The  result  is  that  the  foot  as  a  whole  is  turned 
outward,  the  soft  tissues  are  often  reddened  and  swollen,  while  more  or 
less  pain  is  complained  of.  The  trouble  is  usually  brought  about  by 
much  standing  or  by  carrying  weights,  in  the  case  of  those  whose  health 
is  not  robust.  It  may  also  be  caused  by  knock-knee  or  rachitic  disease 
of  the  ankle-joints.  Rarely  it  is  congenital. 

Hallux  Valgus. — Hallux  valgus  is  to  be  regarded  as  a  pressure  deformity 
due  to  the  use  of  improperly  fitting  boots.  The  great  toe  often  is  forced 
to  assume  a  position  beneath  the  other  toes. 

Abnormal  positions  of  the  articular  surfaces  of  bones  may  also  be 
brought  about  by  faulty  positions  of  the  limbs,  as  in  certain  forms  of 
paralysis,  contractures  of  neuropathic  origin,  or,  again,  by  the  contraction 
of  scar-tissue  in  fascia  or  tendons.  The  nature  of  talipes  varus,  t.  equinus, 
and  t.  calcaneus  is  sufficiently  indicated  by  their  names  and  calls  for  no 
extended  description. 

CIRCULATORY  DISTURBANCES. 

Hyperemia. — Active  Hyperemia. — Active  hyperemia  is  met  with 
in  the  periosteum  and  medulla  in  growing  bone,  and,  pathologically, 
in  various  forms  of  regeneration  and  inflammation. 

Passive  Hyperemia — Passive  hyperemia  is  found  in  general  venous 
stasis  and  whenever  the  free  outflow  of  blood  from  the  bone  is  inter- 
fered with.  Chronic  passive  congestion  appears  to  favor  the  growth  of 
the  bone  and  softjissues,  as  is  well  seen  in  the  clubbed  fingers  of  chronic 
pulmonary  and  cardiac  disease.  Whether  the  peculiar  disease,  first 
described  by  Marie,1  and  called  by  him  hypertrophic  pulmonary  osteo- 
arthropathy,  is  of  this  nature  cannot  at  present  be  settled. 

Hemorrhage. — Hemorrhage  into  the  periosteum  or  marrow  is  not 
very  uncommon.  It  rarely  attains  serious  proportions,  and  the  blood 

1  R6vue  de  M&Iecine,  10: 1890: 1. 


INFLAMMATIONS 


1019 


effused  is,  as  a  rule,  quickly  absorbed.  It  may  follow  traumatism, 
and  occurs  in  new-growths  and  other  destructive  processes.  The  so- 
called  cephalhematomata  are  localized  extravasations  of  blood  beneath 
the  periosteum,  usually  of  the  parietal  bones.  Rarely,  they  are  found 


FIG.  282 


Spatulate  (Hippocratic)  fingers  in  a  case  of  pulmonary  tuberculosis.    (From  the  Medical  Clinic 
of  the  Montreal  General  Hospital.) 

in  the  interior  of  the  bone,  leading  to  elevation  of  the  external  plate. 
Petechial  hemorrhages  are  found  also  in  the  hemorrhagic  diatheses, 
Barlow's  disease,  and  scurvy.  In  Barlow's  disease  the  periosteum  of  the 
long  bones  may  be  dissected  off  from  the  shafts  owing  to  the  extensive 
extravasation  of  blood. 

Embolism. — Embolism  as  a  rule  produces  little  effect  on  account  of 
the  abundant  anastomoses  that  are  present,  but,  according  to  Gussen- 
bauer,  end-arteries  are  to  be  found  at  the  ends  of  the  diaphyses,  obstruc- 
tion of  which  might  lead  to  ischsemic  necrosis. 

Thrombosis. — Thrombosis  of  the  vessels  is  common  after  trauma  and 
fractures,  and  in  the  neighborhood  of  hemorrhages,  inflammatory  and 
necrotic  processes.  It  is  of  no  practical  importance. 


INFLAMMATIONS. 


The  inflammatory  processes  occurring  in  bone  affect  first  and  chiefly 
the  vascular  structures,  that  is  to  say,  the  periosteum  and  bone-marrow. 
We  may  therefore  distinguish  two  forms:  periostitis  and  osteomyelitis. 


1020 


THE  BONES 


causes. 


FIG.  283 


The  compact  material  of  the  bone  may  be  involved  secondarily  in  either 
of  these  conditions  and  usually  in  the  form  of  erosion  and  disintegration. 
Periostitis    and    Osteomyelitis. — Periostitis    and    osteomyelitis   are 
not  infrequently  combined. 

Inflammation  of  bone  may  be  brought  about  by  local  or  systemic 
As  a  rule,  infective   microorganisms   are  at  work,  but   toxic 

agents  and  local  trauma  may  play  a 
contributory  and  sometimes  a  leading 

_  role.      The  causal  agents  reach  the 

^^^dfe.  ^^  affected  part  either  directly  or  through 

mi        ^  **$$&  *^e  kl°°d  stream.     The  most  charac- 

'-^  teristic   form  of   inflammation  is  the 

acute  osteomyelitis  and  periostitis  due 
to  pyogenic  cocci,  but  an  analogous 
condition  is  occasionally  met  with  as 
a  complication  of  some  of  the  com- 
mon infectious  fevers,  such  as  acute 
articular  rheumatism,  scarlatina, 
measles,  typhoid,  variola,  relapsing 
fever,  dysentery,  and  epidemic  paro- 
titis. Here  the  condition  may,  in 
some  cases,  be  due  to  the  action  of 
the  specific  microorganism  of  the 
primary  disease,  but  perhaps  more 
frequently  is  the  result  of  secondary 
or  mixed  infection  with  some  of  the 
pus-forming  cocci. 

The  results  are  various.  Mild 
grades  of  inflammation  and  those  that 
are  early  and  efficiently  treated  may 
heal  with  little  or  no  perceptible  effects. 
Much  more  frequently,  however,  the 
sequelae  are  serious.  These  may  be 
grouped  under  two  main  heads,  disin- 
tegration and  proliferation.  In  many 
forms  of  acute  and  chronic  inflam- 
mation necrosis  of  bone  is  a  marked 
feature,  either  in  the  form  of  a  slow 
eroding  process  of  resorption — caries 
— or  as  an  exfoliation  of  larger  or 
smaller  portions  of  the  bone  en  masse 
—sequestration.  Short  of  this  ex- 
treme result,  the  bone  may  become 
porotic,  owing  to  lacunar  resorption. 
This  results  from  the  destructive 

action  of  the  granulation  tissue  in  the  medullary  spaces  (rarefying 
osteitis).  In  other  cases,  on  the  contrary,  the  function  of  new  bone 
formation  appears  to  be  stimulated  in  some  way.  Thus,  in  the  neigh- 


Femur  cut  longitudinally,  to  show 
rarefaction,  osteosclerosis,  and  new-growth 
of  bone  from  the  periosteum.  Case  of 
osteomyelitis.  (Pathological  Museum, 
McGill  University.) 


ACUTE  INFECTIOUS  OSTEOMYELITIS  1021 

borhood  of  abscesses,  sequestra,  and  tuberculous  areas,  the  bone  becomes 
denser  and  more  compact,  owing  to  the  deposit  of  new  bone  upon  the 
trabeculse  (osteosderosis).  This  may  be  a  primary  condition  in  some 
cases  of  syphilis  and  phosphorus  poisoning.  Not  infrequently,  there  is 
a  new-formation  of  bone  in  the  shape  of  osteophytes,  or  a  diffuse  con- 
centric deposit,  owing  to  excessive  activity  of  the  periosteal  osteogenetic 
layer.  This  may  lead  to  increased  thickness  of  the  bone  and  to  more  or 
less  perfect  restoration  of  its  contour  after  extensive  destruction.  In 
growing  bones,  inflammation  of  the  shaft  of  the  bone,  provided  that  it 
be  not  too  severe  and  not  too  near  the  epiphyseal  cartilage,  may  result 
in  a  marked  increase  in  the  length  of  the  bone.  Should,  however,  the 
inflammatory  process  occur  near  or  at  the  zone  of  ossification,  irregularity 
in  growth  will  occur,  and  should  the  epiphyseal  cartilage  be  separated 
or  destroyed,  growth  will  come  to  a  standstill. 

It  is  hardly  necessary  to  state  that  inflammatory  affections  of  bones 
may  extend  to  the  adjacent  tissues  such  as  the  veins,  muscles,  tendons, 
aponeuroses,  and  skin.  In  this  way  local  abscesses  and  fistulse  are 
produced.  Thrombophlebitis  is  a  dangerous  complication,  inasmuch  as 
it  frequently  leads  to  a  dissemination  of  the  infective  agents  and  may 
thus  produce  systemic  septicemia. 

A  rather  peculiar  form  of  inflammation  of  the  periosteum  has  been 
described  by  Oilier  and  Berg.  The  disease  occurs  more  particularly  in 
young  persons  and  usually  runs  a  mild  course.  The  subperiosteal  exu- 
dation is  clear  and  serous,  or  sometimes  viscid,  containing  fibrin,  fat 
globules,  and  relatively  few  corpuscles.  It  may  be  combined  with  osteo- 
myelitis. The  exact  etiology  of  the  condition  is  not  as  yet  definitely 
made  out. 

Acute  Infectious  Osteomyelitis. — Acute  infectious  osteomyelitis  belongs 
to  the  group  of  septicemic  infections  and  occurs  spontaneously  or 
as  a  complication*  of  various  fevers.  The  spontaneous,  or  so-called 
"idiopathic,"  form  is  most  commonly  due  to  the  Staphylococcus  pyogenes 
aureus  or  albus,  and  is  met  with  usually  in  young  persons.  It  is  a  severe 
affection  characterized  by  great  pain,  fever,  and  symptoms  of  consti- 
tutional involvement.  The  infection  is  hematogenous,  the  original  point 
of  entrance  of  the  germ  being  usually  in  the  skin  or  mucous  membranes. 
The  primary  wound  may  have  been  so  trifling  that  all  trace  and  recollec- 
tion of  it  may  have  disappeared. 

The  process  begins  either  in  the  periosteum  or  in  the  medullary 
canal,  and  is  characterized  by  an  intense  suppurative  and  necrotizing 
inflammation.  The  disease  involves  usually  the  periosteum  and  the 
neighboring  parts.  The  affected  region  is  swollen,  tense,  reddened,  and 
intensely  painful.  The  bone-marrow  is  at  first  congested,  presenting, 
later,  areas  of  hemorrhagic  extravasation,  and  later  still,  numerous 
small  foci  of  suppuration.  Owing  to  the  confluence  of  these  foci  larger 
or  smaller  abscesses  result,  and  the  suppurative  process  may  extend 
throughout  the  medullary  canal  to  the  Haversian  canals  and  periosteum, 
and  even  to  the  surface.  Sometimes  the  epiphyses  and  the  joints  are 
involved.  In  milder  cases  the  process  may  terminate  without  such 


1022 


THE  BONES 


marked  disturbance,  but  usually,  owing  to  the  interference  with  the 
circulation  due  to  the  pressure  of  the  inflammatory  products,  larger  or 
smaller  portions  of  the  bone  may  become  necrosed  and  in  time  exfoliated. 
This  takes  place  both  within  the  bone  and  at  places  where  the  periosteum 
has  been  separated  from  the  underlying  structure.  Large  masses  of 
bone  and,  in  fact,  the  whole  central  portion  of  the  shaft,  may  be  thus 
sequestrated  and  necessitate  surgical  interference.  Owing  to  the  occur- 
rence of  septic  venous  thrombosis,  metastatic  abscesses  may  form  in 
various  parts  and  death  is  a  not  infrequent  result.  The  bones  are  in- 
volved primarily  in  the  following  order:  femur,  tibia,  the  bones  of  the 
upper  extremities,  the  flat  and  short  bones. 


FIG.  284 


Chronic  osteomyelitis  of  the  femur.  The  specimen  shows  great  thickening  of  the  shaft  of  the 
bone,  with  the  formation  of  abscesses.  On  the  left  a  sequestrum  is  well  shown.  (From  the 
Pathological  Museum  of  McGill  University.) 


In  cases  where  a  bone  is  injured,  as  by  crushing,  splintering,  or 
fracture,  a  moderate  amount  of  inflammation  is  set  up  which  may  readily 
heal.  Should,  howrever,  infective  microorganisms  be  circulating  in  the 
blood-stream  the  injured  region  is  liable  to  become  infected  and  a  condi- 
tion similar  to  infective  osteomyelitis  and  periostitis  is  produced  (locus 
resistentise  minoris).  In  cases  of  compound  fracture,  infection  may 
also  take  place  from  the  external  air. 


PAGET'S  DISEASE 


1023 


FIG.  285 


Chronic  Osteomyelits. — Chronic  inflammation  may  be  the  result  of 
a  preexisting  acute  process  or  may  be  chronic  from  the  start.  The 
latter  is  apt  to  be  the  case  in  tuberculosis,  syphilis,  typhoid,  and  actino- 
mycosis.  All  the  changes  just  described  as  occurring  in  the  acute  form 
may  occur  here,  but  the  process  is  more  gradual  and  long-continued. 
Granulation,  suppuration,  and  necrosis  are  marked  features  and  both 
osteoporosis  and  hyperostosis  occur.  Chronic  inflammation  may  also 
arise  by  the  extension  of  inflammatory  processes  from  the  neighboring 
parts,  as,  for  example,  from  ulcers  of  the  skin.  Osteophytes  may  be 
produced  or  diffuse  hyperostosis. 

A  peculiar  form  of  chronic  inflammation  that  should  be  here  mentioned 
is  the  phosphorus  necrosis  which  attacks  the  jaw  bones  of  those  working 
in  match  factories.  The  process  usually  begins  in  the  lower  maxilla 
and  may  extend  to  the  upper  jaw  and, 
rarely,  to  the  bones  of  the  face.  The 
condition  is  brought  about  by  the  fumes 
of  the  yellow  phosphorus,  which,  being 
dissolved  in  the  saliva,  attack  the  gums, 
and,  if  carious  teeth  be  present,  invade 
the  alveolar  processes.  Infection  with 
microorganisms  from  the  buccal  cavity 
also  plays  an  important  part.  In  the 
early  stages  there  is  a  slight  inflamma- 
tion, in  consequence  of  which  the  peri- 
osteum and  bone-marrow  are  stimulated 
and  produce  new  bone,  so  that  the 
jaw  becomes  thickened  and  sclerosed. 
Later,  suppuration  and  necrosis  set  in, 
and  larger  or  smaller  portions  of  the  bone 
are  sequestrated  and  cast  off.  In  this 
way  the  whole  of  the  lower  jaw  may 
be  destroyed.  Rarely,  the  process  runs 
a  much  more  acute  course. 

We  come  now  to  discuss  two  rare  and 
striking  affections  the  etiology  of  which 
is  quite  obscure. 

Paget's  Disease1  (Osteitis  Defor- 
mans). — Paget's  disease  is  a  rare  affec- 
tion found  after  middle  life  and  usually 
in  advanced  age.  In  brief,  there  are 
two  opposing  pathological  processes  at 
work,  resorption  and  osseous  hyperplasia. 
In  some  cases,  owing  to  the  absorption 
of  the  bone,  the  affection  bears  a  close 
resemblance  to  osteomalacia.  The  dis- 
ease begins  with  pains  of  a  rheumatic  character,  which  are  quickly 
followed  by  pathognomonic  changes.  The  bones,  especially  those  sub- 

1  Med.  Chir.  Trans.,  60: 1877:  37. 


Osteitis  deformans.     (Packard.) 


1024  THE  BONES 

jected  to  the  weight  of  the  body,  as  the  spine  and  leg  bones,  become 
curved  and  otherwise  deformed,  while  there  may  also  be  a  considerable 
and  irregular  hyperostosis.  The  parts  chiefly  affected  are  the  bones 
of  the  lower  extremities,  the  spine,  clavicles,  and  the  calvarium.  The 
process  of  resorption  goes  on  both  in  the  spongy  and  compact  parts  of  the 
bone,  and  leads  to  more  or  less  complete  destruction  of  the  bony  plates, 
which  are  replaced  by  a  fatty,  gelatinous,  or  fibrous  tissue,  poor  in  cells 
(osteomyelitis  fibrosa).  Or  the  tissue  may  liquefy,  forming  cysts.  Be- 
sides this,  proliferation  and  new-formation  of  bone  take  place  both  in 
the  periosteum  and  in  the  marrow,  leading  to  great  increase  in  the  mass 
and  density  of  the  bone  (osteosclerosis).  As  a  consequence  of  this, 
irregular  nodular  enlargements  are  formed  in  various  parts  of  the  bones, 
which  may  also  be  enormously  thickened.  Eventually,  calcification  may 
set  in,  and  as  it  becomes  dominant  the  disease  comes  to  an  end.  Richard 
and  Ziegler  regard  the  disease  as  being  strictly  comparable  to  arthritis 
deformans  while  v.  Recklinghausen  believes  it  to  be  allied  to  osteomalacia. 

Hypertrophic  Pulmonary  Osteo-arthropathy. — The  nature  of  the 
second  disease  referred  to,  hypertrophic  pulmonary  osteo-arthropathv, 
is,  if  possible,  still  more  obscure.  Attention  was  first  directed  to  it  by 
Marie1  and  shortly  after  by  Bamberger.2  It  was  precisely  described  by 
the  former,  who  differentiated  it  from  acromegaly,  to  which  it  bears  a 
strong  general  resemblance.  In  the  vast  majority  of  cases  the  disease  is 
found  in  those  suffering  from  chronic  pulmonary  or  cardiac  affections. 

In  a  typical  case  the  hands  and  feet  are  considerably  enlarged  and  the 
bones  of  the  forearm  and  leg  are  also  increased  in  size  toward  their  distal 
ends.  The  finger  tips  are  clubbed.  The  cartilages  of  the  joints  are 
eroded  and  the  synovial  fluid  is  increased.  The  process  in  the  affected 
bones  is  a  periostitis  with  sclerosing  hyperplasia  and  a  rarefying  osteo- 
myelitis. The  face  and  head  are  not  involved.  Cases  are  often  compli- 
cated with  tuberculosis  of  the  spine,  thus  giving  rise  to  deformities. 

So  far  as  is  known,  the  disease  appears  to  be  a  chronic  inflammation, 
brought  about  possibly  by  the  absorption  of  infective  agents  and  products, 
assisted  by  venous  stasis.  The  analogy  with  Hippocratic  fingers  should 
be  remarked  in  this  connection. 

Tuberculosis. — Tuberculosis  is  the  most  common  and  important 
disease  of  bone.  The  affection  attacks  by  preference  children  and  young 
persons,  but  is  not  unknown  in  middle  life.  This  is  probably  to  be  ex- 
plained in  that  the  bones  of  young  growing  individuals  are  more  vascular 
and  susceptible  to  relatively  slight  injuries,  while  the  comparatively  slow 
circulation  in  the  vessels  of  the  medulla  also  predisposes.  Although 
tuberculosis  of  the  bones  often  appears  to  be  a  primary  affection  in  so 
far  as  the  clinical  manifestations  are  concerned,  yet  the  disease  is  probably 
always  secondary  and  an  expression  of  metastasis  from  some  distant 
focus,  usually  in  the  lungs  or  lymphatic  glands.  This  focus  may  be  so 
minute  as  to  escape  observation,  or  may  even  appear  to  have  healed. 
As  a  rule,  the  infective  germs  are  brought  to  the  part  by  the  blood,  but 

>  RSvue  de  M£decine,  10:  1890:  1.  2  Zeit.  f.  klin.  Med.,  18:  1890:  193. 


TUBERCULOSIS 


1025 


occasionally  the  disease  arises  by  extension  from  a  joint  or  other  structure 
in  the  immediate  neighborhood.  Possibly  also  infection  may  take  place 
through  the  lymph-stream.  The  disease  may  be  acute,  in  the  form  of  a 
disseminated  miliary  infection,  but  this  is  always  a  terminal  event  and 
of  less  interest  than  the  more  frequent  chronic  forms. 

The  tuberculous  process  begins  either  in  the  bone-marrow  or  peri- 
osteum, and  manifests  a  preference  for  the  cancellous  structure  and 
epiphyses.  The  bones  most  frequently  involved  are  the  vertebra?, 
femora,  the  bones  of  the  tarsus  and  carpus,  the  ribs,  and  occasionally 
the  skull.  The  disease  does  not  tend  to  attack  the  medulla  except  in 
the  case  of  the  phalanges,  metacarpal  and  metatarsal  bones. 

FIG.  28G 


Tuberculous  caries  of  the  upper  end  of  the  femur:  abscess  in  the  great  trochanter. 
(Pathological  Museum,  McGill  University.) 

The  process  begins  with  the  formation  of  one  or  more  areas  of  tubercu- 
lous granulation,  which  gradually  extend  by  erosion  of  the  bony  structure 
until  they  finally  fuse.  At  the  same  time  new  foci  of  infiltration  are 
being  formed  in  the  neighborhood  of  the  older  areas.  The  bone  trabec- 
ulse  become  gradually  involved  in  a  process  of  resorption  and  caseation, 
and  finally  disintegrate,  and  in  certain  cases  considerable  portions  of 
bone  may  be  exfoliated  en  masse.  In  this  way  in  the  later  stages  caseous 
abscesses  of  varying  size  result.  To  the  naked  eye  these  appear  as 
65 


1026 


THE  BONES 


rounded  or  elongated  yellowish,  necrotic  areas  surrounded  by  grayish 
or  grayish-red,  gelatinous-looking,  granulation  tissue.  In  other  cases 
there  may  be  seen  large  areas  of  necrotic  bone  of  a  yellowish-white  or 
reddish  color  infiltrated  with  inflammatory  cells  and  surrounded  by  granu- 
lation tissue  or  caseopurulent  exudation  and  detritus.  A  much  rarer 
form  than  this  is  the  one  in  which  the  process  is  so  rapid  that  there  is 
very  little  attempt  at  the  formation  of  granulation  tissue.  Instead, 
there  is  a  diffuse  caseating  process  which  extends  rapidly  throughout  the 
bone-marrow.  Occasionally  wedge-shaped  areas  of  necrosis  and  casea- 
tion  are  found,  the  broad  base  of  the  wedge  being  situated  toward  the 
articular  surface.  This  suggests  that  the  condition  is  brought  about  by 

FIG.  287 


Tuberculous  dactylitis  (spina  ventosa).     (From  the  Surgical  Clinic  of  the  Montreal 
General  Hospital.) 

infarction,  a  view  that  is  supported  by  the  experiments  of  Miiller. 
The  articular  surfaces  of  bone  have  been  shown  to  contain  end-arteries, 
and  these  probably  become  occluded  by  infective  emboli  or  by  a  com- 
bination of  embolism  and  thrombosis. 

The  results  of  the  process  are  various.  Small  foci  undoubtedly  may 
heal  by  the  softening  and  absorption  of  the  destroyed  tissue,  which 
is  gradually  replaced  by  connective  tissue,  marrow,  or  bone.  The  larger 
cavities  may,  however,  remain  and  become  delimited  by  dense,  fibrous 
tissue  or  a  zone  of  tuberculous  granulation  tissue.  In  nearly  all  cases  a 
compensatory  process  of  the  nature  of  a  new-formation  of  bone  by  appo- 
sition takes  place.  In  other  cases  rarefaction  occurs  in  the  central  portion 


TUBERCULOUS  PERIOSTITIS 


1027 


FIG.  288 


of  the  bone,  so  that  the  central  canal  becomes  enlarged,  and  with  this 
there  is  a  deposit  of  new  bone  externally  from  the  periosteum  (spina 
ventosd).  This  resorption  and  concomitant  apposition  of  bone  may  also 
be  localized  to  a  particular  place.  In  many  instances,  especially  in  the 
larger  bones,  the  structures  become  thickened  and  sclerosed. 

Tuberculous  Periostitis. — Tuberculous  periostitis  may  be  primary  or 
secondary  to  tuberculous  osteomyelitis  and  arthritis.  The  process  may 
be  localized  in  the  form  of  an  area 
of  tuberculous  granulation  or  a 
caseous  node,  or  may  extend  over 
the  whole  surface  of  the  bone. 
Where  the  inflammation  has 
started  in  the  deeper  parts  there 
may  be  direct  communication  with 
the  exterior.  If  the  process  do 
not  tend  to  heal,  it  goes  from 
bad  to  worse,  the  tuberculous 
foci  caseate  and  soften,  and  the 
infective  agents  are  carried  along 
the  tissue  spaces  and  lymphatics, 
to  invade  the  muscles  and  the 
bloodvessels  and  possibly  the 
joints  by  a  steadily  advancing 
process  of  granulation  and  case- 
ation.  In  this  way  large  cold 
abscesses  or  caseofibroid  nodules 
are  formed.  In  advanced  cases 
the  process  may  reach  the  surface, 
giving  rise  to  tuberculous  sinuses 
and  fistulae  which  discharge  case- 
ous pus.  With  this  there  is,  as  a 
rule,  more  or  less  extensive  super- 
ficial erosion  of  the  bone  with  for- 
mation of  new  bone  from  the  osteo- 
genetic  layer  of  the  periosteum.  In 
the  case  of  the  carpus  and  tarsus 
more  than  one  bone  and  several 
joints  are  usually  involved  in  an 
extensive  destructive  process. 

In  tuberculosis  of  the  vertebrse  those  from  the  seventh  dorsal  to  the 
second  lumbar  are  the  ones  usually  involved.  The  process  begins  super- 
ficially and  tends  to  invade  the  bodies  of  the  vertebrae,  the  ligaments  are 
destroyed,  and,  owing  to  the  weight  of  the  body,  the  spine  may  collapse, 
forming  an  angular  curvature  (Pott's  disease).  The  vertebral  canal 
may  be  opened  up  and  compression  of  the  cord  from  tuberculous  deposit 
or  pressure  of  the  dislocated  bones  may  result,  giving  rise  to  a  spastic 
paralysis.  Large  prevertebral  cold  abscesses  are  occasionally  formed, 
which  may  burrow  most  extensively.  The*  usual  course  is  for  the  abscess 


Tuberculous  erosion  of  the  vertebrae.     (From 
the  Pathological  Museum  of  McGill  University.) 


1028  THE  BONES 

to  extend  retroperitoneally  downward  into  the  pelvis.  It  tends  to  point 
below  Poupart's  ligament  or  lower  in  the  thigh  (psoas  abscess),  or,  again, 
may  excavate  the  gluteal  region  and  extend  backward,  dissecting  the 
soft  tissues  away  from  the  sacrum.  In  one  case  which  we  sectioned  the 
abscess  discharged  into  the  trachea.  Here,  amphoric  breath  sounds 
and  metallic  tinkling  were  heard  in  a  limited  area  near  the  spine,  evi- 
dently due  to  the  presence  of  air  in  the  abscess  cavity.  A  tuberculous 
abscess  may  extend  through  the  sacrosciatic  notch,  giving  rise  to  symp- 
toms of  obstinate  sciatica.  Should  the  disease  heal,  the  deformity 
usually  remains.  Tuberculosis  of  the  atlas,  axis,  and  the  base  of  the 
skull  is  rare,  but  is  of  importance,  since,  when  the  ligaments  are  destroyed, 
a  sudden  strain  may  cause  dislocation  and  the  odontoid  process  of  the 
atlas  is  then  driven  forcibly  into  the  medulla,  causing  instant  death.  The 
process  more  often  than  not  does  not  completely  heal,  but  gradually 
extends,  giving  rise  to  secondary  infection,  amyloid  disease  and  exhaus- 
tion. In  some  cases  the  tubercle  bacilli  become  disseminated,  reaching 
the  lungs  and  other  distant  parts. 

Syphilis. — The  syphilitic  manifestations  in  bone  vary  considerably, 
according  as  the  disease  is  congenital  or  acquired.  In  the  first  case, 
the  affection  manifests  itself  at  the  line  of  ossification  of  the  long  bones, 
usually  the  femora.  The  lesion  is  really  a  specific  osteochondritis. 
The  line  of  calcification  is  broader  than  normal,  more  irregular,  and  of 
a  whitish  or  whitish-yellow  color. 

Microscopically,  the  zone  of  ossification  is  irregular,  the  bone-trabeculae 
vary  in  thickness,  sometimes  containing  islets  of  cartilage,  and  the  medul- 
lary spaces  are  irregular  in  size.  In  other  cases  the  process  of  ossification 
is  still  further  interfered  with;  the  cartilage  is  soft  and  swollen;  the  epi- 
physes  may  be  enlarged,  owing  to  proliferation  of  the  cartilage,  and  may 
be  separated  from  the  shaft  by  an  extensive  soft,  grayish-yellow,  or  red- 
dish zone,  in  which  are  necrotic  areas.  In  advanced  cases  the  epiphyses 
may  be  more  or  less  completely  separated  from  the  diaphysis.  The  inter- 
ference with  the  normal  process  of  ossification  leads  to  a  somewhat  char- 
acteristic form  of  dwarfing. 

In  the  acquired  form  of  the  disease  the  characteristic  lesion  is  the 
gumma,  which  may  be  situated  in  the  periosteum  or  marrow.  Periosteal 
gummas  are  much  the  commoner.  Here,  at  first,  we  see  a  localized, 
somewhat  flattened,  swelling  of  gelatinous  appearance  and  elastic  con- 
sistency. Later,  this  may  assume  a  more  grayish  appearance  and 
become  firmer,  owing  to  the  presence  of  granulation  tissue.  Dry,  whitish, 
necrotic  areas,  not  unlike  caseation,  with  more  or  less  fibrosis,  are  fre- 
quently met  with.  As  the  process  heals  it  leaves  a  dense,  fibrous  scar. 
At  the  points  where  the  periosteal  gummas  are  situated  there  are  con- 
siderable erosion  and  caries  of  the  underlying  bone.  This  process  may 
occur  in  any  part  of  the  skeleton,  but  is  most  commonly  met  with  in  the 
calvarium.  It  begins  in  the  external  layer,  may  extend  to  the  diploe 
and  the  inner  table,  finally  reaching  the  dura.  According  to  the  extent 
of  the  disease,  the  destruction  of  the  bone  may  be  almost  microscopic, 
or  furrows  and  excavations  may  be  produced,  or,  again,  large  portions 
of  the  calvarium  may  become  exfoliated. 


SYPHILIS 
FIG.  289 


1029 


Periostitis  with  destructive  inflammation  (osteoporosis)  affecting  the  frontal  and  temporal  bones 
supposedly  due  to  syphilis.     (From  the  Pathological  Museum  of  McGill  University.) 


FIG.  290 


Syphilitic  osteoporosis  of  the  calvarium  with  perforation,  due  to  multiple  gummas. 
(From  the  Pathological  Museum  of  McGill  University.) 


1030  THE  BONES 

Syphilitic  Osteomyelitis. — Syphilitic  osteomyelitis  is  rare  in  the  long 
bones  but  is  met  with  occasionally  in  the  phalanges  and  the  diploe  of  the 
cranial  vault.  Gelatinous  or  fibrogelatinous-looking  foci,  often  of  a  some- 
what purulent  character,  are  formed,  of  a  grayish-yellow  color,  in  which 
the  bone  is  becoming  necrotic.  In  the  neighborhood  the  less  affected 

Fio.  291 


Sclerosis  of  the  calvarium,  of  syphilitic  origin.  Note  the  thickness  of  the  segment  of  bone 
(at  the  lower  part  of  the  picture),  which  is  also  dense  and  ivory-like.  (Pathological  Museum, 
McGill  University.) 

bone  shows  a  tendency  to  hyperostosis.  Under  proper  medication  the 
disease  may  come  to  a  standstill  and  finally  heal.  The  granulation  tissue 
disappears,  the  caseoid  detritus  is  absorbed,  dead  bone  is  sequestrated  and 
cast  off,  and  any  defects  are  either  filled  up  by  new  bone  or  bridged  over 
by  connective  tissue.  The  bone  in  the  neighborhood  frequently  becomes 


MADURA  FOOT 


1031 


dense  and  sclerotic,  and  of  a  texture  and  hardness  resembling  ivory. 
In  all  forms  of  this  affection  osteoplasia  is  a  marked  feature,  and,, in 
fact,  in  one  type  of  periostitis,  may  dominate  the  anatomical  picture. 
It  should  be  mentioned  that,  where  large  masses  of  bone  are  being 
sequestrated,  inflammation  may  extend  to  the  soft  tissues  and  skin,  so 
that  inflammatory  exudation  and  necrotic  material  are  discharged 
externally  with  the  formation  of  suppurating  sinuses. 

Actinomycosis. — This  is  usually  found  in  the  maxillae,  vertebrae,  and 
bones  of  the  thorax.  Infection  usually  takes  place  through  the  alimentary 
tract.  The  infective  agent  is  the  ray-fungus  (actinomyces  bovis) ,  which 
seems  to  be  frequently  present  on  grass  and  hay.  The  disease  has  been 
known  to  follow  pricking  the  gums  with  a  needle,  or  may  invade  the  alveolar 
process  through  decayed  teeth.  The  infection  may  also  enter  from  the 
cecum  and  appendix,  whence  it  spreads  to  the  retroperitoneal  tissues, 
thence  to  the  ilium.  At  first  a  periostitis  is  produced  and  the  fungus 
gradually  extends  into  the  interior  of  the  bone,  which  is  rarefied  and 
infiltrated  with  granulation  tissue.  There  is  considerable  destruction 
of  the  bone.  Microscopically,  the  affected  structures  are  involved  in 
a  granulating  necrotizing  process.  By  proper  methods  the  ray-fungus 
can  be  detected  in  the  inflammatory  tissues. 


FIG.  292 


Mycetoma.     Fungus  surrounded  by  a  dense  accumulation  of  leukocytes.      X  300. 
(Dr.  Hyde's  case;   from  a  photomicrograph.) 

Madura  Foot. — Madura  foot  is  a  disease  of  the  bones  of  the  tarsus 
closely  resembling  actinomycosis.     The  infective  agent  is  a  fungus,  in 


1032  THE  BONES 

some  cases,  the  mycetoma  pedis,  an  organism  allied  to  the  actinomyces; 
in  other  instances  an  aspergillus  has  been  found.  The  disease  is  most 
common  in  India,  but  a  few  instances  have  been  met  with  on  the  American 
continent.  One  case  has  been  reported  in  this  country  which  is  remark- 

FIG.  293 


Osseous  lesions  in  mycetoma.     (Hyde.) 

able  in  that  it  occurred  in  a  person  who  had  spent  his  whole  life  in 
America.1  The  lesions  produced  are  similar  to  those  in  actinomycosis, 
leading  to  rarefaction  and  destruction  of  the  affected  bones,  with  numerous 
discharging  sinuses,  the  pus  from  which  contains  the  specific  organism 
(vide  also  p.  924). 

1  Adami  and  Kirkpatrick,  Trans.  Assoc.  Amer.  Phys.,  10  :  1895  :  82. 


ATROPHY  1033 

Lepra. — In  leprosy,  granulomas  containing  bacilli  may  be  found  in 
the  periosteum  and  bone-marrow,  causing  more  or  less  osteoporosis  and, 
where  an  extremity  is  involved,  mutilation. 

Variola. — According  to  Chiari,1  in  variola  an  osteomyelitis  may  be 
found  characterized  by  the  formation  of  multiple  minute,  yellowish  foci 
with  gray  centres,  varying  in  size  from  that  of  a  millet-seed  to  that  of  a 
split  pea. 

Microscopically,  these  consist  of  epithelioid  cells,  a  few  leukocytes,  and 
a  fibrinous  exudate  with  central  necrosis. 

Parasites. — Echinococcus  and  Cysticercus  cellulQsceh&ve  been  met  with, 
forming  cysts. 

RETROGRESSIVE  METAMORPHOSES. 

The  structure  of  the  bony  framework  of  the  body  is  in  health  under- 
going constant  change.  In  the  child,  while  there  is  a  certain  amount  of 
breaking  down  of  the  substance  of  the  bone,  vegetative  and  productive 
forces  are  predominant,  with  the  result  that  the  bone  increases  in  size 
and  strength  until  it  attains  its  perfect  structure.  In  the  adult,  breaking 
down,  or  resorption,  as  it  is  called,  also  goes  on,  but  is  compensated  by 
a  continuous  deposit  of  bone  by  the  process  of  apposition.  In  the 
aged,  however,  resorption  is  in  excess,  so  that  the  bone  becomes  smaller, 
lighter,  and  more  fragile.  Lacunar  resorption,  both  under  normal  and 
pathological  conditions,  is  brought  about  by  the  agency  of  large,  multi- 
nucleated  cells,  the  osteoclasts  (myeloplaxes),  situated  in  the  periosteum 
and  bone-marrow.  These  take  up  their  position  upon  the  bony  trabec- 
ulse  and  gradually  erode  their  way  into  the  structure,  forming  excava- 
tions, called  Howship's  lacunae. 

Atrophy. — In  the  rapid  resorption  of  the  bone  characteristic  of 
certain  diseases,  the  osteoclasts  are  greatly  increased  in  number  and 
lie  closely  packed  together.  The  result  of  this  is  that  the  surface  of 
the  bone  becomes  rough,  eroded,  and  irregular.  Should  the  process  come 
to  an  end,  the  projecting  ridges  are  absorbed,  there  is  a  deposit  of  new 
bone  in  the  hollows,  and  the  surface  of  the  bone  again  becomes  smooth. 
Should  the  resorption  be  most  marked  next  the  medullary  cavity,  the 
external  appearance  of  the  bone  is  not  altered,  but  the  cavity  is  enlarged, 
the  trabeculse  become  gradually  thinner,  and  may,  in  parts,  disappear 
(excentric  atrophy).  When  the  process  begins  externally,  the  bone 
becomes  gradually  thinner  and  local  defects  are  manifested  (concentric 
atrophy).  In  other  cases  the  compact  substance  of  the  bone  becomes 
porous,  owing  to  the  widening  of  the  Haversian  canals.  This  is  known 
as  osteoporosis.  According  to  Pommer,2  atrophy  of  bone  may  be  a 
relative  matter,  that  is  to  say,  the  amount  of  lacunar  resorption  does  not 
exceed  the  normal,  but  there  is  a  diminished  deposit  of  new  bone  by 
apposition,  so  that  the  bone  becomes  smaller,  or,  again,  the  atrophy 
may  be  absolute. 

1  Ziegler's  Beitrage,  9:  1891. 

2  Ueber  die  Osteoklastentheorie,  Virch.  Archiv,  92:  1883:  449. 


1034 


THE  BONES 


Atrophied  bones  are  light  and  fragile,  easily  broken  or  sawn.  The 
medullary  substance  will  vary  in  appearance  according  to  the  extent  of 
the  affection.  It  may  be  hyperplastic,  presenting  the  appearance  of 
lymphoid  marrow,  fatty,  or  the  fat  may  be  replaced  by  a  semitranslucent, 
gelatinous-looking  substance  (serous  atrophy).  As  a  consequence  of  the 
excessive  resorption  of  the  solider  portions,  the  bones  become  brittle 
and  unable  to  support  their  accustomed  burden,  and  may  readily  fracture 
(symptomatic  osteopsathyrosis,  fragilitas  ossium).  Atrophy  of  bone  may 
arise  as  a  senile  or  marantic  change,  from  pressure,  disuse,  or  from 
neurotrophic  disorders. 

FIG.  294 


Atrophy  of  the  bodies  of  the  vertebrae  from  the  pressure  of  an  aneurism. 
Museum,  McGill  University.) 


(Pathological 


Senile  and  Marantic  Atrophy. — Senile  and  marantic  atrophy  may  affect 
the  skeleton  as  a  whole,  but  the  former  is  apt  to  involve  more  ex- 
tensively the  flat  bones,  the  calvarium,  the  scapulae,  and  the  pelvis. 
The  process  begins  at  the  points  which  are  devoid  of  muscular  attach- 
ments. The  atrophy  may  be  concentric  or  excentric,  and  the  bone  may 
also  become  more  porous.  The  facies  so  characteristic  of  old  age  is 
due  to  atrophy  of  the  maxillse,  the  alveolar  processes  of  which  may 
disappear  entirely.  In  the  case  of  the  calvarium,  the  whole  of  the  outer 
table  and  the  diploe,  or  even  portions  of  the  inner  table,  may  be  destroyed. 


ATROPHY 


1035 


In  some  instances  there  is  a  deposit  of  new  bone  on  the  surface  of  the 
inner  table.  This  is  most  frequently  seen  in  the  frontal  bone.  The 
vertebrae  become  porous  and  diminished  in  size,  so  that  the  bodily  height 
is  diminished.  The  dorsal  curvature,  so  often  seen  in  elderly  people,  is 
due  largely  to  the  absorption  of  the  anterior  portion  of  the  intervertebral 
disks. 

FIG.  295 


Rarefaction  of  the  shaft  of  the  humerus,  due  to  carcinoma. 
McGill  University.) 


(Pathological  Museum, 


Pressure  Atrophy. — Atrophy  from  pressure  is  of  course  local.  A 
familiar  instance  is  the  depression  in  the  bones  of  the  calvarium  due  to 
the  Pacchionian  bodies.  Hydrocephalus  and  intracranial  growths  lead 
to  atrophy  of  the  calvarium.  Pressure  atrophy  is  also  brought  about  by 
aneurisms,  tumors,  scars  in  the  skin  and  subcutaneous  tissues,  the  pressure 
of  the  umbilical  cord  on  the  embryo. 

Inflammatory  Atrophy. — Atrophy  may  also  follow  inflammation. 

Atrophy  from  Disuse. — Atrophy  from  disuse  is  met  with  especially  in 
the  limbs,  as  in  amputations,  fractures,  chronic  arthritis,  joint  and  bone 
inflammations. 


1036  THE  BONES 

Neuropathic  Atrophy. — The  neuropathic  forms  result  from  some  dis- 
order of  the  central  nervous  system,  as  dementia  paralytica,  tabes 
dorsalis,  syringomyelia,  anterior  poliomyelitis.  In  many  cases,  however, 
the  atrophy  is  attributable  to  disuse  as  well  as  to  disease. 

[  FIG.  296 


The  femur  and  tibia  of  an  idiot,  showing  the  simple  atrophy  of  disuse.  The  contrast  in  size 
between  the  shaft  and  the  extremities  of  the  bones  is  marked.  (From  the  Pathological  Museum 
of  McGill  University.) 

Death. — Death  of  bone  takes  two  forms — caries  and  necrosis.  Caries 
is  a  slow  disintegration  of  the  bone  into  fine  and  almost  imper- 
ceptible particles,  and  is  analogous  to  suppuration  of  the  soft  tissues. 
Necrosis  may  be  compared  to  gangrene  and  is  death  of  bone  occurring 
en  masse.  Caries  is  practically  always  due  to  inflammation.  Necrosis 
may  be  due  to  inflammation,  interference  with  the  proper  circulation  of 
the  part,  traumatism,  or  chemical  and  thermic  agencies.  As  a  rule, 
when  a  portion  of  a  bone  dies  it  becomes  separated  from  the  healthy 
part  by  a  zone  of  reactive  inflammation,  where  resorption  and  exfolia- 
tion is  actively  going  on.  In  such  cases  the  necrotic  portion  is  termed 
a  sequestrum. 


HALISTERESIS  1037 

Halisteresis. — A  striking  and  important  retrogressive  change  some- 
times found  in  bone  is  halisteresis,  a  condition  in  which,  while  the  organic 
substance  of  the  bone  remains  comparatively  unaltered,  there  is  a  notable 
diminution  in  the  amount  of  lime  salts,  so  that  the  bone  becomes  soft 
and  yielding.  The  process  may  be  restricted  to  a  small  area  in  a  bone, 
as,  for  instance,  in  the  neighborhood  of  a  tumor,  or  may  be  more  widely 
spread  throughout  a  whole  bone,  or  even  the  greater  part  of  the  skeleton. 
The  more  extensive  affection  is  commonly  known  by  the  name  of  osteo- 
malacia  (mollities  ossium,  malacosteon). 

The  pathological  changes,  here,  consist  in  the  main  of  decalcification 
of  the  old  bone,  with,  at  the  same  time,  a  tendency  to  the  formation  of 
new  bone,  which,  however,  remains  imperfectly  calcified.  The  process 
of  decalcification  begins  at  the  periphery  of  the  bone-trabeculae  and 
gradually  extends  to  the  deeper  parts.  The  line  of  demarcation 
between  the  normal  and  the  altered  bone  is  sometimes  even  and  con- 
tinuous, or  may  be  irregular  with  excavations,  like  Howship's  lacunae. 
Frequently  there  is  formed  an  intermediate  zone,  where  the  lime  salts 
are  not  completely  absorbed  but  remain  in  the  tissue  in  the  form  of  a 
crumbling  detritus  preliminary  to  their  removal.  In  the  course  of  the 
disease  the  original  bone  canals  become  enlarged,  and,  following  upon 
the  absorption  of  the  salts,  new  canals  are  formed  in  the  ground  sub- 
stance. The  matrix  itself  may  appear  to  be  homogeneous,  or  may 
present  a  finer  or  coarser  fibrillation.  Some  of  the  bone-corpuscles 
may  be  preserved  but  many  are  atrophied  or  have  disappeared,  leaving 
small  cavities.  In  some  cases  there  is  a  formation  of  new  osteoid  tissue, 
which  for  a  long  time,  or  perhaps  permanently,  remains  uncalcified. 
This  new  tissue  may  be  quite  dense,  containing  only  a  few  spaces,  or  it 
may  present  a  laminated  or  fibrillated  structure  with  large  corpuscles. 
Osteoclasts  and  Howship's  lacunas  are  not  more  numerous  than  in  normal 
bone.  The  condition  of  the  marrow  varies.  It  may  be  reddish,  with 
giant  cells,  yellowish  and  fatty,  gelatinous,  or  even  fibroid.  Hemorrhages 
and  pigment  are  commonly  found  in  the  marrow. 

As  would  be  expected,  such  changes  in  the  structure  and  consistence 
of  the  bones  lead  to  marked  interference  with  their  function.  The 
bones  are  no  longer  able  to  support  the  weight  of  the  body  or  oppose 
muscular  contractions,  so  that  curvatures,  fractures,  and  indentations 
are  not  uncommon.  The  bone  becomes  so  soft  that  it  is  wax-like  and 
is  readily  cut  with  the  knife  (osteomalacia  cerea).  In  other  cases  resorp- 
tion  of  the  bone  is  so  excessive  that  there  is  a  huge  medullary  cavity 
with  a  mere  shell  of  bone  beneath  the  periosteum,  so  that  the  bone  is 
light  and  brittle  (osteomalacia  fragilis).  When  the  vertebral  column  is 
involved,  lordosis,  kyphosis,  and  scoliosis  are  frequently  met  with,  with 
all  that  this  implies.  The  clavicles  and  ribs  may  be  much  deformed, 
and  the  thorax  is  flattened  from  side  to  side,  the  anteroposterior  diameter 
being  increased.  In  the  pelvis,  owing  to  the  weight  of  the  body  and 
the  pressure  of  the  femora,  the  acetabular  regions  are  driven  in,  the  pubes 
is  pushed  forward,  while  the  promontory  descends.  The  tuberosities 
of  the  ilia  are  more  or  less  approximated.  The  cavity  of  the  pelvis  is 


1038  THE  BONES 

thus  greatly  reduced,  a  deformity  which  is  of  the  greatest  importance 
in  regard  to  the  question  of  parturition.  Not  only  is  there  deformity,  but 
the  bones  actually  shrink,  so  there  is  a  double  reason  for  the  production 
of  a  contracted  pelvis.  In  the  lower  extremities  there  is  at  first  an 
exaggeration  of  the  natural  curves  of  the  bones,  but  later  there  are  more 
acute  curvatures  or  twists.  In  the  femur  the  greatest  deformity  is  found 
just  below  the  trochanter.  Where  bending  or  fractures  have  taken  place 
there  is  an  attempt  at  repair  by  the  formation  of  new  osteoid  tissue 
along  the  concave  side  of  the  curvature  or  at  the  site  of  the  fracture. 

The  true  cause  of  osteomalacia  is  quite  obscure.  The  disease  is 
found  both  in  the  old  and  in  the  young,  but  is  most  common  between 
the  third  and  fourth  decade.  It  is  usually  met  with  in  women,  especially 
in  those  who  are  pregnant  or  unusually  prolific,  while  it  is  only  rarely 
found  in  men.  It  is  noteworthy  that  the  affection  is  endemic  in  certain 
localities,  such  as  the  Rhine  valley,  Westphalia,  Flanders,  and  northern 
Italy,  although  cases  are  not  unknown  in  other  parts  of  Europe.  It 
appears  to  be  rare  on  the  North  American  continent,  Dock1  only  finding 
record  of  ten  cases.  Among  the  direct  exciting  causes  the  most  important 
is  pregnancy,  which  in  the  majority  of  instances  initiates  the  affection 
or  leads  to  relapses  and  exacerbations. 

Numerous  theories  have  been  advanced  to  explain  the  condition. 
Some,  like  v.  Winckler,  think  that  unhygienic  surroundings  and  modes 
of  life,  unsuitable  or  poor  food,  insufficient  clothing,  repeated  preg- 
nancies, or  prolonged  lactation,  are  the  important  predisposing  causes. 
Hanau's  observation,  that  in  25  to  30  per  cent,  of  puerperal  women, 
osteophytes  and  osteoid  tissue  are  present  in  the  cranial  bones,  suggests 
that  osteomalacia,  at  least  in  puerperal  cases,  may  be  due  to  an  exag- 
geration of  a  physiological  process.  That  there  is  some  connection 
between  the  disease  and  the  genital  apparatus  would  seem  to  be  indi- 
cated by  the  fact  that  it  is  sometimes  cured  by  removal  of  the  ovaries. 
On  these  grounds  Fehling  has  enunciated  the  theory  that  osteomalacia 
is  a  trophoneurosis  due  to  reflex  irritation  from  the  ovaries.  In  view 
of  the  frequent  existence  of  hyperemia  of  the  bone-marrow,  v.  Reckling- 
hausen  is  of  the  opinion  that  the  disease  is  due  to  a  local  irritation  and 
stimulation  of  the  bloodvessels  of  the  bones.  Virchow  also  believed  the 
condition  to  be  of  an  inflammatory  or  hyperemic  nature.  Others,  like 
Volkmann,  think  that  in  addition  to  circulatory  disturbances  there  is 
some  abnormality  of  the  nerve-supply  in  the  medulla.  Examination  of 
the  central  nervous  system,  however,  does  not  reveal  any  special  evi- 
dence of  this.  It  used  to  be  thought,  too,  that  an  excess  of  lactic  acid 
in  the  blood  was  the  cause  of  the  solution  of  the  calcareous  salts  of  the 
bone.  It  is,  however,  not  the  case  that  there  is  an  excess  of  this  sub- 
stance in  the  blood  in  osteomalacia,  nor  has  it  been  found  possible  to 
produce  the  disease  in  experimental  animals  by  feeding  them  with 
this  acid. 

1  Amer.  Jour.  Med.  Sci.,  109:1895:449. 


HYPERPLASIA 


1039 


FIG.  297 


PROGRESSIVE  METAMORPHOSES. 

While  under  ordinary  circumstances  bone  may  be  regarded  as  the  most 
stable  and  unchanging  tissue  of  the  body,  waste  and  repair  are  to  some 
extent  always  going  on.  In  the 
adult  these  two  opposing  processes 
are  almost  perfectly  balanced,  so 
that  for  a  time  at  least  the  volume 
and  the  texture  of  the  skeleton 
remain  constant.  On  occasion, 
however,  regenerative  processes 
may  become  more  active,  as,  for 
example,  in  the  process  of  repair 
of  bone  after  injuries,  and  some- 
times result  in  an  excess  of  growth 
over  and  above  the  obvious  needs 
of  the  organism. 

Hyperplasia. — The  causes  un- 
derlying hyperplasia  of  bone  are 
often  obscure.  Some  forms,  such 
as  leontiasis  ossea,  local  and  gener- 
alized gigantism,  are  congenital 
and  primary,  being  apparently  due 
in  the  main  to  excessive  or  dis- 
ordered nutrition  during  prenatal 
existence.  Much  more  commonly 
the  process  is  acquired  and  second- 
ary. A  common  example  of  this 
is  the  repair  that  takes  place  after 
fractures,  and  the  increase  in  length 
and  thickness  of  bones  in  certain 
cases  of  inflammation  and  trauma- 
tism.  Experimentally,  increase  in 
the  length  of  bones  has  been  pro- 
duced by  driving  ivory  pegs  into  the 
growing  end  of  the  bone.  In  other 
cases,  hyperplasia  seems  to  be  due 
to  chemical  substances  circulating 
in  the  blood.  Thus,  the  exhibition 
of  phosphorus  and  arsenic  (Gies,1 
Kassowitz,2  Maas,3  Wegner4)  stimulates  bone  production  in  experimental 
animals. 


Fracture  of  the  humerus  wit'i  the  formation 
of  an  enormous  permanent  callus.  The  patient 
from  whom  this  specimen  was  taken  was  a 
lunatic,  who  kept  his  arm  in  almost  constant 
motion.  As  a  consequence,  splints  could  not 
be  kept  properly  applied.  (From  the  Patho- 
logical Museum  of  McGill  University.) 


1  Einfluss  des  Arsens  auf  den  Organismus,  Arch.  f.  exper.  Path.,  8:  1877. 

2  Zeit.  f.  klin.  Med.,  7:  1884:  36. 

8  Tageblatt  d.  Leipsiger  Naturforschervers,  1872. 

4  Ueberden  Einfluss  des  Phosphors  auf  den  Organismus,  Virch.  Archiv,  55: 1872 : 11. 


1040  THE  BONES 

In  this  category  also  may  be  placed  those  hypertrophies  of  the  bone 
and  soft  tissues  associated  with  disorders  of  internal  secretion,  as,  for 


FIG.  298 


Normal  skull.  Skull  from  a  case  of  acromegaly.     (Osborne.) 

FIG.  299 


New-growth  of  osteophytes  about  the  hip-joint,  the  result  of  chronic  arthritis.     (From 
the  Pathological  Museum  of  McGill  University.) 


CALLUS  1041 

example  in  acromegaly.  An  increase  in  the  length  of  the  bones,  par- 
ticularly those  of  the  lower  extremities,  is  frequently  observed  also  after 
castration. 

Prolonged  passive  congestion  seems  also  to  favor  overgrowth  of  tissue, 
as  in  the  clubbed  fingers  of  those  suffering  from  chronic  pulmonary  and 
cardiac  affections. 

Bony  hyperplasia  is  sometimes  also  compensatory,  for  example,  the 
well-known  enlargement  of  the  fibula  in  ununited  fracture  of  the  tibia. 

The  overproduction  of  bone  may  manifest  itself  in  several  ways. 
When  a  bone  becomes  enlarged,  either  as  a  whole  or  in  part,  the  condi- 
tion is  termed  hyperostosis.  Again,  when  the  density  of  bone  is  increased 
owing  to  the  formation  of  new  trabeculse  and  the  deposit  of  an  excess 
of  lime  salts,  so  that  the  structure  becomes  more  compact,  we  speak  of 
osteosclerosis.  Both  conditions  may  be  combined.  Local  outgrowths 
of  bone  are  called  osteophytes  or  exostoses.  These  may  be  seen  in  the 
neighborhood  of  inflammatory  processes,  and  at  the  points  of  insertion 
of  the  tendons  where  these  are  subjected  to  excessive  muscular  traction. 
Enostoses  are  local  new-formations  of  bone  within  the  spongiosa.  It  is 
hard,  however,  in  some  cases  to  draw  the  line  between  local  hyperostoses 
and  true  tumor-formation. 

Callus  (see  vol.  i,  p.  557). — When  a  bone  is  fractured  or  splintered, 
as  from  some  traumatic  cause,  regenerative  processes  are  initiated  in 
the  periosteum  and  bone-marrow,  which,  provided  that  the  process  be 
not  complicated  by  infection  or  senile  or  other  cachexia,  lead  in  the 
course  of  a  few  weeks  to  consolidation  and  more  or  less  perfect  repair 
of  the  injury. 

In  the  ordinary  course  of  events,  immediately  on  receiving  the  fracture, 
there  are  more  or  less  tearing  and  bruising  of  the  neighboring  soft 
tissues,  together  with  extravasation  of  blood.  A  moderate  amount  of 
inflammation  sets  in,  with  effusion  of  fluid  and  infiltration  of  leukocytes 
into  the  structures  in  the  neighborhood  of  the  injury.  This  subsides  in 
from  five  to  six  days.  About  the  second  day  the  cells  of  the  periosteum 
and  bone-marrow  show  signs  of  proliferation  in  that  they  are  enlarged 
and  their  nuclei  are  undergoing  karyokinesis.  In  the  next  few  days 
the  number  of  the  proliferating  cells  is  greatly  increased  and  the  endo- 
thelium  of  the  bloodvessels  now  begins  to  take  part  in  the  process, 
so  that  about  the  fourth  day  the  osteoblastic  layer  of  the  periosteum  is 
converted  into  a  vascular  germinal  tissue.  Under  the  microscope  this 
consists  of  large  polymorphous  cells,  containing  frequent  mitotic  figures, 
embedded  in  a  partly  homogeneous,  partly  fibroid,  stroma.  After  the 
fourth  day,  the  germinal  layer  begins  to  be  differentiated  into  chondroid 
and  osteoid  tissue,  which,  in  turn,  is  rapidly  converted  into  bone.  After 
the  lapse  of  a  week,  the  ends  of  the  fractured  bone  are  embedded  in  a 
large  number  of  young  osteophytes  and  osteoid  spicules.  In  this  way 
is  produced  about  the  injured  region  a  spindle-shaped  sheath,  or  natural 
splint,  called  the  external  callus.  A  prolongation  of  this  between  the 
ends  of  the  fractured  portions  constitutes  the  intermediary  callus.  Simi- 
larly, a  callus  is  formed  within  the  cavity  of  the  bone,  if  one  of  the  hollow 
66 


1042 


THE  BONES 


bones,  constituting  the  internal  or  myelogenic  callus.  This  is  produced 
by  the  osteoblasts  which  are  grouped  into  masses  and  are  transformed 
into  osteoid  and  eventually  into  osseous  tissue.  In  the  neighborhood  of 
the  fractured  portion  the  periosteal  germinal  layer  may  be  converted 
in  part  into  hyaline  cartilage  and  in  part  into  fibrous  connective  tissue, 
which,  in  time,  is  transformed  into  bone.  In  the  course  of  from  two  to 


FIG.  300 


FIG.  301 


A  badly-set  fracture  of  the  femur,  showing 
union  with  interruption  of  the  central  canal. 
(From  the  Pathological    Museum  of    McGill 
University.) 


Femur;  ununited  fracture  through  the  great 
trochanter;  excessive  growth  of  callus.  (From 
the  Pathological  Museum  of  McGill  Univer- 
sity.) 


three  weeks  the  fractured  ends  are  more  or  less  completely  reunited. 
The  amount  of  callus  resulting  from  this  process  varies  considerably 
at  times,  being  dependent  on  individual  idiosyncrasy,  the  condition  of 
the  bone,  the  nature  of  the  fracture,  and  the  amount  of  deformity. 

In  addition  to  the  formation  of  new  tissue,  and  to  some  extent  syn- 
chronous with  it,  an  opposite  process  is  at  work,  namely,  resorption. 
The  fractured  ends  become  somewhat  rounded  off  and  splinters  of  bone 


TUMORS  1043 

separated  from  the  main  mass  are  absorbed.  The  callus,  which,  at  the 
end  of  the  sixth  or  seventh  week,  consists  of  a  rather  soft  and  porous 
bony  substance,  is  gradually  converted  into  denser  bone  by  means 
of  lacunar  resorption,  the  formation  of  medullary  spaces,  and  the 
thickening  of  the  trabeculse  through  the  agency  of  the  osteoblasts.  In 
this  way  the  permanent  or  definitive  callus  is  substituted  for  the  temporary 
one.  In  the  course  of  months  or  years,  according  to  the  amount  of 
traumatism  and  deformity,  the  permanent  callus  is  still  farther  modi- 
fied. Excess  bone  is  removed  and  the  weak  spots  are  strengthened 
until  a  more  or  less  perfect  return  to  functional,  if  not  anatomical,  in- 
tegrity is  complete.  In  severe  dislocations  of  the  parts  the  medullary 
cavity  of  the  bone  is  not  usually  restored. 

The  process  as  just  described  may  be  materially  modified  by  certain 
untoward  factors.  Thus,  infection,  inflammation,  or  necrosis  may 
delay  the  union  of  the  fragments,  or,  again,  the  condition  of  senility  or 
cachexia  may  prevent  it.  When  two  bones  are  in  close  proximity  and 
one  only  is  broken,  the  resulting  callus  may  involve  the  uninjured  bone, 
producing  a  synostosis.  In  fractures  near  or  involving  a  joint  an  exuber- 
ant formation  of  osteophytes  may  lead  to  ankylosis  of  the  joint.  Should 
the  separated  fragments  be  improperly  replaced,  be  too  far  apart,  or 
should  a  large  amount  of  bone  be  destroyed,  or,  again,  should  muscle 
or  fascia  intervene,  the  parts  may  fail  to  unite.  Should  the  fragments 
be  united  immovably  by  fibrous  tissue,  the  condition  is  known  as  patho- 
logical syndesmosis.  In  other  cases  fibrous  union  takes  place,  leading 
to  the  establishment  of  a  false  joint,  pseudarthrosis.  In  still  other  cases 
a  true  joint,  with  a  more  or  less  perfect  approximation  to  the  ball  and 
socket  type  with  a  capsule,  may  be  formed — nearthrosis. 

As  might  be  expected,  the  process  of  healing  is  completed  more 
quickly  in  children,  taking  place  in  from  two  to  three  weeks  in  those 
under  two  years  of  age,  while  in  adults  it  may  take  six  to  eight  weeks. 
As  we  have  already  seen,  it  may  be  much  prolonged  and  even  fail 
to  occur. 

Tumors. — The  tumors  that  develop  primarily  in  the  bones  belong 
to  the  connective-tissue  group  and  originate  from  the  periosteum,  the 
bone-marrow,  or  the  cartilage.  In  accordance  with  their  genesis  they 
assume  the  type  of  fibroma,  myxoma,  lipoma,  angioma,  chondroma,  osteoma, 
myeloma,  sarcoma,  and  various  admixtures  thereof.  The  secondary 
tumors  are  usually  various  forms  of  carcinoma. 

Like  that  of  tumor-growth  generally,  the  etiology  of  the  neoplasms  of 
bone  is  somewhat  obscure.  Traumatism,  however,  such  as  a  fracture 
or  blow,  seems  to  play  a  relatively  important  part  (callus  tumors),  as 
does  also  inflammation.  Irregularities  in  ossification  were  believed  by 
Virchow  to  account  for  the  chondromas,  especially  those  arising  in  the 
neighborhood  of  the  epiphyseal  sutures. 

The  primary  tumors  are  usually  solitary,  but  occasionally  assume  the 
form  of  multiple,  isolated,  and  independent  growths.  The  presence  of 
tumors  in  bones,  especially  when  of  the  malignant  type,  leads  usually  to 
considerable  lacunar  resorption  of  the  structure,  so  that  the  bone  may 


1044  THE  BONES 

become  greatly  deformed.  Besides  this,  there  is  often  a  production  of 
new  bone  from  the  periosteum,  owing  to  the  stimulation  of  the  osteo- 
genetic  layer,  with  the  result  that  the  new-growth  may  be  more  or  less 
completely  enclosed  within  a  bony  shell.  Not  only  so,  the  cells  of  the 
tumors  and  of  their  supporting  stroma  show  a  peculiar  liability  to 
undergo  metaplasia  into  bone,  and  a  more  or  less  perfect  osseous  frame- 
work may  be  produced  within  the  growth.  Sarcomas  and  carcinomas 
are  most  likely  to  manifest  this  tendency.  The  ground  substance  in 
some  cases  also  undergoes  petrifaction. 

FIG.  302 


Spindle-celled  periosteal  sarcoma  of  the  hand.     (From  the  surgical  clinic  of  the  Montreal 

General  Hospital.) 

Sarcoma. — The  most  important  and  frequent  of  the  primary  tumors 
of  bones  is  the  sarcoma.  Of  this  there  are  two  varieties,  the  myelogenic 
and  the  periosteal.  The  former  are  rapidly  growing  tumors  which  tend 
to  produce  great  rarefaction  and  expansion  of  the  shaft  of  the  affected 
bone.  Microscopically,  they  are  giant-celled,  round-celled,  spindle- 
celled,  and  alveolar.  They  are  dealt  with  more  at  length  elsewhere 
(see  p.  234  et  seq.). 

The  periosteal  sarcomas  are  usually  of  spindle-  or  mixed-celled  type, 


SARCOMA  1045 

but  occasionally  are  composed  of  round  cells.  They  may  be  found  in 
any  part  of  the  skeleton,  but  are  most  frequent  near  the  ends  of  the  long 
bones,  in  the  upper  maxilla,  and  on  the  shoulder  girdle.  The  denser 
forms  are  closely  allied  to  the  fibromas,  and  the  two  conditions  may  pass 
almost  imperceptibly  one  into  the  other.  The  term  epulis  is  applied 
clinically  to  either  a  fibrous  or  fibrosarcomatous  periosteal  new-growth 
in  the  buccal  and  nasal  cavities. 


FIG.  303 


s*  *  . 


Spindle-celled  sarcoma  of  the  periosteum.     Winckel  obj.  No.  6,  without  ocular.      (From 
the  collection  of  Dr.  A.  G.  Nicholls.) 

Periosteal  sarcomas  arise  at  first  on  one  side  of  the  bone,  and  tend 
gradually  to  envelop  it.  The  underlying  bone  becomes  rarefied  and 
destroyed,  or  may,  on  the  contrary,  be  transformed  into  very  dense 
tissue.  These  tumors  frequently  produce  bone  in  the  form  of  plates  and 
spicules  of  osteoid  tissue  without  calcification  (osteoid  sarcoma).  In 
other  cases  a  denser  anastomosing  framework  of  bony  processes  is  pro- 
duced from  which  finer  spicules  and  plates  grow  out  in  a  radiating 
manner  into  the  substance  of  the  softer  tissues  (psteosarcoma,  ossifying 
sarcoma).  Cartilaginous  and  sarcomatous  growths  may  be  combined 
(chondrosarcomd) ,  or  cartilage,  bone,  and  sarcoma  (chondroosteosarcomd). 

An  important  clinical  type  in  the  giant-celled  sarcoma,  which  origi- 
nates on  the  alveolar  process  or  in  the  antrum  of  Highmore.  It  is 
small,  firm,  and  relatively  slow  growing,  and  is  one  of  the  least  malig- 
nant forms  of  the  sarcomas,  since  when  removed  it  does  not  always 
recur. 

Microscopically,  it  is  dense  and  fibrous  and  of  sessile  form.      On 


1046  THE  BONES 

section  it  is  of  a  brick-red  color,  owing  to  the  fact  that  hemorrhage  into 
its  substance  is  common.  Microscopically,  it  consists  of  fibrous  tissue, 
with  masses  of  spindle  and  multinucleated  giant  cells. 

The  myeloma  is  a  peculiar  and  interesting  tumor  of  bone  described  at 
length  elsewhere  (p.  236). 

Osteoma. — Of  the  benign  growths,  perhaps  the  commonest  is  the 
osteoma.  It  is  not  always  possible  to  draw  the  line  between  osteo- 
phytes  and  hyperostoses  of  inflammatory  origin  and  tumors  proper. 
The  true  osteomas  are  usually  found  in  early  childhood  or  during  the 
developmental  period  of  life,  and  may  even  be  inherited.  Especially 
when  the  exostoses  are  multiple,  or  derived  from  cartilage,  it  is  likely 
that  they  are  due  to  some  aberration  in  the  growth  of  the  skeleton,  for 
in  such  cases  other  disturbances  of  development  are  apt  to  be  present. 
Osteomas  are  formed  from  the  periosteum  (exostoses)  or  from  the  bone- 
marrow  (enostoses).  They  may  arise  also  by  metaplasia  from  fibrous 
tissue  or  cartilage. 

According  to  their  structure,  osteomas  may  be  divided  into  two  forms, 
one  composed  of  dense  compact  bone — osteoma  eburneum;  the  other 
formed  of  cancellous  bone — osteoma  spongiosum.  Small  exostoses  are 
rounded,  conical,  nodular,  or  fungoid  in  appearance,  while  the  larger 
ones  are  bulbous,  warty,  irregular,  or  even  pectinate.  The  fibrous 
exostoses  develop  especially  in  connection  with  the  bones  of  the  skull 
and  the  flat  bones  of  the  trunk,  and  the  cartilaginous  ones  at  the  diaphy- 
seal  ends  of  the  long  bones.  Occasionally,  in  the  case  of  exostoses  near 
a  joint  one  sees  a  closed  membranous  sac  resembling  a  bursa,  and 
structurally  similar  to  the  synovial  membrane,  associated  with  the 
tumor  (exostosis  bursata).  This,  rarely,  contains  free  bodies,  and  is 
supposed  to  be  derived  from  the  cartilage  of  the  joint  or  a  misplaced 
"rest." 

The  enostoses  are  found  most  frequently  in  the  diploe  of  the 
calvarium  and  in  the  bones  of  the  face. 

Chondroma. — Chondromas  are  lobulated  tumors,  composed  usually 
of  hyaline  cartilage,  and  are  enveloped  in  a  fibrous  capsule  which  sends 
prolongations  into  the  substance  of  the  growth.  They  are  usually  due 
to  some  disturbance  in  the  development  of  the  growing  bone  whereby 
portions  of  the  primitive  cartilage  become  displaced.  They  may  origi- 
nate in  the  periosteum  and  medulla  as  well  as  from  the  cartilage.  Accord- 
ing to  their  position  on  the  surface  of  the  bone  or  within  its  interior, 
we  may  make  a  division  analogous  to  that  of  the  osteomas,  into  ecchon- 
dromas  and  enchondromas.  They  are  found  most  frequently  in  children 
and  young  growing  persons,  and  may  be  congenital.  As  a  rule,  they  are 
multiple,  and  are  met  with  most  commonly  on  the  bones  of  the  hands 
and  lower  extremities,  less  frequently  on  the  trunk,  and  still  more 
rarely  on  the  calvarium.  They  are  particularly  liable  to  undergo  retro- 
gressive manifestations,  such  as  fatty  and  mucinous  degeneration 
(myxochondroma)  calcification,  and  liquefaction,  with  the  formation  of 
cysts.  Metaplasia  into  bone  not  infrequently  takes  place  (osteoid- 
chondroma,  osteochondroma).  Sarcomatous  transformation  is  also  rather 


FIBROMA  AND  MYXOMA  1047 

apt  to  occur  (chondrosarcoma  osteochondrosarcoma)  which  may  be 
associated  with  metastasis.  Simple  chrondromas,  it  should  be  re- 
membered, may  occasionally  produce  metastases  also.  In  this  case  the 
secondary  growths  reproduce  all  the  features  of  the  primary  growth, 
but  do  not  tend  to  invade  and  destroy  the  neighboring  tissues. 

FIG.  304 


Multiple  chondromas.     (From  the  surgical  clinic  of  the  Montreal  General  Hospital.)  t 

Fibroma. — Fibromas  are  derived  from  the  periosteum,  or,  more  rarely, 
from  the  bone-marrow.  They  are  found  most  frequently  in  connection 
with  the  bones  of  the  face  and  skull.  Those  occurring  in  the  nasal  and 
buccal  cavities  form  one  variety  of  nasopharyngeal  polyps  and  of  the 
growths  known  clinically  as  epulis.  They  are  met  with  less  commonly 
on  the  bones  of  the  trunk  and  still  more  rarely  in  the  extremities. 

Fibromas  form  nodular  tumors  of  varying  consistency,  which  under 
the  microscope  may  be  densely  fibrous  or  more  cellular,  so  that  they 
may  not  be  easily  distinguished  from  sarcomas,  into  which  they  im- 
perceptibly merge.  Occasionally,  they  are  more  vascular  and  contain 
large  blood  channels  and  spaces  (fibroma  teleangiectaticum).  Again,  they 
may  undergo  metaplasia  into  bone  (psteofibroma,  ossifying  fibroma). 

Myxoma  and  Fibromyxoma. — Myxoma  and  fibromyxoma  are,  on  the 
whole,  rare.  They  originate  from  the  periosteum  or  bone-marrow.  They 
frequently  undergo  liquefaction,  and  may  form  bone  (psteomyxoma),  or, 
again,  present  sarcomatous  transformation  (myxosarcomd).  They  may 
be  single  or  multiple. 


1048  THE  BONES 

Lipoma. — Lipomas  are  extremely  rare.  They  have  been  known  to 
arise  from  the  periosteum,  and  are  often  associated  with  striated  muscle 
fibers  (Sutton). 

Angioma. — Pure  angiomas  are  also  excessively  rare,  but  have  been 
described  in  connection  with  the  vertebrae  (Virchow),  the  calvarium, 
femur,  sternum,  and  palate  (Pe'an).  Combinations  of  angioma  with 
endothelioma,  chondroma,  and  osteoma  have  also  been  recorded. 

FIG.  305 


d     -    • 


Section  from  a  hemangio-endothelioma  of  bone,  a,  large  vascular  spaces  filled  with  erythro- 
cytes  and  surrounded  by  large,  clear,  cubical  endothelial  cells,  which  in  parts,  as  at  e,  form  solid 
masses;  6,  stroma;  d,  larger  and  c,  smaller  bloodvessels.  (Driessen.) 

The  secondary  tumors  of  the  bone  are  the  carcinoma  and  sarcoma. 
The  latter  are  rare  and  usually  of  the  melanotic  variety.  The  peri- 
thelial  angiosarcomas,  more  especially  those  of  the  thyroid,  kidney,  and 
suprarenal,  are  particularly  liable  to  produce  metastases  in  the  bones 
which  take  the  form  of  vascular  pulsating  growths,  strongly  suggesting 
aneurisms. 

Carcinoma  arises  by  the  direct  extension  of  a  carcinoma  of  the 
adjacent  soft  parts  or  by  metastasis,  and  forms  either  a  diffused  infiltra- 
tion or  a  nodular  growth.  Metastatic  carcinoma  is  said  to  be  most 
frequently  secondary  to  carcinoma  of  the  breast,  prostate,  thyroid, 
and  bronchi.  The  bones  involved,  according  to  v.  Recklinghausen, 
are,  in  order  of  frequency,  the  vertebrae,  femur,  ribs,  sternum,  humerus, 
and  cranium.  The  secondary  deposits  are  usually  to  be  found  in  those 
parts  of  the  bones  which  are  subject  to  the  greatest  traction  or  pressure. 
Retrograde  metastasis  to  the  head  of  the  humerus  is  occasionally  met 
with  in  carcinoma  of  the  breast. 

Carcinomatous  infiltration  is  usually  associated  with  a  marked  pro- 


CYSTS  1049 

liferation  of  the  periosteum  and  bone-marrow,  while  the  bone  itself 
undergoes  lacunar  resorption  and  finally  disintegration.  Occasionally, 
the  process  of  lacunar  resorption  is  associated  with  the  formation  of 
new  osteoid  tissue  devoid  of  lime  salts  (carcinomatous  osteomalacia)  or 
true  bone.  In  this  case  the  medullary  spaces  of  the  osteoid  or  bony 
substance  are  infiltrated  with  carcinoma  cells. 

FIG.  306 


Secondary  carcinoma  of  the  head  of  the  humerus.     (From  the  Pathological  Museum  of 

McGill  University.) 

Cysts. — Cysts  are  usually  due  to  softening  of  portions  of  solid  tumors, 
as  the  chondroma,  myxoma,  and  sarcoma.  Colliquative  cysts  are  some- 
times also  found  in  osteomalacia  and  osteitis  deformans. 

True  cystomas  are  rare  except  the  variety  known  as  the  dentigerous 
cystoma  found  in  the  maxilla.  This  is  supposed  to  originate  in  the  mis- 
placed matrix  of  a  tooth. 


THE  JOINTS  AND  CARTILAGES. 

A  joint,  or  diarthrosis,  is  an  association  of  two  or  more  bones  in  such 
a  way  that,  while  they  are  closely  approximated  and  held  together  by 


1050  THE  JOINTS 

a  capsule,  they  are  separated  by  a  space  so  as  to  permit  a  certain  amount 
of  movement.  The  capsule  is  composed  of  dense,  unyielding,  fibrous 
tissue,  lined  by  a  soft,  thin,  and  vascular  membrane  covered  with  flattened 
cells,  known  as  the  synovial  membrane.  The  cavity  contains  a  small 
quantity  of  limpid  fluid,  the  synovia. 

Pathological  changes  affecting  the  joints  may  originate  in  the  synovial 
sac  and  extend  to  the  articular  cartilage,  the  ends  of  the  bones,  and 
even  to  the  surrounding  soft  tissues,  or  arise  by  extension  from  the 
neighboring  structures. 


CONGENITAL  ANOMALIES. 

The  anomalies  of  development  occurring  in  the  joints  are  practically 
those  of  the  bones  themselves.  When  certain  bones  are  absent  or 
abnormal  the  associated  joints  are  necessarily  affected.  Of  interest 
from  the  point  of  view  of  orthopedic  surgery  are  such  conditions  as 

FIG.  307 


Double  club-foot  (talipes  varus).     (From  the  surgical  clinic  of  the  Montreal  General  Hospital.) 

genu  valgum  and  varum,  morbus  coxae,  club-foot,  spinal  curvatures,  and 
dislocations.  Some,  at  least,  of  the  cases  of  club-foot  appear  to  be  due 
to  antenatal  affections  of  the  spinal  cord,  e.  g.,  anterior  poliomyelitis. 
Genu  valgum  is  occasionally  a  stigma  of  general  infantilism. 

Congenital  Luxation. — Congenital  luxation  most  frequently  involves 
the  hip-joint.  It  is  due  to  hypoplasia  of  the  acetabulum  and  head 
of  the  femur,  or  to  abnormal  positions  of  the  limbs  and  excessive 
pressure  during  intra-uterine  life. 


INFLAMMATIONS  1051 


CIRCULATORY  DISTURBANCES. 

These  are  comparatively  unimportant.  Active  hyperemia  is  met  with 
in  the  early  stages  of  inflammation.  Passive  hyperemia  occurs  under 
the  same  conditions  as  elsewhere. 

Hemorrhage  into  a  joint  cavity  is  usually  due  to  trauma,  such  as  a 
sprain,  contusion,  dislocation,  or  fracture.  It  may  also  occur  in  the 
hemorrhagic  diatheses  and  as  a  neuropathic  manifestation.  Small 
effusions  quickly  disappear,  larger  ones  are  partly  absorbed,  and  the 
remaining  portions  are  substituted  by  fibrous  tissue  derived  from  the 
proliferation  of  the  cells  of  the  synovial  membrane.  The  cells  lining 
the  synovial  sac  also  proliferate  and  may  extend  over  the  clot.  In 
severe  cases  the  clot  may  not  be  entirely  absorbed  and  may  break  loose, 
forming  one  variety  of  "floating  body"  in  the  joint.  Not  infrequently, 
considerable  reactive  inflammation  is  set  up,  leading  to  the  production 
of  adhesions  and  fibrous  ankylosis. 


INFLAMMATIONS. 

Acute  inflammation  of  the  joints  may  involve  first  and  chiefly  the 
synovial  membrane — acute  synovitis — the  cartilages — chondritis — etc. 
In  severe  cases  not  only  the  joint,  but  the  bones  and  soft  tissues  may 
be  involved — osteitis  and  periarthritis. 

Acute  Arthritis  and  Synovitis.— These  disorders  may  be  produced 
by  direct  trauma  or  the  extension  of  inflammatory  processes  from 
structures  near  the  joints.  In  many  cases  there  is  a  hematogenic  origin. 
As  examples  of  the  first  form,  punctured  wounds,  gunshot  injuries,  and 
the  like  may  be  cited;  of  the  second,  the  joint  changes  in  infective 
osteomyelitis ;  of  the  third,  the  polyarthritis  of  acute  inflammatory  rheu- 
matism. The  hematogenic  forms  are  in  the  vast  majority  of  cases 
due  to  infective  microorganisms,  and  are  found  in  such  conditions  as 
inflammatory  rheumatism,  septicemia,  gonorrhoea,  scarlatina,  measles, 
typhoid  fever,  pneumonia,  erysipelas,  and  dysentery.  In  the  diseases 
just  mentioned,  the  inflammation  of  the  joints  is  in  some  cases  due  to 
the  specific  microorganism  of  the  disease  in  question,  but  not  infre- 
quently to  secondary  infection  with  pus-producing  cocci. 

In  the  milder  grades  of  inflammation,  and  in  the  early  stages  of  the 
severe  forms,  the  synovial  membrane  is  congested  and  swollen,  especially 
about  the  folds,  and  there  may  be  occasional  small  extravasations  of 
blood,  with  exudation  of  thin  yellowish  fluid  containing  a  few  delicate 
flocculi  of  fibrin  (serous  synovitis,  hydrops  articuli).  This  form  is  apt  to 
be  lighted  up  by  trauma,  such  as  sprains  or  contusions,  floating  bodies 
in  the  joints,  and  occurs  also  in  the  lighter  grades  of  inflammatory  rheu- 
matism, gonorrhoea,  and  osteomyelitis.  Swelling  of  the  joint  results, 
with  possibly  some  redness.  In  more  severe  cases  the  exudate  is  more 
abundantly  fibrinous  (synovitis  serofibrinosa),  and  in  some  instances 
the  exudate  may  be  mainly  fibrin,  with  but  little  fluid  (synovitis  fibrinosa 


1052  THE  JOINTS 

sive  sicca).  In  other  cases  the  effusion  may  become  purulent  or  may 
have  been  purulent  from  the  start  (synovitis  purulenta,  empyaema  articuli). 
Here  the  synovial  membrane  is  thickened  and  swollen,  infiltrated 
with  inflammatory  products,  and  covered  with  a  fibrinopurulent  or 
purulent  deposit,  while  the  cavity  of  the  joint  contains  a  variable  quantity 
of  turbid  fluid.  In  the  severest  forms  the  synovial  membrane  may  be 
partially  destroyed,  and  the  articular  cartilages  undergo  fatty  degenera- 
tion, fibrillation,  and  ultimately  necrosis.  The  whole  joint  may  in  time 

FIG.  308 


Chronic  osteoarthritis  of  the  knee  in  a  horse.     (From  the  Pathological  Museum  of 
McGill  University.) 

become  disorganized.  The  process  frequently  extends  to  the  exposed 
ends  of  the  bones,  the  soft  tissues,  and  along  the  lymphatics.  The  pus 
may  burrow  widely  along  the  lines  of  least  resistance,  and  general 
infection  may  be  set  up.  Osteomyelitis  or  lymphangitic  abscesses 
sometimes  also  result.  This  form  is  the  one  most  commonly  seen  as  a 
complication  of  pyemia,  measles,  scarlatina,  and  puerperal  infection. 

Acute  synovitis  and  arthritis  frequently  heal,  leaving  little  or  no 
trace,  or  may  pass  on  into  a  chronic  condition.  Not  infrequently, 
where  there  has  been  necrosis  of  the  structures  composing  the  joint, 
more  or  less  fibrous  proliferation  takes  place  in  the  process  of  repair, 


GOUT  1053 

and  osteophytic  outgrowths  may  form  about  the  joints,  so  that  ankylosis 
occasionally  results. 

A  peculiar  form  of  polyarthritis  is  occasionally  met  with  in  children, 
which  is  apparently  of  an  infective  nature.  It  runs  a  low  febrile  course, 
and  the  joint  manifestations  are  accompanied  by  enlargement  of  the 
lymph-nodes  and  spleen.  It  is  known  as  Still's  disease. 

Arthritis  Urica. — Arthritis  urica  (gout,  podagra)  is  a  form  of  acute 
inflammation  due  to  the  deposit  in  the  joint  and  adjacent  structures 
of  salts  of  uric  acid,  together  with  phosphate  and  carbonate  of  lime 
and  hippuric  acid.  These  salts  are  precipitated  in  the  form  of  fine 
needles  and  granules,  not  only  in  the  cells  and  matrix  of  the  cartilages, 
but  also  in  the  synovial  membranes,  ligaments,  and  soft  tissues.  This 
leads  to  irritation  and  inflammatory  infiltration,  with  the  exudation 
of  a  serous  fluid  into  the  joint.  The  metatarsophalangeal  joint  of  the 
great  toe  is  first  involved,  but  other  joints,  as  those  of  the  fingers,  hands, 
and  knees,  may  be  involved.  It  is  characteristic  of  the  disease  that 
it  tends  to  relapse  and  in  time  leads  to  chronic  changes  in  the  joints. 
Between  the  acute  paroxysms  the  pain  and  swelling  subside,  but  the 
chalk-like  deposit  still  remains  upon  the  cartilages.  In  severe  or  pro- 
longed cases  the  articular  cartilages  eventually  undergo  necrosis  and 
disintegrate,  the  synovial  membrane  becomes  thickened,  and  the  salts 
may  be  deposited  in  such  quantities  as  to  form  concretions  (chalk- 
stones,  tophi).  The  tendons,  periosteum,  and  bones  may  be  similarly 
involved.  The  deposit  may  be  so  great  that  the  soft  parts  necrose 
and  the  chalk  presents  externally.  Ulceration  and  abscess-formation 
are  common.  While  the  joint-changes  constitute  a  striking  picture 
in  the  disease,  they  form  only  one  aspect  of  the  affection,  which  is  a 
systemic  one,  due  to  disordered  metabolism.  Arterial  sclerosis,  digestive 
disorders,  skin  eruptions,  and  degenerative  changes  in  the  kidneys 
are  frequent  accompaniments. 

Chronic  Inflammations. — In  the  present  state  of  our  knowledge  it  is 
impossible  to  make  an  entirely  satisfactory  classification  of  the  chronic 
morbid  processes  affecting  the  joints.  There  are,  it  is  true,  certain 
broad  generalizations  which  enable  us  to  differentiate  anatomically 
several  forms  in  a  rough  way,  but  all  possible  combinations  and  inter- 
mediate gradations  exist.  Yet  we  are  often  in  the  dark  as  to  the 
etiological  factors  at  work  in  certain  cases,  and  even  when  these  are 
known,  they  at  times  give  rise  to  widely  differing  anatomical  pictures. 

In  general  we  may,  perhaps,  divide  these  processes  into  the  inflamma- 
tory and  the  degenerative.  The  former  group  includes  those  due  to  all 
forms  of  infection,  traumatism,  gout,  and  toxic  causes.  The  latter 
embraces  the  senile  and  neurotrophic  forms.  Inasmuch  as  in  the  latter 
group  the  inflammatory  features  are  comparatively  trifling  and,  indeed, 
sometimes  in  abeyance,  it  is  open  to  debate  whether  they  are  properly 
to  be  regarded  as  inflammatory  or  whether  they  should  not  be  classed 
with  the  retrogressive  metamorphoses.  The  leading  forms  will  be 
found  in  the  accompanying  table: 


1054 


THE  JOINTS 


Chronic  Arthritis  < 


A.  Inflammatory 


(a)  Exudative 


1.  Arthritis  serosa. 

2.  Arthritis  purulenta. 

3.  Arthritis  urica. 

4.  Specific:    tuberculo- 

sis, syphilis,  actin- 
omycosis. 


Arthritis 
etica. 


ankylop- 


f(a)  Senile  . 


ica. 
3.  Arthritis  deformans. 

Arthritis  ulcerosa  sicca. 


I B.  Degenerative 


f  1.  Tabes  dorsalis. 

(b)Neurotrophicin\  *'  S5™gomyelia. 

j  3.  Acute  antenor  polio 
L          myelitis. 


Chronic  Serous  Arthritis. — Chronic  serous  arthritis  is  a  somewhat 
sluggish  affection  characterized  by  the  effusion  of  a  thin  serous  exudate. 
The  affection  results  from  an  acute  or,  more  often,  a  recurring  synovitis. 
The  knee  is  affected  most  frequently,  and  next,  the  shoulders,  hips, 
and  elbows.  The  lesions  may  be  bilateral  or  unilateral.  The  effusion 
in  some  instances  may  be  so  great  that  the  synovial  membrane  is  forced 
out  through  the  fibers  of  the  capsule  in  the  form  of  hernial  protrusions. 
The  changes  in  the  synovial  membrane  and  the  cartilages  are  often 
comparatively  trifling,  but  in  long-standing  cases  the  capsule  may  be- 
come thickened  and  the  cartilages  fibrillated.  The  folds  and  fringes 
of  the  synovial  membrane  are  enlarged  and  vascularized,  and  some- 
times extend  as  a  pannus  over  the  articular  surfaces.  The  affection 
frequently  follows  exposure  to  cold  in  those  of  a  rheumatic  tendency, 
but  often  also  traumatism,  as  in  contusions,  sprains,  and  the  incarcera- 
tion of  synovial  fringes  or  free  bodies  in  the  joint. 

Chronic  Suppurative  Arthritis. — Chronic  suppurative  arthritis  results 
from  hematogenic  infection,  traumatism,  or  the  extension  of  disease 
from  the  adjacent  parts.  The  cavity  of  the  joint  is  filled  with  purulent 
or  seropurulent  fluid,  and  there  is  a  deposit  of  fibrin  and  pus  on  the 
synovial  membrane  and  the  surface  of  the  cartilages.  The  cartilages 
show  cloudiness,  fibrillation,  and  various  grades  of  degeneration,  amount- 
ing sometimes  to  necrosis.  The  synovial  membrane  and  the  capsule  are 
infiltrated  with  inflammatory  products.  Not  infrequently,  the  inflam- 
mation extends  to  the  bone  and  the  surrounding  soft  tissues.  In  this 
way  caries  and  necrosis  may  be  brought  about  and  abscess-formation 
in  the  capsule  and  its  neighborhood.  Healing  takes  place  by  the  exfolia- 
tion of  dead  bone  and  cartilage,  the  absorption  of  the  exudate  and  its 
discharge,  the  production  of  new  bone  from  the  periosteum  and  bone- 
marrow.  Fibrous  and  bony  ankylosis  not  infrequently  result. 

Chronic  Gouty  Arthritis. — Chronic  gouty  arthritis  has  been  sufficiently 
referred  to  in  discussing  the  acute  form. 

Tuberculosis. — Tuberculous  arthritis,  in  so  far  as  the  clinical 
features  are  concerned,  may  occur  as  a  primary  disease.  In  this  case 


TUBERCULOSIS  1055 

the  infection  is  hematogenic  in  origin.  How  this  is  brought  about  is 
not  clear,  but  it  is  possible  that  the  bacilli  of  tuberculosis  may  enter 
at  some  point  in  the  respiratory  or  alimentary  tracts,  and,  without  causing 
a  local  lesion  at  the  point  of  invasion,  may  on  occasion  be  carried  to 
some  distant  point,  as,  for  instance,  a  joint.  It  is  much  more  probable, 
however,  that  there  is  some  local  focus,  either  obvious  or  concealed, 
from  which  the  infection  proceeds.  As  has  been  demonstrated  con- 
clusively by  the  experiments  of  Schiiller  and  Krause,  among  others, 
a  slight  injury  to  a  bone  or  joint  will  determine  the  localization  of  the 
germs  at  that  point.  In  the  majority  of  cases,  two-thirds  according 
to  Krause  and  Konig,  the  affection  of  the  joints  arises  by  the  extension 
of  previously  existing  tuberculous  disease  of  the  bones.  In  discussing 
tuberculosis  of  bone,  it  was  pointed  out  that  the  disease  most  frequently 
occurs  at  the  ends  near  the  epiphyseal  junction,  so  that  involvement 
of  the  joint  is  readily  brought  about.  In  other  cases  it  is  probable 
that  infection  takes  place  by  way  of  the  lymphatics.  The  progress 
of  the  affection  is  characteristically  sluggish,  and,  while  often  unattended 
by  distressing  subjective  symptoms,  may  in  time  lead  to  destruction 
and  complete  disorganization  of  the  joint.  The  disease  is  preeminently 
one  of  the  developmental  period  of  life,  for,  according  to  Gibney,  about 
84  per  cent,  of  cases  are  found  in  persons  under  fourteen  years  of  age. 

It  usually  begins  by  the  production  of  minute  tubercles,  which  in 
course  of  time  increase  considerably  in  size  and  number.  In  acute 
miliary  tuberculosis  of  the  synovial  membrane,  which  is  but  one  mani- 
festation out  of  many  of  a  systemic  distribution  of  the  bacilli,  com- 
paratively few  tubercles  may  be  produced,  and  there  may  be  little  or 
no  inflammatory  reaction  of  any  moment  in  the  joint. 

In  other  cases,  where  the  foci  are  more  numerous,  the  synovial  mem- 
brane is  reddened,  swollen,  infiltrated  with  inflammatory  products,  and 
is  converted  into  a  soft  grayish-red  granulation  tissue  containing  abun- 
dant tubercles  (arthritis  granulosa).  There  is  frequently  an  exudation 
into  the  joint  cavity  of  a  serous  (hydrops  articuli  tuberculosus) ,  sero- 
fibrinous,  fibrinopurulent,  or  purulent  exudate  (empycema  tuber  culosum). 
More  or  less  abundant  shreds  and  flakes  of  fibrin  cover  the  granulation, 
and  the  so-called  "rice-bodies"  (corpora  oryzoidea)  may  be  found  in 
the  joint.  These  bodies  are  smooth,  soft,  and  rather  elastic,  of  a  gelati- 
nous, whitish  or  grayish-white  appearance.  On  section  they  present 
a  concentric  lamination.  They  are  supposed  to  be  derived  from  fibrin 
or  bits  of  synovial  fringe  that  have  become  detached  and  have  under- 
gone hyaline  degeneration.  The  synovial  membrane  may  undergo 
a  simple  inflammatory  proliferation  and  may  extend  into  the  joint 
in  the  form  of  folds  or  villi  of  an  cedematous  or  gelatinous  appearance. 
Or,  again,  there  may  be  papillary  or  polypoid  thickenings  of  the 
membrane.  Tubercles  situated  beneath  the  synovial  membrane  may 
in  the  course  of  their  growth  invade  the  cavity  of  the  joint  (arthritis 
nodosa  or  tuberosd).  In  other  cases  the  tubercles  are  extremely 
minute,  perhaps  not  visible  to  the  naked  ^eye,  and  the  membrane  is 
only  slightly  thickened.  A  layer  of  vascular  granulation  tissue  is 


1056  THE  JOINTS 

formed  from  the  synovial  sac  and  spreads  gradually  over  the  articular 
surfaces,  converting  the  cartilage  into  connective  tissue  (synovitis  pan- 
nosa).  In  cases  where  the  granulation  tissue  extends  to  the  cartilages 
and  remains  in  contact  with  them,  disintegration  and  absorption  of  the 
cartilage  takes  place,  with  destruction  of  the  cartilage  cells  and  the 
invasion  of  the  cell  capsules  with  inflammatory  leukocytes.  Later, 
the  cartilage  may  be  invaded  by  vessels,  and  undergoes  a  patchy  and 
mucinous  degeneration  or  is  converted  into  loose  fibrous  tissue.  Simul- 
taneously, resorption  of  the  neighboring  bone  may  take  place.  The 
advancing  layer  of  the  granulation  tissue  encroaches  upon  the  interior 
at  the  edge  of  the  cartilages,  gradually  dissecting  them  away  from  the 
deeper  structures  and  finally  extending  to  the  bone  and  the  medullary 
cavity.  The  medullary  substance  loses  its  fat  and  is  converted  into 
a  vascular  lymphoid  marrow. 

As  a  consequence  of  these  changes,  the  joint  becomes  enlarged  and 
presents  a  pale,  smooth,  rather  shiny  appearance  (white  swelling). 
Sooner  or  later,  the  process  extends  from  the  cartilage  and  bone  to  the 
surrounding  soft  tissues.  Caseous  nodules  are  formed  which  coalesce 
and  break  down  into  tuberculous  or  "cold"  abscesses.  These  may 
enlarge  and  burrow  their  way  to  the  surface,  and  fistulae  result.  The 
joint  may  be  disorganized  to  such  an  extent  that  dislocation  of  the 
bones  takes  place.  When  the  process  tends  to  heal,  numerous  osteo- 
phytes  are  produced  in  the  neighborhood  of  the  diseased  part,  and 
fibrous  or  fibroosseous  ankylosis  may  result. 

Comparatively  few  bacilli  are  to  be  found  in  the  effusion,  but  it  is 
usually  possible  to  produce  tuberculosis  in  susceptible  animals  by  the 
injection  of  the  fluid.  Further,  as  the  studies  of  Lartigau  have  demon- 
strated, bacilli  may  be  detected  in  a  joint,  even  where  the  disease  has 
apparently  healed,  and  may  be  of  a  comparatively  high  grade  of  viru- 
lence. Thus,  the  disease  may  be  disseminated  to  distant  parts  or  break 
out  again  after  months  or  years.  The  joints  usually  affected  are  the 
hip,  vertebrae,  knee,  ankle,  shoulder,  elbow,  tarsus,  and  carpus. 

Syphilis.  —  In  congenital  syphilis  an  exudative  arthritis,  associated 
with  thickening  of  the  capsule  and  disintegration  of  the  cartilages, 
occurs  as  a  primary  affection  or  follows  osteochondritis.  Gummas  in 
neighboring  parts  may  extend  to  the  joint. 

In  acquired  syphilis  the  joint  changes  may  appear  during  the  period 
of  eruption  or  in  the  later  stages.  In  the  first  event  there  is  produced 
a  diffuse  serous  synovitis,  not  unlike  that  of  acute  rheumatism.  Rarely, 
a  similar  state  of  things  may  be  observed  in  late  syphilis,  but  it  is  more 
common  to  find  gummatous  infiltration  of  the  capsule  with  thickening 
of  the  capsule  and  synovial  sheath,  together  with  erosion  and  fibrilla- 
tion of  the  cartilages.  These  changes  may  be  primary  or  may  be 
secondary  to  specific  inflammation  of  the  periosteum  and  bone-marrow. 

Actinomycosis. — Primary  actinomycosis  of  the  joints  is  unknown. 
The  affection  usually  arises  by  metastasis  or  extension  from  neighbor- 
ing parts.  The  cervical  vertebrae,  the  elbow,  the  tibiotarsal,  and  hip- 
joint  have  been  found  involved. 


ARTHRITIS  DEFORMANS  1057 

Arthritis  ankylopoetica  chronica  is  a  peculiar  anatomical  form  of 
arthritis  due  to  various  etiological  causes.  The  main  features  are  the 
formation  of  adhesions  between  the  cartilages  with  vascularization  and 
fibrous  transformation  of  the  cartilages.  The  condition  may  result 
from  acute  exudative  inflammation,  and  tuberculosis  or  other  chronic 
destructive  affections. 

In  the  early  stages  the  synovial  membrane  is  somewhat  injected  and 
thickened,  while  the  surface  of  the  cartilages  is  rough  and  fibrillated. 
The  cartilages  are  here  and  there  vascularized  and  the  opposing  surfaces 
are  more  or  less  adherent.  The  deeper  layers  of  the  cartilages  are  also 
gradually  converted  into  medullary  spaces  through  resorption  brought 
about  by  the  encroachment  of  the  subchondral  medullary  substance. 
Some  of  the  islets  of  cartilage  thus  isolated  may  in  time  be  converted 
into  bone.  Thus,  the  cartilages  are  gradually  transformed  into  a  vascular 
fibrous  or  fibroosseous  tissue.  The  joint  cavity  is  traversed  by  dense 
fibrous  bands  and  is  converted  into  a  number  of  small  spaces,  bounded 
by  dense  fibrous  tissue  and  containing  synovial  fluid.  In  very  advanced 
cases  the  whole  of  the  articular  cartilage  may  disappear  and  be  replaced 
by  fibrous  tissue,  so  that  the  original  structure  of  the  joint  becomes  well- 
nigh  unrecognizable.  Not  only  so,  but  the  newly  formed  fibrous  tissue 
may  in  time  be  converted  into  a  mass  of  spongy  bone  and  complete 
osseous  ankylosis  result. 

Polyarthritis  Chronica  Rheumatica. — The  disease  commonly  known 
as  chronic  rheumatism,  or  polyarthritis  chronica  rheumatica,  is  of  some- 
what uncertain  etiology.  It  is  found  commonly  in  old  persons,  and 
may  result  from  repeated  attacks  of  acute  rheumatism  or  may  come 
on  insidiously.  It  usually  begins  in  the  phalanges  or  metacarpal  and 
carpal  bones,  but  the  larger  joints,  such  as  the  knees  and  ankles,  are 
not  infrequently  involved.  The  disease  is  usually  steadily  progressive, 
chronic  in  its  course,  occasionally  with  acute  exacerbations,  and  leads 
to  more  or  less  limitation  of  movement.  Proliferation  of  bone  is  rather 
marked,  so  that  the  affection  presents  a  close  resemblance  to  arthritis 
deformans. 

Arthritis  Deformans. — Arthritis  deformans  (rheumatoid  arthritis,  rheu- 
matic gout,  arthrite  seche)  is  a  rather  common  disease  of  the  joints, 
characterized,  on  the  one  hand,  by  degenerative  changes  in  the  articular 
cartilages  and  bone,  and,  on  the  other,  by  a  marked  production  of  new 
bone.  The  disease  may  be  monarticular,  but  is  more  usually  poly- 
articular.  It  has  been  known  to  follow  repeated  traumatic  insults, 
fractures,  or  infectious  disease,  or,  rarely,  is  spontaneous.  The  affection 
begins  usually  in  the  third  decade  of  life  or  later,  and  may  run  a  chronic 
course  for  many  years,  with  occasional  acute  exacerbations.  It  has 
been  known,  however,  to  attack  young  children.1  Women  are  more 
commonly  affected  than  men  (1  to  5).  The  etiology  is  quite  obscure. 
Some  have  regarded  it  as  neuropathic  in  nature,  on  the  analogy  of 

1  Nicholls,  Rheumatoid  Arthritis  in  Young  Children,  Montreal  Medical  Journal, 
25:1896-97:97. 
67 


1058  THE  JOINTS 

Charcot's  joints.  This  is  supported  by  the  fact  that  muscular  wasting, 
contractures,  glossiness  of  the  skin,  and  paresthesise  are  frequently 
observed.  Others  regard  it  as  a  degenerative  process,  a  mark  of  actual 
or  premature  senility.  Certain  recent  observers  have  found  in  the 
synovial  fluid  of  these  cases  cocci,  which  they,  on  experimental  grounds, 
believe  to  be  specific.  The  proof  of  this,  however,  is  not  as  yet  conclu- 
sive. Considering  the  fact  that  certain  cases  begin  acutely  or  present 
acute  exacerbations,  it  is  not  impossible  that  some  at  least  are  infective 
in  nature.  The  close  relationship  to  gout,  which  used  to  be  insisted 
upon,  is  now  no  longer  accepted.  Lately,  the  view  has  been  advanced 
that  arthritis  deformans  is  due  to  a  toxin  of  enterogenous  nature. 

Anatomically,  the  lesions  produced  may  be  summed  up  as  degenera- 
tion of  the  cartilages,  together  with  the  formation  of  new  bone-marrow 
and  osteophytes.  Considerable  deformity  of  the  joints  results,  with 
more  or  less  complete  limitation  of  movement.  The  superficial  layers 
of  the  articular  cartilages  become  fibrillated  and  fissured,  while  in  the 
deeper  parts  there  are  areas  of  necrosis  and  softening.  Further,  owing 
to  some  stimulation,  the  bone-marrow  begins  to  proliferate  and  a  new 
vascularized  marrow  invades  the  deeper  layers  of  the  cartilage  and 
grows  into  the  degenerated  areas  just  referred  to.  The  intervening 
cartilage  that  remains  is  gradually  converted  into  osteoid  tissue  and 
eventually  into  bone.  Sometimes  there  is  an  overgrowth  of  the  cartilage, 
leading  to  the  formation  of  nodular  excrescences,  which  may  in  time 
project  into  the  medullary  spaces.  Coincidently  with  the  changes 
just  described,  the  joint  capsules  and  the  synovial  membranes  become 
thickened.  Occasionally,  the  folds  and  fringes  of  the  latter  encroach 
upon  the  joint  cavity  and  are  infiltrated  with  fat,  forming  the  so-called 
lipoma  arborescens.  Small  portions  of  the  altered  synovial  membrane 
may  be  converted  into  cartilage  or  bone.  Should  these  break  loose, 
they  form  free  bodies  in  the  joint.  In  the  bone  itself  noteworthy  changes 
take  place,  in  the  form  of  resorption  of  the  trabeculse,  resulting  in  con- 
siderable loss  of  substance  and  alterations  in  the  shape  of  the  bone. 
In  course  of  time  the  newly  formed  osteoid  tissue  in  its  turn  undergoes 
similar  retrogressive  changes.  The  marrow  of  that  part  of  the  bone 
near  the  joint  loses  much  of  its  fat  and  is  converted  into  gelatinous  or 
lymphoid  marrow.  If  the  resorption  of  the  bone  be  extensive,  it  may 
be  more  or  less  substituted  by  gelatinous  fibrous  tissue.  In  other 
cases  the  marrow  softens  and  liquefies,  producing  small  cysts.  The 
tissues  bounding  these  cysts  may  proliferate  and  produce  new  bone 
by  metaplasia. 

In  course  of  time  extensive  changes  in  the  size  and  contour  of.  the 
bone  take  place.  In  the  case  of  the  hip-joint,  for  example,  the  neck 
of  the  femur  is  more  or  less  shortened,  the  head  is  enlarged,  and  nodular 
excrescences  or  osteophytes  form  about  it  and  around  the  edge  of  the 
acetabulum.  Where  the  cartilage  is  completely  absorbed  the  exposed 
bone,  owing  to  pressure  and  friction,  becomes  flattened,  smooth,  and 
eburnated.  Where  cysts  in  the  bone  are  laid  bare  by  erosion,  irregular 
grooves  and  depressions  are  formed  and  the  bony  surfaces  not  in  con- 


PLATE   XV 


X-ray   Photograph   of   the   Hand,   showing    the   changes    in    the 
bones   resulting   from    rheumatoid   arthritis. 

The  dislocation  of  the   phalanges  with  erosion  and  osteophytie 
outgrowths  are  well  seen. 


(From  Dr.  Finley's  Clinic,  Montreal  General  Hospital.) 


ARTHRITIS  CHRONICA   ULCEROSA  SICCA  1059 

tact  are  covered  by  extensions  of  the  synovial  membranes.  In  this 
way  marked  deformity  of  the  articulations  results.  Ankylosis  is  a 
common  event  due  to  thickening  of  the  capsule  and  the  formation  of 
bony  outgrowths  in  the  capsule  and  along  the  edge  of  the  bone.  A 
common  deformity  is  the  ulnar  deflection  seen  in  the  fingers.  Dis- 
locations of  various  kinds  occur. 

FIG.  309 


Spondylitis  deformans;  curvature  of  the  spine,  with  ankylosis  due  to  subperiosteal 
osteogenesis.     (Pathological  Museum,  McGill  University.) 

Arthritis  deformans  oftenest  attacks  the  hip-joint  and  the  knees,  but  is 
frequently  found  in  the  shoulders  and  elbows  and  in  the  smaller  joints  of 
the  hands  and  feet.  When  the  vertebral  column  is  involved  the  condition 
is  known  as  spondylitis  deformans.  This  often  leads  to  bowing  of  the 
trunk  forward,  with  limitation  of  movement. 

Arthritis  Chronica  Ulcerosa  Sicca.— Arthritis  chronica  ulcerosa 
sicca,  or  senile  arthritis,  is  in  some  ways  not  unlike  arthritis  deformans, 
but  the  degenerative  side  of  the  process  is  more  marked  while  the  pro- 


1060  THE  JOINTS 

liferation  of  bone  and  cartilage  is  less  obtrusive.  As  its  name  implies, 
it  is  found  in  advanced  life,  and  is  to  be  regarded  as  a  degenerative 
process  resulting  from  deficient  nutrition.  It  occasionally  follows 
rheumatic  and  other  inflammatory  disturbances,  and  has  been  known 
to  occur  where  the  bones  have  been  kept  for  a  long  time  in  one  position. 
In  other  cases  it  is  a  neurotrophic  disturbance.  Most  commonly  the 
hip-joint  is  affected  (morbus  coxoe  senilis),  but  the  shoulder,  elbow, 
finger-joints,  and  patella  may  be  attacked. 

FIG.  310 


Charcot's  joint.     The  illustration  shows  also  very  well  the  hyperextension  of  the  leg  on  the  thigh 
from  laxness  of  the  joint.     (From  the  Medical  Clinic  of  the  Montreal  General  Hospital.) 

The  lesions  found  consist  in  fibrillation  and  fissuring  of  the  articular 
cartilages  with  some  superficial  erosion.  At  the  periphery,  where  the 
synovial  membrane  is  attached,  the  cartilage  undergoes  gelatinous  and 
fibrous  transformation,  and  may  even  disappear.  In  advanced  cases  the 
whole  of  the  articular  cartilages  may  be  lost,  so  that  the  underlying  bone 
is  laid  bare.  This  in  turn  may  be  eroded,  or  become  compacted  and 
eburnated.  The  capsules  and  synovial  membranes  are  frequently  thick- 
ened, leading  to  capsular  ankylosis.  Occasionally,  instead  of  this,  the 


CHARCOT'S  JOINTS  1061 

fibrous  bands  undergo  necrosis  and  disintegration.  Calcification  and 
amyloid  transformation  are  sometimes  to  be  noticed  in  the  degenerating 
cartilage,  in  the  fibrous  capsule,  and  adhesions. 

Neuropathic  Joints. — The  neurotrophic  changes  in  joints  are  of  con- 
siderable clinical  interest  and  importance.  They  are  found  chiefly  in 
tabes  dorsalis  (Charcot's  joints),  syringomyelia,  anterior  poliomyelitis, 
compression  and  destruction  of  the  spinal  cord,  and  after  the  severance 
of  nerves.  As  a  rule,  the  condition  comes  on  quickly,  without  pain,  and 
leads  to  great  enlargement  of  the  joints,  owing  to  the  accumulation  of  fluid 
within  the  synovial  sac.  Degenerative  changes  are  in  excess  and  quickly 
bring  about  disintegration  of  the  joint.  Besides  this,  there  are  atrophy 
of  the  ends  of  the  bones,  and  sometimes  osteoporosis,  with  erosion  and 
thickening  of  the  membranes.  Spontaneous  luxation  is  common.  In 
tabes,  the  lesion  is  found  chiefly  in  the  lower  extremities,  in  the  knee  or 
hip,  but  the  shoulder,  elbow,  and  smaller  joints  of  the  hands  and  feet 
do  not  escape.  The  condition  is  single  or  multiple.  In  syringomyelia 
the  lesion  occurs  chiefly  in  the  upper  extremities,  depending  upon  the 
site  of  the  disease  in  the  cord. 

Parasites. — Echinococcus  disease  may  invade  the  joints  from  the 
neighboring  parts. 


RETROGRESSIVE  METAMORPHOSES. 

Degenerative  changes  may  affect  the  articular  surfaces  or  the  investing 
membranes. 

Degeneration.  —  Fatty  Degeneration.  — Fatty  degeneration  of  the 
cartilages  is  not  uncommon.  It  occurs  in  general  marasmus  and  as  a 
senile  change,  from  interference  with  the  circulation,  and  from  inflam- 
mation. The  fat-globules  are  deposited  within  the  cells. 

Hyaline  Degeneration. — In  hyaline  degeneration  the  capsule  may  be 
involved  as  well  as  the  cartilage  and  its  cells.  The  cartilage  is  con- 
verted into  a  homogeneous  semitranslucent  mass,  or  breaks  up  into 
flakes. 

Amyloid  transformation  is  also  met  with. 

Mucoid  Degeneration. — The  so-called  mucoid  degeneration  is  a  peculiar 
change  found  principally  in  the  costal  cartilages  of  old  persons  and  also 
upon  the  articular  surfaces  of  the  long  bones.  The  cement  substance 
of  the  cartilage  liquefies  and  the  fibrillae  of  the  matrix  are  dissociated 
or  separated  into  bundles,  giving  the  cartilage  a  curious  fibrillated 
structure.  In  advanced  cases  the  cartilage  may  break  up  into  clumps 
or  fine  granular  detritus.  The  cartilage  cells  are  in  part  destroyed, 
but  those  remaining  proliferate,  so  that  small  clusters  may  be  seen  lying 
within  the  same  limiting  membrane.  At  first  the  cartilage  is  gray  and 
transparent,  but  later  becomes  streaked,  opaque,  or,  if  calcification 
occur,  dense  and  white.  The  softening  may  proceed  to  such  a  degree 
that  cysts  filled  with  fluid  are  formed.  The  process  of  softening  is 
frequently  met  with  in  old  age,  and  is  an  important  feature  in  connec- 


1062  THE  JOINTS 

tion  with  chronic  arthritis.  If  the  degenerated  areas  are  near  the 
medullary  cavity  or  the  perichondrium,  vessels  may  grow  into  them  with 
proliferation  of  cells,  so  that  the  destroyed  parts  are  substituted  by 
fibrous  tissue,  bone-marrow,  and  eventually,  in  some  cases,  with  bone. 
Besides  the  mucinous  changes,  cartilage  may  undergo  a  retrograde 
metaplasia  into  fibrocartilage  and  fibrous  tissue.  This  is  common  in 
connection  with  chronic  inflammation. 

Necrosis  and  Caries. — Necrosis  and  caries  of  the  cartilage  occur 
with  suppurative  and  other  forms  of  inflammation. 

Pigmentation. — Pigmentation  of  the  cartilages  is  usually  due  to  the 
absorption  of  the  hematoidin  from  blood  effused  within  the  cavity. 

Ochronosis  is  a  peculiar  and  rare  condition,  in  which  the  cartilage 
assumes  a  brownish  or  blackish  hue.  The  cause  is  unknown. 

Infiltration. — In  gout  there  is  a  deposit  within  the  cartilages  and  the 
capsule  of  needles  of  urates.  The  capsule  of  the  joints  may  undergo  all 
the  degenerative  changes  just  described  as  affecting  the  cartilage. 

INJURIES  AND  THEIR  REPAIR. 

When  a  cartilage  is  injured  there  is  invariably  more  or  less  degenera- 
tion of  the  specific  cells  in  the  form  of  swelling,  vacuolation,  albuminous 
and  fatty  degeneration,  and  even  necrosis.  The  regenerative  power 
of  cartilage  is  slight,  and  only  in  rare  cases  and  in  young  persons  is 
new  cartilage  formed.  The  repair  is  made  by  cartilage  or  bone.  In 
the  event  of  fracture,  restoration  is  brought  about  by  proliferation  of  the 
cells  of  the  perichondrium  producing  fibrous  tissue  and  bone.  In  the 
case  of  the  articular  cartilages,  any  loss  of  substance  is,  as  a  rule,  only 
imperfectly  made  good.  Here  repair  takes  place  by  means  of  fibrous 
tissue.  Portions  of  the  articular  and  semilunar  cartilages,  if  they  break 
loose,  may  become  free  in  the  joint  cavity. 

Joints  may  be  injured  by  puncture,  contusion,  torsion,  dislocation, 
subluxation,  or  fracture  of  the  bones  within  the  capsule.  The  amount 
of  injury,  of  course,  varies.  The  capsule  may  be  crushed,  pulled  upon, 
or  actually  torn. 

In  a  dislocation  the  end  of  one  of  the  bones  forming  a  joint  is  found 
in  an  abnormal  position  outside  the  capsule.  In  subluxation  the  dislo- 
cation is  only  partial.  In  some  cases,  the  articular  cartilages  or  the  end 
of  the  bone,  may  be  injured  or  fractured  (complicated  dislocation). 

The  results  of  such  accidents  depend  largely  upon  whether  infec- 
tion takes  place  or  not.  In  the  more  trifling  injuries  recovery  takes 
place,  and  restoration  of  function  is  eventually  complete.  In  severer 
traumatism  the  changes  initiated  are  not  unlike  those  occurring  in  frac- 
ture of  the  bones.  Immediately  following  a  dislocation  or  intracapsular 
fracture  there  is  more  or  less  abundant  hemorrhage  into  the  joint.  Later, 
inflammation  sets  in.  The  capsule  and  surrounding  parts  become  swollen, 
cedematous,  infiltrated  with  inflammatory  products,  and  there  may  be 
effusion  into  the  synovial  cavity.  In  simple  non-infected  cases  the  inflam- 
mation rapidly  reaches  its  height  and  then  resolves.  The  effused  blood 


INJURIES  AND  THEIR  REPAIR  1063 

and  the  inflammatory  exudate  are  absorbed.  In  rare  cases  it  happens 
that  portions  of  the  coagulum  may  persist  and  become  organized,  forming 
one  variety  of  "free  bodies"  in  the  joints.  When  the  dislocation  is  re- 
duced, the  tear  in  the  capsule  is  repaired  by  the  formation  of  a  cellular 
material  which  in  time  becomes  differentiated  into  dense  connective  tissue. 
Should  fracture  have  taken  place,  it  heals,  as  do  ordinary  fractures  of  bone 
and  cartilage.  In  time  the  joint  may  return  to  its  normal  anatomical 
condition  and  function  be  perfectly  restored.  Should  reposition  not  be 
effected,  the  capsule  and  ligaments  contract,  the  articular  surfaces 
waste  away,  the  joint-cavity  is  invaded  by  fibrous  tissue,  and  the  dis- 
used muscles  atrophy.  The  end  of  the  dislocated  bone  will  atrophy 
or  become  attached  in  its  abnormal  situation  by  fibrous  tissue.  Should 
it  rub  against  a  bony  surface,  a  new  articulation  in  the  form  of  a  shallow 
socket  is  in  time  produced.  This  is  in  part  due  to  pressure  atrophy. 
Round  about  the  newly  formed  groove  the  bone  proliferates  and  forms 
a  ring.  In  this  way  a  more  or  less  perfectly  functionating  joint  is 
produced.  Useless  muscles  atrophy,  others  become  elongated,  and  the 
soft  tissues  in  time  adapt  themselves  to  the  altered  condition  of  things. 
In  some  cases  a  new  capsule  is  formed  by  the  proliferation  of  cells 
derived  from  the  original  capsule  or  from  the  soft  tissues  in  the  neighbor- 
hood. If  the  dislocated  bone  be  not  movable,  partial  or  complete 
fibrous  or  fibrous  and  osseous  union  takes  place  (ankylosis). 

Where  a  joint  is  resected  the  result  varies  according  to  circumstances. 
The  ends  of  the  bones  at  the  point  of  injury  become  rounded  off  and 
a  proliferation  of  connective  tissue  from  the  periosteum  and  bone- 
marrow  takes  place,  so  that  the  two  ends  are  united  by  fibrous  bands, 
in  which  bone  sometimes  develops.  If  the  parts  be  kept  at  rest,  a  strong 
immobile  union  takes  place.  If,  however,  motion  be  kept  up,  designedly 
or  otherwise,  a  new  joint  results.  In  young  persons  articular  cartilages 
may  be  formed.  In  time,  the  parts  return  in  a  considerable  degree 
to  their  normal  appearance  and  a  capsule  secreting  synovia  may  even 
be  produced. 

As  a  result  of  injuries  and  some  forms  of  inflammation  a  joint  may 
become  fixed — ankylosis.  If  completely  immobile,  the  condition  is 
called  true  or  complete  ankylosis ;  if  partially  movable,  false  or  incomplete 
ankylosis.  Ankylosis  is  usually  brought  about  by  the  proliferation 
of  fibrous  tissue  into  or  around  the  region  of  injury.  Should  the  intra- 
articular  cartilages  be  preserved  in  whole  or  in  part,  they  sometimes 
undergo  direct  metaplasia  into  fibrous  tissue  or  fibrocartilage,  or  into 
mucinous  tissue,  which  gradually  becomes  fibrous.  In  some  cases 
the  newly  formed  fibrous  tissue  is  trifling  in  amount  and  the  ankylosis 
is  produced  mainly  by  cartilage  or  bone.  In  other  cases  the  ankylosis 
is  due  to  fibrous  tissue  or  cartilage  and  bone  intermingled.  In  still 
another  class  of  cases,  the  interference  with  mobility  is  due  to  some 
cause  outside  the  capsule  (extracapsular  ankylosis),  such  as,  abundance 
of  osteophytes,  for  example,  in  arthritis  deformans;  thickening  and 
contraction  of  the  capsule;  adhesions  of  tendons;  the  formation  of 
bony  bridges;  contractions  and  paralyses  of  muscles. 


1064  THE  JOINTS 


PROGRESSIVE  METAMORPHOSES. 

Metaplasia. — The  phenomenon  of  metaplasia  is  frequently  exempli- 
fied in  the  case  of  cartilage.  Cartilage  is  rather  an  inert  tissue,  and 
when  destroyed  is  apt  to  be  replaced  by  fibrous  tissue  or  occasionally 
bone.  Ordinary  hyaline  cartilage  in  the  joints  may  be  transformed 
into  fibrocartilage  or  fibrous  tissue  in  certain  cases  of  inflammation. 
Mucinous  metaplasia  is  very  common.  Transformation  into  bone 
occurs  both  in  inflammation  and  as  a  senile  change. 

Hyperplasia. — Hyperplasia,  both  of  the  cartilages  and  the  connective 
tissue  of  joints,  is  a  frequent  event  in  most  forms  of  chronic  inflamma- 
tion, especially  in  tuberculosis  and  arthritis  deformans.  Not  only 
may  the  fibrous  capsule  and  its  synovial  lining  be  diffusely  thickened, 
but  new  cartilage  and  bone  may  be  developed.  The  cartilages  may 
also  be  diffusely  thickened,  or  nodular  and  papillary  excrescences  may 
be  formed.  Overgrowth  of  the  cartilage  is  seen  also  in  the  hyperplastic 
form  of  that  rare  disease  called  by  Kauffmann  chondrodystrophia  foetalis. 

Tumors. — Primary  tumors  of  the  joints  are  rare.  A  curious  form 
is  the  so-called  lipoma  arborescens,  which  is  rather  common  in  cases  of 
tuberculosis  and  arthritis  deformans.  Here  the  synovial  membrane 
becomes  thickened  and  hypertrophic,  and  is  thrown  into  numerous 
folds  and  papillae,  in  which  fat  is  subsequently  deposited.  Sarcoma 
may  originate  in  the  capsule. 

The  secondary  involvement  of  a  joint  by  tumors  of  the  adjacent 
parts  is  not  uncommon. 


INDEX. 


ABIOTROPHY,  521 

Abortion,  878 

Abscess  of  lungs,  301,  303 

of  mammary  gland,  885 

retromammary,  884 

tuboovarian,  851 
Acanthosis  nigricans,  968 
Achlorhydria,  358 
Achondroplasia,  1010 
Acid-fast  bacilli,  307,  757 
Acne  of  sebaceous  glands,  976 
Acrochordon,  957,  976 
Acromegaly,  684,  1041 
Actinomycosis  of  bones,  1031 

of  buccal  cavity,  388 

of  cerebrum,  559 

of  Fallopian  tubes,  847 

of  heart,  149 

of  intestines,  443 

of  joints,  1056 

of  kidney,  758 

of  liver,  476 

of  lungs,  318 

of  mammary  gland,  888 

of  muscles,  987 

of  oesophagus,  401 

of  pericardium,  140 

of  pharynx,  595 

of  pia-arachnoid,  548 

of  skin,  924 

of  spleen,  228 

of  stomach,  412 

of  thyroid  gland,  691 

of  tonsil,  395 

of  vulva,  813 

Acute  yellow  atrophy  of  liver,  460 
Addison's  disease,  701,  951 
Adenocele,  895 
Adenoids,  393 
Adenolymphocele,  210 
Agnathia,  381 
Ague-cake,  226 
Agyria,  526 
Ainhum,  949 
Air  embolism,  55 
Albinism,  627,  908 
Albuminuria,  726 
Alopecia,  909 
Amastia,  883 
Amnion,  878 
Amotio  retinae,  644 


Amyelia,  527 

Amyloid  bodies  of  prostate,  794 

Amyotrophic  lateral  sclerosis,  596 

Anasarca,  106 

Anastomosis,  34,  37 

Anemia,  aplastic,  94 

of  choroid,  632 

of  heart,  145 

of  liver,  456 

pernicious,  92 

of  spinal  cord,  596 

of  retina,  637 

secondary,  91 

of  skin,  912 
Anencephaly,  524,  526 
Aneurism,  189,  196 
Aneurismal  varix,  199 
Angina  Ludovici,  396 
Angioneurotic  oedema,  113 
of  mamma,  884 
of  skin,  910 
Anidrosis,  973 
Aniridia,  628 
Ankylosis,  1043,  1051,  1053,  1054,  1057, 

1059,  1063 
Anophthalmia,  607 
Anorchidism,  799 
Anorexia  nervosa,  356 
Anthracosis,  307 
Anthrax,  925 
Anuria,  723 
Aorta,  atheroma  of,  194 

atresia  of,  144 

stenosis  of,  144 
Aortitis,  193 
Apneumatosis,  283 

Apoplexia  pulmonum  vascularis,  285 
Apoplexy,  cerebral,  78 

of  uterus,  827 
Appendicitis,  444 
Arcus  senilis,  624 
Argyria,  526,  631,  763,  951 
Arhinencephaly,  526 
Arrhythmia  of  heart,  125 
Arteriectasis,  197 
Arteries,  190 
Arteriolith,  192 
Arteriosclerosis,  175,  184,  194 
Arteritis,  191 
Arthritis,  acute,  1051 

chronic,  1053 

deformans,  1057 
Ascites,  107 


1066 


INDEX 


Ascites,  chyliform,  111 

chylous,  111 

pseudochylous,  111 
Asphyxia,  252 
Asteatosis,  975 
Asthma,  243 
Astomia,  381 
Ataxia,  hereditary  cerebellar,  595 

locomotor,  588 
Ataxic  paraplegia,  594 
Atelectasis,  255,  283,  290 
Ateleiosis,  1005 
Atelomyelia,  526 
Ateloprosopia,  381 
Atheroma  of  aorta,  194 

of  sebaceous  cysts,  975 
Atresia  of  aorta,  144 

of  Fallopian  tubes,  843 

of  pulmonary  valve,  144 

of  uterus,  822 

of  vagina,  815 
Atrophy  of  auditory  nerve,  650 

of  choroid,  636 

of  Fallopian  tubes,  847 

of  heart,  150 

of  kidney,  759 

of  liver,  478 

of  lymphatic  glands,  215 

of  mammary  gland,  888 

of  muscles,  988 

of  nerves,  603 

of  optic  nerve,  648 

of  ovaries,  852 

of  pancreas,  499 

of  prostate,  795 

of  retina,  644 

of  skin,  948 

of  vulva,  813 

Auditory  nerve,  atrophy  of,  650 
tumors  of,  651 


B 


BALANITIS,  788 
Balanoposthitis,  788 
aspergillina,  788 
Ball-thrombus,  70 
Barlow's  disease,  1019 
Bedsores,  948 
Belching,  349 
Beri-beri,  601 
Bile,  the,  363 
Biliary  calculi,  491 
cirrhosis,  471 

Bladder,  urinary,  abnormal  contents  of, 
778 

calculi  in,  779 

circulatory  disturbances  of,  775 

congenital  anomalies  of,  774 

cystitis  of,  775 

degenerations  of,  779 

dislocations  of,  774 

diverticula  of,  774,  780 

hypertrophy  of,  779 

paracystitis,  776 


Bladder,  urinary,  parasites  of,  778 
pericystitis,  776 
pilimictio,  778 
syphilis  of,  777 
tuberculosis  of,  777 
tumors  of,  780 

Blastomycetic  dermatitis,  930 
Blood  platelets,  101 
Blushing,  26 
Bone-marrow,  231 
Bones,  acromegaly  of,  684 

actinomycosis  of,  1031 

atrophy 'of,  1033 

callus  of,  1041 

caries  of,  1020,  1036 

chondrodystrophia  foetalis,  1010 

clubbed  fingers,  1018,  1041 

coxa  vara,  1018 

cretinism,  1006 

cysts  of,  1049 

dwarfism,  1005 

enostosisof,  1041, 1046 

epulis  of,  1045,  1047 

exostosis  of,  1041,  1046 

gigantism,  1013 

halisteresis,    1037 

hallux  valgus,  1018 

Howship's  lacunae,  1033, 1037 

hyperostosis,  1041 

hypertrophic       pulmonary      osteo- 
arthropathy,  1018,  1024 

kyphosis,  1017,  1037 

leontiasis  ossea,  1013 

leprosy  of,  1033 

lordosis,  1018,  1037 

Madura  foot,  1031 

nearthrosis,  1043 

necrosis  of,  1020,  1036 

osteitis  of,  1019 

osteogenesis  imperfecta,  1011 

osteomalacia,  1037 

osteomyelitis,  1020 

osteophytes,  1041 

osteoporosis,  1033 

osteopsathyrosis,  1013,  1034 

osteosclerosis,  1021,  1024,  1041 

periostitis,  1020 

phosphorus  necrosis  of,  1023 

pseudarthrosis,  1043 

rickets,  1007 

scoliosis,  1017,  1037 

sequestration  of,  1036 

syndesmosis,  1043 

synostosis,  1043 

syphilis  of,  1028 

tuberculosis  of,  1024 

variola  of,  1033 
Borborygmi,  350 
Bowman's  theory,  716 
Brachygnathia,  381 
Bromidrosis,  973 
i  Bronchial  occlusion,  242,  280 
'  Bronchiectasis,  261,  276,  279,  281 
Bronchiolitis  exudativa,  242,  278 
Bronchitis,  276 
Bronchocele,  691 


INDEX 


1067 


Broncholiths,  279 
Bronchopneumonia,  301 
Bubo,  indolent,  214 

virulent,  211,  918 
Bubonic  plague,  214 
Bulbomyelitis,  556 
Bursitis,  1001 


CACHEXIA  strumipriva,  677 
Caisson  disease,  56,  574 
Calcification,  44,  75 
Calculi,  biliary,  491 
pancreatic,  498 
preputial,  790,  814 
salivary,  396 
urinary,  779 
Callus,  951,  1041 
Canalization,  74 
Cancer  atrophicans,  900 

en  cuirasse,  902 
Cancrum  oris,  383 
Canities,  971,  972 
Capillary  embolism,  50 

thrombosis,  70 
Caries,  390,  1020,  1036 
Carnification  of  lungs,  326 
Carotid  body,  677,  711 
Caruncle,  urethral,  782,  784 
Cavernitis,  788 
Cavernoma,  483 
Cataract,  651 
Cephalhematoma,  1019 
Cerebellar  ataxia,  hereditary,  595 
Cerebral  apoplexy,  78 
diplegia,  535 
palsy,  infantile,  535 
Cerebrum  and  cerebellum,  actinomycosis 

of,  559 

circulatory  disturbances  of,  551 
congenital  anomalies  of,  523 
encephalitis,  555 
parasites  of,  567 
retrograde    metamorphoses    of, 

560 

syphilis  of,  559 
trauma  of,  567 
tuberculosis  of,  559 
tumors  of,  563 
Chalicosis,  307 
Chancre,  789 

Chancroid  of  penis,  790,  918 
of  skin,  789 
of  vulva,  813 

Charcot-Leyden  crystals,  243,  278 
Charcot-Neumann  crystals,  233 
Charcot's  joint,  1061 
Chemosis,  106,  609 
Cheyne-Stokes  respiration,  253 
Chimney-sweep's  cancer,  808 
Chloasma,  950 
Chlorosis,  90 
Cholangitis,  489 
Cholecystitis,  490 


Cholelithiasis,  491 
Cholera  asiatica,  435 
Cholesteatoma,  548,  563,  663 
Chondrodystrophia  foetalis,  1010 
Chordee,  783 
Chordoma,  540 
Chorea,  Huntingdon's,  562 
Chorio-epithelioma  malignum,  872 
Choroid  albinism,  632 

anemia  of,  632 

atrophy  of,  636 

choroiditis,  633 

coloboma  of,  632 

detachment  of,  636 

hemorrhage  in,  633 

hyperemia  of,  632 

syphilis  of,  636 

trauma  of,  637 

tuberculosis  of,  636 
Choroiditis,  633 
Chromatophoroma,  966 
Chromidrosis,  974 
Chyliform  ascites,  111 
Chylocele,  805 
Chylous  ascites,  111 

hydrothorax,  111,  325 
Chyluria,  727 
Cirrhosis,  biliary,  471 

of  liver,  465 

of  mammse,  886 

pericellular,  471 

portal,  466 
Cleft  palate,  381 
Clitoris,  436,  444 

cornu  cutaneum  of,  814 

elephantiasis  of,  814 

hypertrophy  of,  810 
Clubbed  fingers,  1018,  1041 
Coccygeal  gland,  685,  713 
Colitis,  436,  444 
Coloboma  of  choroid,  632 

of  eye,  608 

of  optic  nerve,  646 

of  retina,  637 

Colpohyperplasia  cystica,  818 
Comedo,  975 

Compression  of  lungs,  291,  326 
Concato's  disease,  335,  791 
Condyloma  of  penis,  791 

of  skin,  956 

of  vulva,  812 
Congestion  of  optic  nerve,  646 
Conjunctiva,  chemosis  of,  609 

conjunctivitis,  609 

acute  catarrhal,  610 
membranous,  611 
purulent,  610 
follicular,  612,  613 
granular,  612 
papillary,  612 
JParinaud's,  611 
phlyctsenulosa,  613 
variolous,  611 
vernal,  613 

cysts  of,  615 

eczema  of,  613 


1068 


INDEX 


Conjunctiva,  hemorrhage  in,  609 

hyperemia  of,  609 

leprosy  of,  614 

oedema  of,  609 

pinguecula,  615 

pterygium,  615 

symblepharon,  617 

syphilis  of,  614 

tuberculosis  of,  614 

tumors  of,  616 

xerosis  of,  614 
Constipation,  351 
Contraction  of  stomach,  407 
Cor  villosum,  138 
Corectopia,  628 
Cornea,  arcus  senilis,  624 

degenerations  of,  625 

embryotoxon,  618 

facets,  620 

herpes  of,  622 

hydrophthalmus  anterior,  618 

keratitis,  619 
bullosa,  621 
interstitial,  620 
keratomalacia,  323 
keratomycosis  aspergillina,  624 
marginal,  621 
neuroparalytic,  621 
phlyctenular,  622 
punctata,  624 
ulcerative,  622 
vascular,  621 

leprosy  of,  624 

leukoma  of,  620 

nebula  of,  620 

pannus  of,  621 

pigmentation  of,  625 

staphyloma  anterior,  620 

syphilis  of,  624 

tuberculosis  of,  624 
Corneal  facet,  620 
Cornu  cutaneum,  814,  952 
Corpora  amylacea,  321,  794 

oryzoidea,  1001,  1055 
Coryza,  264 
Coughing,  251 
Coxa  vara,  1018 
Craniorachischisis,  524 
Cranioschisis,  524 
Craniotabes,  1009 
Craw-craw,  932 
Cretinism,  1006 
Curschmann's  spirals,  243,  278 
Cutis  laxa,  958 
Cyanotic  induration,  30 
of  kidney,  733 
of  lungs,  286 
of  spleen,  223 
Cyclencephaly,  526 
Cyclitis,  630,  631 
Cyclopia,  264,  607 
Cystitis,  775 

Cystosarcoma  phyllodes,  899 
Cysts  of  conjunctiva,  615 

of  Ivmphatic  glands,  210 

of  mammary  gland,  888,  903 


Cysts  of  ovaries,  855 
of  pancreas,  503 
of  penis,  792 
of  prostate,  795 
of  retina,  644 
of  thymus  gland,  698 
tuboovarian,  843 
of  vagina,  819 


DARIER'S  disease,  955 
Deaf-mutism,  673 
Decubitus  of  oesophagus,  402 

of  skin,  948 
Deferenitis,  809 
Degeneration  of  bladder,  779 

of  cornea,  625 

of  endocardium,  166,  169 

of  heart,  146,  150 

of  kidney,  760 

of  joints,  1061 

of  liver,  478 

of  lungs,  321 

of  lymphatic  glands,  215 

of  muscles,  992 

of  retina,  644 
Deglutition,  343,  345 
Dementia  paralytica,  561 
Dermatitis.     See  Skin. 
Dermatographia,  933 
Dermatolysis,  958 
Dermatomycosis,  927 
Dermatomyositis,  983 
Dermoids,  337,  390,  616,  862 
Diabetes  insipidus,  722 

mellitus,  500 
Diabetic  gangrene,  949 
Diarrhoea,  350 
Diastematomyelia,  533 
Digestion,  mechanics  of,  343 
Digestive  ferments,  action  of,  354 
Dilatation  of  heart,  131,  156 

of  intestines,  423 

of  oesophagus,  397,  398 

of  stomach,  407 
Diphtheria  of  bronchi,  278 

of  conjunctiva,  611 

of  external  auditory  meatus,  660 

of  oesophagus,  400 

of  pharynx,  394 

of  stomach,  410 

of  vagina,  818 

of  vulva,  812 
Diplomyelia,  533 
Dislocations  of  bladder,  774 

of  joints,  1050,  1062 

of  ovaries,  849 
Disseminated  sclerosis,  562 
Diverticula  of  bladder,  774,  780 

of  intestine,  423 

Meckel's,  422 

of  oesophagus,  398 

of  pericardium,  136 

of  stomach,  404 


INDEX 


1069 


Diverticula  of  uterus,  826 
Drug  rashes,  947 
Ductus  arteriosus,  144 
Duodenitis,  443 
"Dust-bodies,"  102 
Dwarfism,  1005 
Dyscoria,  638 
Dysentery,  432 
Dyspnoea,  251 
Dystrichiasis,  613 


EAR,  external,  656 
internal,  671 
middle,  665 
Ecchymosis,  78,  911 
Echinococcus  disease  of  bladder,  779 
of  bones,  1033 
of  bronchi,  279 
of  liver,  477 
of  lung,  320 
of  lymphatic  channels,  204 

glands,  215 
of  mediastinum,  336 
of  meninges,  567 
of  muscles,  987 
of  myocardium,  150 
of  ovary,  852 
of  pericardium,  141 
of  pituitary  body,  709 
of  pleurae,  "331 
of  prostate,  795 
of  spleen,  228 
of  thyroid  gland,  691 
of  tunica  vaginalis  testis,  807 
of  uterus,  832 
Ecthyma,  918 
Ectopic  gestation,  879 
Eczema  of  conjunctive,  613 

of  skin,  935 

Elephantiasis  of  clitoris,  814 
neuromatosa,  605 
of  penis,  792 
of  scrotum,  808 
of  skin,  953 
of  vulva,  814 
Embolism,  47,  48 
of  liver,  458 
of  lung,  289 
Embryotoxon,  618 
Emphysema,  pulmonary,  257,  292 
Encephalitis,  555 
Encephalocele,  524,  525 
Encephalomalacia,  554 
Encephalomyelitis,  556 
Endarteritis  obliterans,  192 
Endocarditis,  161 
Endocardium,  circulatory  disturbances  of, 

160 

congenital  anomalies  of,  159 
degenerations  of,  166,  169 
endocarditis,  161 

acute  verrucose,  162 
ulcerative,  163 


Endocardium,  endocarditis,  chronic,  165 

hematoma  of,  160 

sclerosis  of,  165 

syphilis  of,  167 

traumatism  of,  168 

tuberculosis  of,  167 
Endometritis,  828,  829 
Enteritis,  430 
Enterocystoma,  451 
Enteroliths,  451 
Enteroplegia,  423 
Enteroptosis,  406 
Entropion,  613 
Eosinophilia,  77 
Ephelides,  950 
Epicanthus,  607 

Epidemic  cerebrospinal  meningitis,  544 
Epididymitis,  799 
Episcleritis,  626 
Epistaxis,  79,  264 
Epithelioma,     multiple    benign    cystic, 

964 

Epulis,  388,  1045, 1047 
Ergotism,  591,  947 
Erosion  of  stomach,  416 
Erysipelas,  926 
Erythema,  910,  914,  937 
Erythrasma,  929 
Erythrocytes,  86 
Erythromelalgia,  26 
Eustachian  tube,  664 
Exanthemata,  941 
Exophthalmic  goitre,  690,  699 
Exophthalmos,  654 
Extroversion  of  bladder,  774 
Eye,  anophthalmia,  607 

coloboma,  608 

cyclopia,  607 

exophthalmos,  654 

glaucoma,  650 

hydrophthalmus,  607 

melanosis,   627 

microphthalmia,  607 

panophthalmitis,  625,  632 

persistent  hyaloid  artery,  608 

phthisis  bulbi,  632 

sympathetic  ophthalmitis,  632 


FALLOPIAX  tubes,  actinomycosis  of,  847 
atresia  of,  843 
atrophy  of,  847 
congenital  anomalies  of,  842 
foreign  bodies  in,  847 
hematosalpinx,  844 
hydrops  profluens,  843 
hydrosalpinx,  843 
hypertrophy  of,  847 
parasites  of,  847 
physopyosalpinx,  845 
pyosalpinx,  845 
rupture  of,  844 


1070 


INDEX 


Fallopian  tubes,  salpingitis,  844 
salpingocele,  843 
syphilis  of,  846 
tuberculosis  of,  846 
tuboovarian  cyst,  843 
tumors  of,  847 
Fat-embolism,  54 
Fat-necrosis,  372,  497,  500 
Favus  of  nails,  973 

of  skin,  927 
Fibroids  of  uterus,  834 
Fibrosis  of  heart,  146 
Fistulas  of  uterus,  827 

of  vagina,  816 

Flexions  of  uterus,  822,  825 
Foetus,  abortion,  878 

ectopic  gestation,  879 
lithokelyphos,  881 
lithopedion,  879 
maceration  of,  879,  881 
papyraceus,  879 
Foot,  perforating  ulcer  of,  949 
Foreign  bodies  in  Fallopian  tubes,  847 
in  heart,  149 
in  uterus,  833 
in  vagina,  819 
Frambesia,  926 

"Free-bodies"  in  peritoneal  cavity,  511 
in  joints,  999,  1028,  1051,  1058 


GALACTOCELE,  903 
Galactophoritis,  886 
Ganglion  crepitans,  1001 
Gangrene,  45 

hospital,  917 

of  lungs,  301 

of  skin,  949 

of  vulva,  813 
Gas-embolism,  56 
Gastric  juice,  355 
Gastritis,  409 
Gastromalacia,  414 
General  paralysis  of  insane,  561 
Genu  valgum,  1018 
Gerontoxon,  624 
Gigantism,  1013 
Glanders  of  kidney,  759 

of  liver,  482  " 
,  of  lungs,  319 

of  lymphatics,  204 

of  muscles,  987 

of  nasal  cavity,  266 

of  skin,  926 

of  spleen,  228 

of  testis,  803 
Glaucoma,  650 
Gl£nard's  disease,  406 
Goitre,  688,  692 
Gonorrhoea,  782 
Gout,  999,  1053 
Graves'  disease,  689,  690,  699 
Guinea  worm,  932 
Gynecomastia,  888 


HAIR,  alopecia,  909,  971 
canities,  972 
hypertrichosis,  709,  972 
mpnilethrix,  972 
trichorrhexis  nodosa,  972 
Hair  balls  in  stomach,  413 
Hairy  tongue,  387 
Halisteresis,  1037 
Hallux  valgus,  1018 
Hanot's  cirrhosis,  470 
Harelip,  381 

Heart  (myocardium),  anemia  of.  145 
atrophy  of,  150 
actinomycosis  of,  149 
degenerations  of,  146,  150 
dilatation  of,  131,  156 
fibrosis  of,  146 
foreign  bodies  in,  149 
Iwperemia  of,  145 
inflammations  of,  147 
myomalacia,  145 
parasites  of,  150 
rupture  of,  154 
segmentation  of,  152 
syphilis  of,  149 
trauma  of,  149 
tuberculosis  of,  149 
tumors  of,  158 
Hematemesis,  79,  408 
Hematidrosis,  79,  974,  91 1 
Hematocele,  79,  869 
Hematocolpos,  816 
Hematoidin,  80 
Hematoma  of  endocardium,  160 

of  uterus,  827 
Hematometra,  826 
Hematomyelopore,  574 
Hematopericardium,  79,  136 
Hematosalpinx,  826,  843,  844 
Hematothorax,  79,  325 
Hematuria,  79,  770,  778 
Hemochromatosis,  470,  481,  951 
Hemoconia,  102 
Hemoglobinuria,  727 
Hemolymph  glands,  209 
Hemolysis,  68,  89 
Hemophilia,  81 
Hemoptysis,  310 
Hemorrhage,  76 

into  choroid,  633 
into  conjunctiva,  607 
into  liver,  458 
into  oesophagus,  399 
into  retina,  639 
into  skin,  911 
into  spinal  cord,  574 
into  suprarenal  glands,  701 
into  synovia!  sacs,  1051 
Hemorrhoids,  429 
Hemosiderin,  80 
Hemosiderosis,  481 
Hepar  lobatum,  475 
Hepatitis,  459 
HepatoptosiSj  406,  455 


INDEX 


1071 


Hereditary  cerebellar  ataxia,  595 

Hernia  of  intestines,  425 

Herpes,  26,  776,  789,  934,  945 

Heterochrpmia,  627 

Heterotopia  of  spinal  cord,  533 

Hiccoughing,  349 

Hidradenitis,  974 

Hidrocystoma,  974 

Hippocratic  fingers,  1018 

Hirschsprung's  disease,  423 

Hodgkin's  disease,  217 

Hospital  gangrene,  917 

Hottentot  apron,  811 

Housemaid's  knee,  1001 

Howship's  lacunie,  1033 

Huntingdon's  chorea,  562 

Hutchinson's  teeth,  381 

Hyaloid  artery,  persistent,  608 

Hyaloserositis,  335,  473,  509 

Hydatidiform  mole,  876 

Hydramnios,  878 

Hydremia,  21 

Hydroa  vacciniforme,  938 

Hydrocele,  106,  805,  806,  865 

Hydrocephalus,  106,  525;  527,  528,  1017 

Hydrometra,  826 

Hydromyelocele,  525 

Hydronephrosis,  759,  771 

Hydrophthalmos,  607 

Hydrops  articuli,  1051 

follicularis,  853 

profluens,  843 
Hydrorrhachis,  525,  529 
Hydrorrhoea  gravidarum,  871 
Hydrosalpinx,  843 
Hydrothorax,  106,  325 
Hygroma  burssp,  1001 
Hyperchlorhydria,  358 
Hyperemia,  24 

of  choroid,  632 

of  conjunctiva,  609 

of  heart,  145 

of  lungs,  285 

of  retina,  638 
Hyperidrosis,  973 
Hyperinosis,  85 
Hypernephroma,  705,  768 
Hyperoriychia,  908,  973 
Hyperpiesis,  180 
Hypertrichosis,  909 
Hypertrophy  of  bladder,  779 

of  clitoris,  810 

of  Fallopian  tubes,  847 

of  heart,  155 

of  intestines,  451 

of  kidney,  764 

of  mammary  gland,  888 

of  muscles,  995 

of  prostate,  795 

of  stomach,  416 

of  uterus,  833 
Hyphidrosis,  973 
Hypinosis,  85 
Hypophysis,  683,  705 
Hypopyori,  620,  624,  625,  630 
Hysterocele,  822,  825 


ICHTHYOSIS,  907 

Icterus,  371,  489,  951 

Ileitis,  444 

Impetigo,  917,  945 

Infantile  cerebral  palsy,  535 

Infantilism,  1005 

Infarction,  39,  288 

Insufficiency  of  heart  valves,  168 

Intermittent  claudication,  174 

Intestines,   circulatory   disturbances   of, 

429 

colitis,  430,  444 
congenital  anomalies  of,  422 
dilatation  of,  423 
diverticula,  acquired,  423 
enteritis,  430 

acute  catarrhal,  431 
desquamative,  431 
dvsentery,  432 
follicular,  431 
membranous,  432 
paratyphoidal,  439 
tuberculous,  439 
typhoidal,  436 
hernia  of,  425 
intestinal  sand,  451 
intussusception,  424 
megacolon,  423 
obstruction  of,  428 
stenosis  of,  424 
tumors  of,  451 
|          volvulus  of,  425 
Intussusception,  424 
Inversion  of  uterus,  822 
Irideremia,  608,  628 
Iridochoroiditis,  630 
Iridocyclitis,  630 
Iritis,  629 

plastic,  629 
serous,  630 
suppurative,  630 


JOINTS,  ankylosis  of,   1043,   1051,   1053, 

1054,  1057,  1059,  1063 
arthritis,  acute  gonorrhceal,  1051 

gouty,  1053 

purulent,  1052 

rheumatic,  1051 
chronic  actinomycotic,  1056 

ankylopoetic,  1057 

deforming,  1057 

gouty,  1054 

neurbtrophic,  1061 

rheumatic,  1057 

serous,  1054 

syphilitic,  1056 

tuberculous,  1054 

ulcerating,  1059 
degenerations  of,  1061 
echinococcus  disease  of,  1061 
injuries  of,  1062 


1072 


INDEX 


Joints,  luxation  of,  1050,  1062 
ochronosis,  1062 
periarthritis,  1051 
Still's  disease,  1053 
tumors  of,  1064 
"white  swelling,"  1056 


KAKKE,  601 
Keloid,  958 
Keratitis.     See  Cornea. 
Keratodermia,  952 
Keratomalacia,  623 
Keratomycosis  aspergillina,  624 
Keratoscleritis,  626 
Keratosis,  657,  955 
Kidney,  actinomycosis  of,  758 

anomalies  of,  728 

atrophy  of,  759 

degenerations  of,  760 

glanders  of,  759 

hypertrophy  of,  764 

infarct  of,  733 

infiltrations  of,  762 

inflammations  of,  735 

leprosy  of,  759 

parasites  of,  769 

pelvis  of,  parasites  of,  772 
pyelitis,  771 
tuberculosis  of,  772 
tumors  of,  773 

of  pregnancy,  744 

syphilis  of,  758 

tuberculosis  of,  755 

tumors  of,  764 
Koplik's  spots,  382,  941 
Kraurosis  vulvae,  813 
Kupfer's  cells,  105 
Kyphosis,  1017 


LABIA,  810 

Lacing-lobe  of  liver,  455 

Laennec's  cirrhosis,  466 

Landry's  paralysis,  579 

Laryngitis,  269 

Lateral  sclerosis,  587 

Lens,  650 

Lentigo,  950 

Leontiasis  ossea,  1013 

Leprosy  of  bones,  1033 
of  conjunctiva,  614 
of  cornea.  624 
of  epididymis,  902 
of  kidney,  759 
of  lymphatics,  204 
of  lymph-nodes,  215 
of  nasal  cavity,  266 
of  peripheral  nerves,  602 
of  pleura,  331 
of  skin,  923 


Leprosy  of  spinal  cord,  581 

of  spleen,  228 

of  testis,  802 
Leucoderma,  950 
Leukemia,  98,  216 

of  liver,  457 

of  lymphatic  glands,  218 
Leukocytes,  94 
Leukocytosis,  96 
Leukoma,  620 
Leukopathia,  908,  950 
Leukoplakia,  387,  402 
Lichen,  939,  945 
Liebermeister's  grooves,  4  .V> 
Lipemia,  86 
Lipoma  arborescens,  1000,  1058 

retroperitoneal,  512 
Lithokelyphos,  881 
Lithopedion,  879 
Livedo,  910 
Liver,  actinomycosis  of,  476 

amyloid,  479 

anemia  of,  456 

atrophy  of,  478 

cirrhosis  of,  465 

cloudy  swelling  of,  478 

congenital  anomalies  of,  455 

degenerations  of,  478 

embolism  of,  458 

fattv  changes  in,  478 

glanders  of,  482 

hemochromatosis,  470,  481 

hemorrhage  of,  458 

hemosiderosis  of,  481 

hepatitis,  acute,  459 

hepatoptosis,  4  ."">."> 

lacing-lobe  of,  455 

leukemia  of,  457 

Liebermeister's  grooves  of,  455 

necroses  of,  481 

nutmeg,  456 

oedema  of,  458 

parasites  of,  477 

perihepatitis,  472 

pigmentation  of,  480 

pseudocirrhosis  of,  472 

syphilis  of,  475 

tropical  abscess,  464 

tuberculosis  of,  474 

tumors  of,  483 
Lochiometra,  826 
Locomotor  ataxia,  588 
Lorclosis,  1018 
Lud wig's  theory,  716 
"Lumpy-jaw,"  386 
Lungs,  abscess  of,  301,  303 

actinomycosis  of,  318 

anthracosis  of,  307 

apneumatosis  of,  283 

atelectasis  of,  283,  290 

bronchopneumonia,  301 

carnification  of,  326 

chalicosis,  307 

compression  of,  291,  326 

congenital  anomalies  of,  283 

degenerations  of,  321 


INDEX 


1073 


Lungs,  embolism  of,  289 
emphy sema  of,  257,  292 
gangrene  of,  301 
glanders  of,  319 
hemoptysis  of,  310 
hepatization  of,  298 
hyperemia  of,  285 
h  ypostatic  pneumonia,  302 
induration  of,  286,  305 
infarction  of,  288 
oedema  of,  284 
parasites  of,  319 
peribronchitis,  301 
pigmentation  of,  286 
pneumonia,  295 
pneumonokoniosis,  305,  307 
pneumonomalacia,  321 
pseudotuberculosis,  317 
siderosis,  307 
syphilis  of,  318 
tuberculosis  of,  307 
tumors  of,  322 
Lupus,  919 

erythematosus,  940 
of  vulva,  813 
Lymphadenia,  216 
Lymphadenitis,  210,  212 
Lymphangiectasis,  107,  204 
Lymphangitis,  203 
Lymphatic  system,  103,  208 

glands,  adenolymphocele,  210 
atrophy  of,  215 
bubo,  acute,  211,  918 

indolent,  214 
bubonic  plague,  214 
cysts  of,  210 
degenerations  of,  215 
lymphadenia  of,  216 

Hodgkin's  disease,  216 
leukemia,  218 
lymphosarcoma,  218 
lymphadenitis,  210 
acute,  210 

bubonic  plague,  214 
catarrhaf  or  simple,  210 
fibrinous,  212 
hemorrhagic,  212 
suppurative,  211 
chronic,  212 

leprosy,  215 
syphilis,  214 
tuberculosis,  212 
parasites  of,  215 
status  lymphaticus,  209,  698 
tumors  "of,  218 
Lymphocytosis,  97 
Lymphorfhagia,  204 
Lymphosarcoma,  218 
Lymph-scrotum,  808 
Lymph-varices,  210 


M 

MACROCHEILIA,  207,  387 
Macrogenesy,   1013 
68 


Macroglossia,  207,  387 
Macrosomia,  1013 
Macrostomia,  381 
Macrotia,  655 
Madura  foot,  924,  1031 
Malacosteon,  1039 
Mastication,  343,  345 
Mammary  gland,  abscess  of,  885 

actinomycosis  of,  888 

angioneurotic  oedema  of,  884 

atrophy  of,  888 

circulatory  disturbances  of,  884 

congenital  anomalies  of,  883 

cysts  of,  888,  903 

galactocele,  903 

galactophoritis,  886 

gynecomastia,  888 

hypertrophy  of,  888 

mastitis,  885 

milk-fistulse,  885 

paramastitis,  884 

parasites  of,  903 

ret  romammary ^abscess,  884 

syphilis  of,  887-1 
chancre,  887 
diffuse  mastitis,  888 
gumma,  887 

thelitis  of,  885 

tuberculosis  of,  886 

vicarious  menstruation,  884 

witch's  milk,  883 
Mastitis,  acute,  885 
chronic,    886 
carcinomatosa,  886 
Measles,  941 

Meckel's  diverticulum,  422 
Mediastinitis..  334 
Mediastino-pericarditis,  140,  335 
Mediastinum,  congenital  anomalies  of,  334 
inflammations  of,  334 
parasites  of,  336 
syphilis  of,  335 
tuberculosis  of,  335 
tumors  of,  336 
Megacolon,  423 
Megaloblasts,  88 
Megalocytes,  88 
Megalophthalmos,  618 
Melanosis  oculi,  627 
Melena,  79,  408 
i  Membrana  tympani,  662 
'  Memere's  disease,  672 
!  Meningitis,  cerebral,  536 

epidemic  cerebrospinal,  544 

syphilitic,  547 

tuberculous,  545 
spinal,  570,  572 

cerebrospinal,  544 

syphilitic,  573 

tuberculous,  572 
Meningocele,  524 
Meningo-encephalitis,  542,  546 
Meningo-encephalocele,  525,  526 
Meningomyelitis,  579 
Menorrhagia,  79,  828,  869 
Mesaortitis  syphilitica,  178 


1074 


INDEX 


Metritis,  831,  870,  871 
Melrorrhagia,  79,  827  869 
Micrencephaly,  527 
Microbrachius,  1005 
Microcephaly,  527,  1005,  1016 
Microcytes,  88 
Microgyria,  528,  535 
Micromastia,  883 
Micromelus,  1005,  1016 
Microphthalmia,  607,  618 
Micropus,  1005 
Microsomia,  1005 
Microstomia,  381 
Microthelia,  883 
Microtia,  655 
Milium,  976 
Milk-fistula,  885 
Milk  spots,  141 
Mitral  valve  insufficiency,  168 

stenosis,  168 

Moenckeberg's  sclerosis,  180 
Mollities  ossium,  1037 
Molluscum  contagiosum,  965 
Monilethrix,  972 
Morbus  coxae  senilis,  1060 
Morphcea,  923 
Mortification,  44 

Multiple  benign  cystic  epithelioma,  964 
Mummification,  993 
Muscles,  actinomycosis  of,  987 

atrophy  of,  988 

circulatory  disturbances  of,  981 

congenital  anomalies  of,  980 

degenerations  of,  992 

glanders  of,  987 

hypertrophy  of,  995 

myositis,  982,  983 

myotonia  congenita,  995 

parasites  of,  987 

polymyositis,  983 

primary  myopathy,  990 

pseudohypertrophy  of,  991 

syphilis  of,  986 

tuberculosis  of,  985 

tumors  of,  996 
My  asthenia  gravis,  686 
Mycetoma  pedis,  924 
Mycosis  aspergillina,  279 
Myeloblasts,  94 
Myelocystocele,  525,  526 
Myelocytes,  95,  98 
Myeloma,  235,  238,  574,  1046 
Myelomatosis,  236 
Myocarditis,  147 
Myocardium,    142.     See  Heart. 
Myomalacia,  145 
Myopathy,  primary,  990 
Myositis,  982 

ossificans,  983 
Myotonia  congenita,  995 
Myringitis,  662 


NAILS,  favus,  973 

hyperonychia,  973 


Nails,  leukopathia,  972 
onychia,  973 
onychogryphosis,  973 
oi;\  chomvcosis,  973 
paronychia,  973 
Nanosomia,  1005 
Nasal  polyps,  267 
Nearthrosis,  1043 
Nebula,  620 

Necrosis  of  bones,  1020,  1036 
of  liver,  481 
phosphorus,  1023 
Nephritis,  735 

acute  diffuse,  745 
interstitial,  744 
kidney  of  pregnancy,  744 
parenchymatous.  742 

desquamative    papillary, 

744 

glomerulitis,  743 
hemorrhagic,  743 
chronic  diffuse,  746 

^lomerulitis,  746 
hemorrhagic,  746 
interstitial,  747 

primary  contracted  kidney,  750 
suppurative,  751 
Nephrohthiasis,  763 
Nephroptosis,  406 
Nerves,  peripheral,  atrophy  of,  603 
inflammations  of,  600 
leprosy  of,  602 
syphilis  of,  602 
tuberculosis  of,  602 
tumors  of,  603 
Neuritis,  peripheral,  600 
Neuron  concept,  515 
Neuropathic  oedema,  113.  884,  932 
N  euro  ret  initis,  647 
Nevi,  908,  909 
Noma  of  face,  383 

vulviP,  813 
Nutmeg  liver,  456 


OCHRONOSTS,  1062 
Odontinoid,  390 
Odontoma,  390 
(Edema  glottidis,  269,  273 
(Esophagitis,  399 
(Esophagomalacia,  402 
(Esophagus,  actinomycosis  of,  401 
congenital  anomalies  of,  397 
decubitus  of,  402 
dilatation  of,  397,  398 
diverticula  of,  398 
hemorrhage  of,  399 
oesophageal  varices,  399 
leukoplakia,  402 
cesophagitis,  399 
catarrhal,  399 
corrosive,  400 
exfoliative,  400 
follicular,  400 


INDEX 


1075 


(Esophagus,    cesophagitis,   membranous, 

400 

phlegmonous,  400 
pustular,  400 
cesophagomalacia,  402 
peptic  ulcer,  402 
perforation  of,  399 
stenosis  of,  397 
syphilis  of,  401 
thrush,  401 
tuberculosis  of,  401 
tumors  of,  402 
Oligemia,  22 
Oligohydramnios,  878 
Oliguria,  723 
Omentum,  507 
Onychia,  973 
Onychogryphosis,  973 
Onychomycosis,  973 
Onyx,  624 
Oophoritis,  850 
Opaline  plaques,  385,  512 
Ophthalmia  neonatorum,  610 
Ophthalmitis  sympathetica,  632 
Optic  nerve,  atrophy  of,  648 
coloboma  of,  646 
congestion  of,  646 
neuritis,  646 

neuroretinitis,  647 
papillitis,  646,  647 
retrobulbar,  648 
oedema  of,  646 
scotoma,  648 
syphilis  of,  648 
tuberculosis  of,  648 
tumors  of,  648 
Orbit,  inflammations  of,  654 

tumors  of,  654 
Orchitis,    799 
Oriental  furuncle,  926 
Osteitis,  1019 

deformans,  1057 

Osteo-arthropathy,    hypertrophic   pul- 
monary, 1019,  1024 
Osteogenesis  imperfecta,  1011 
Osteomalacia,  1037 
Osteomyelitis,  1020 
Osteophytes,  1041 
Osteoporosis,  1033 
Osteopsathyrosis,   1013,   1034 
Osteosclerosis,  1021,  1024,  1041 
Othematoma,  657 
Otitis,  externa,  659 

interna  (labyrinthitis),  672 
media,  666 
Otomycosis,  660 
Ovaries,  atrophy  of,  852 

circulatory  disturbances  of,  850 
congenital  anomalies  of,  848 
cysts  of,  855 
dislocations  of,  849 
hydrocele  of,  844 
hyperplasia  of,  853 
inflammations  of,  850 
syphilis  of,  852 
tuberculosis  of,  852 


Ovaries,  tumors  of,  854 
Ovula  nabothi,  830 
Oziena,  265 

syphilitica,  266 


PACHYDERMIA  laryngis,  274 
Paget's  disease  of  bones,  1023 

of  nipple,  899,  970 
Palsy,  infantile  cerebral,  535 
Pancreas,  atrophy  of,  499 

calculi  of,  498 

circulatory  disturbances  of,  493 

cysts  of,  503 

fat-necrosis  of,  500 

inflammations  of,  494 

self-digestion,  501 

sialodochitis  pancreatica,  494 

syphilis  of,  499 

tuberculosis  of,  498 

tumors  of,  501 
Pancreatitis,  acute  hemorrhagic,  496 

chronic,  497 

degenerative  parenchymatous,  495 

suppurative,  495,  497 
Panophthalmitis,  632 
Papillitis,  646,  647 
Papules,  914 
Paracystitis,  776 
Paradoxical  embolism,  49 
Paragonimiasis,  320 
Paralysis  of  insane,  general,  561 

Landry's,  579 
Paramastitis,  884 
Parametritis,  828,  866 
Paranephritis,  754,  757 
Paraphimosis,  788 
Parasites  of  bladder,  778 

of  cerebrum,  567 

of  Fallopian  tubes,  847 

of  heart,  150 

of  kidney,  769 

of  liver,  477 

of  lungs,  319 

of  lymphatic  glands,  215 

of  mammary  gland,  903 

of  mediastinum,  336 

of  muscles,  987 

of  pelvis  of  kidney,  772 

of  retina,  644 

of  skin,  931 

of  uterus,  832 

of  vagina,  819 
Parathyroids,  678,  687 
Paratyphoid  fever,  439 
Parinaud's  conjunctivitis,  611 
Paronychia,  973 
Parotitis,  395 

Passive  congestion,  general,  27 
Patent  foramen  ovale,  143 
Pectus  carinatum,  1008 
Pediculosis,  932 
Pellagra,  947 
Pemphigus,  937 


1076 


INDEX 


Penis,  anomalies  of,  787 

balanitis,  788 

cavernitis,  788 

chancroid  of,  790,  918 

chordee  of,  783 

condyloma  of,  791 

cysts  of,  792 

elephantiasis  of,  792 

herpes  of,  789 

injuries  of,  792 

keratosis,  791 

paraphimosis,  788 

phagedena,  790,  791 

phimosis,  788 

syphilis  of,  789,  918 

tuberculosis  of,  790 

tumors  of,  791 
Peptic  ulcer,  402,  414 
Perforating  ulcer  of  foot,  (.'1(.) 
of  nasal  septum,  266 
Periarteritis,  184 

nodosa,  192 
Periarthritis,  1051 
Peribronchitis,  301 
Pericarditis,  acute,  138 

dry  fibrinous,  138 
fibrinous,  137,  139 
hemorrhagic,  138 
suppurative,  138 

chronic,  140 

syphilitic,  140 
tuberculous,  140 

Pericardium,  circulatory  disturbances  of, 
136 

congenital  anomalies  of,  135 

diverticula  of,  136 

inflammations,  137 

pneumatopericardium,  136 

pyopericardium,  138 

tumors  of,  141 
Pericellular  cirrhosis,  471 
Pericholangitis,  491 
Pericholecystitis,  491 
Pericystitis,  491 
Perigastritis,  412 
Perihepatitis,  472 
Perilymphadenitis,  211 
Perilymphangitis,  203 
Perimetritis,  866,  872 
Perinephritis,  753 
Perioophoritis,  866 
Periorchitis,  805 
Periostitis,  1020 
Perisalpingitis,  845 
Perisplenitis,  225 
Peritonitis,  505 
Perityphlitis,  444 
Perivaginitis,  818 
Perl's  test,  92,  481 
"Perlsucht,"  330 
Pernicious  anemia,  92 
Pernio,  916 
Pes  planus,  1018 
Petechiee,  78,  911 
Phagedena  of  penis,  790,  791 

tropical,  926 


Phagedena  of  vulva.  813 
Pharyngitis,  391 
Phimosis,  788,  791 
Phlebectasia,  201 
laryngis,  269 
Phlebitis,  202 
Phleboliths,  793 
Phlebosclerosis,  203 
Phlyctenulae,  613,  622 
P I  losphorus  necrosis,  1023 
Phthisis  bulbi,  632 
Pliynui,  914 
Physometra,  826 
PhysopyoBalpinx,  845 
Phytobezoar,  413 
Pilimictio,  778 
Pineal  gland,  711 
Pinguecula,  615 

Pituitary  body.     See  Hypophysis. 
Pityriasis  rosea,  935 

rubra,  935 
Placenta,  875 
Plasmorrhexis,  88,  102 
Plasmoschisis,  62,  88,  102 
Plethora,  22 
Pleurae,  324 
Plica  polonica,  932 
Pneumatopericardium,  136 
Pneumonia,  aspiration,  302 
chronic,  305 
dissecans,  305 
hypostatic,  302 
lobar,  297,  298 
lobular,  297,  301 
metastatic,  304 
miliary,  297 
per  extensionem,  305 
pleurogenetic,  297 
pneumonic  phthisis,  314 
purulent,  303 
vagus  pneumonia,  302 
"white"  pneumonia,  318 
Pneumonokoniosis,  305 
Pneumonomalacia,   321 
Pneumonomycosis  aspergillina,  319 
Pneumoperitoneum,  511 
Pneumothorax,  246 
Poikilocytosis,  92 
Poliencephalitis,  556 
Poliomyelitis,  576,  586 
Poliosis  circumscripta,  950 
Polyarthritis,  1051 
Polychromatophilia,  88 
Polycoria,  628 
Polycythemia,  86 
,  Polydactylism,  1016 
Polymastia,  883 
Polymyositis,  983 
Polyneuritis,  602 
Polyorchidism,  798 
Polythelia,  883 
Polytrichia,  909 
Pompholyx.  938 
Porencephaly,  526,  535,  555 
Portal  cirrhosis,  466 
pylephlebitis,  462 


INDEX 


1077 


Portal  thrombosis,  462 
Posthitis,  788 
Pott's  disease,  1027 
Pregnancy,  kidney  of,  744 
Preputiafcalculi,  790,  814 
Proctitis,  450 
Prolapse  of  uterus,  822 

of  vagina,  816 
Prostate,  amyloid  bodies  of,  794 

anomalies  of,  793 

atrophy  of,  795 

echinococcus  cysts  of,  795 

hypertrophy  of,  795 

inflammations  of,  793 

phleboliths,  793 

tuberculosis  of,  793 

tumors  of,  797 
Prurigo,  934 

Psammoma,  540,  550,  566,  651,  857 
Pseudarthrosis,  1043 
Pseudochylous  ascites,  111 
Pseudocirrhosis  of  liver,  472 
Pseudoglioma,  640 
Pseudohypertrophy  of  muscles,  991 
Pseudoleukemia,  216 
Pseudomyxoma,  865 
Pseudotuberculosis,  317 
Psoriasis,  938,  946 
Pterygium,  615 

Pulmonary  valve,  atresia  of,  144 
insufficiency  of,  168 
stenosis  of,  144,  168 
Pulpitis,  390 

Pupillary  membrane,  persistent,  627 
Purpura,  911 
Pustule,  914 

malignant,  925 
Pyelitis.     See  Kidney  pelvis. 
Pylephlebitis,  portal,  462 
Pyloric  stenosis,  406 
Pyocele,  retro-uterine,  833 
Pyometra,  826 
Pyopericardium,   138 
Pyorrhoea  alveolaris,  383 
Pyosalpinx,  843,  845,  846 
Pyothorax,  329 
Pyrosis,  349 


Q 


QUINCKE'S  test,  92 


RACHITIC  "rosary,"  1008 
Ranula  pancreatica,  503 
Raynaud's  disease,  658,  949 
Recklinghausen's  disease,  958 
Retina,  anemia  of,  637 

artery  of,  638 

atrophy  of,  644 

colloid  of,  644 

coloboma  of,  637 

cysts  of,  644 


Retina,  fatty  degeneration  of,  644 

hemorrhage  of,  639 

hyperemia  of,  638 

ossification  of,  645 

parasites  of,  644 

pseudoglioma,  640 

retinitis,  640 

separation  of,  644 

veins,  dilatation  of,  640 
Retinitis,  albuminuric,  641 

chronic  diffuse,  642 

circinate,  643 

diabetic,  642 

disseminated,  642 

proliferans,  640 

punctata  albescens,  643 

simplex,  641 

Solaris,  644 

suppurative,  644 
Retromammary  abscess,  884 
Retroperitoneal  lipoma,  512 
Retropharyngeal  abscess,  392 
Rhagades,  914 
Rhinitis,  264 
Rhinoliths,  266 
Rhinoscleroma,  366,  922 
"  Rice-bodies,"  1001,  1055 
Rickets,  1007 
Ringworm,  928 
Rodent  ulcer,  970 
Rosacea,  910 
Roseola,  910 
;  Rupia,  946 
Rupture  of  Fallopian  tubes,  844 

of  heart,  154 

of  oesophagus,  399 

of  uterus,  826 


S 


SALIVA,  354 

Salivary  calculi,  396 

Salpingitis,  844 

Salpingocele,  843 

Scabies,  931 

Scarlatina,  942 

Scleritis,  626 

Scleroderma,  952 

Sclerosis,  amyotrophic  lateral,  596 

of  endocardium,  165 

lateral,  587 

Moenckeberg's,  180 

posterior,  588 
Scoliosis,  1017,  1037 
Scotoma,  648 
Scrophuloderma,  922 
Scrotum,  chimney- r  •••  cep's  cancer  of,  808 

elephantiasis"  o(y  808 

lymphangiectasis  of,  808 
Sebaceous  glands,  acne  of,  976 
acrocordon,  957,  976 
asteatosis  of,  975 
atheroma  of,  976 
comedo  of,  975 
milium  of,  976 


1078 


INDEX 


Sebaceous  glands,  seborrhoea  of,  974 
tinea  sycosis  of,  976 
tumors  of,  977 
Seborrhoea,  974 
Secondary  anemia,  91 
Segmentation  of  heart  fibers,  152 
Seminal  vesicles,  808 
Sequestration,  1020 
Sialodochitis  pancreatica,  494 
Sialoliths,  396,  498 
Siderosis,  307 

Skin,  actinomycosis  of,  924 
ainhum  of,  949 
albinism,  908 
anemia  of,  912 

angioneurotic  redema  of,  910 
anthrax  of,  925 
atrophy  of,  948 

blastomycetic  dermatitis  of,  930 
callus  (callositas)  of,  951 
chancre  of,  789 
chancroid  of,  789 
condyloma  of,  956 
cornu  cutaneum  of,  952 
craw-craw  of,  932 
cutis  laxa,  958 
decubitus  of,  948 
dermatitis  of,  913 
ambustionis,  916 
calorica,  916 
congelationis,  916 
exfoliativa,  935 
venenata,  916 
dermatolysis,  958 
dermatomycosis,  927 
drug  rashes  of,  947 
ecthyma  of,  918 
eczema  of,  935 
ergotism  of,  947 
erysipelas  of,  926 
erythema  of,  937 
erythrasma  of,  929 
exanthems  of,  941 
favus  of,  927 
frambesia  of,  926 
gangrene  of,  949 
glanders  of,  926 
Guinea-worm,  932 
hemorrhage  into,  911 
herpes  of,  934 
hospital  gangrene  of,  917 
hydroa  vacciniforme  of,  938 
hyperemia  of,  910 
ichthyosis  of,  907,  952 
impetigo  of,  945 
keloid  of,  958 
keratodermia  of,  952 
keratosis  of,  955 
leprosy  of,  923 
leukoderma  of,  908 
lichen  of,  939,  945 
livedo  of,  910 
lupus  of,  919 

erythematosus  of,  940 
Madura  foot,  924 
nevi  of,  908 


Skin,  oedema  of,  910 
parasites  of,  931 
pellagra,  947 
pemphigus  of,  937 
perforating  ulcer  of,  949 
pigmentation  of,  950 
pityriasis  of,  935 
pompholyx  of,  938 
prurigo  of,  934 
psoriasis  of,  938 
Kaynaud's  disease  of,  949 
rhinoscleroma  of,  922 
ringworm,  928 
rodent  ulcer  of,  970 
rosacea  of,  910 
roseola  of,  910 
scabies  of,  931 

sclerema  neonatorum  of,  953 
scleroderma  of,  952 
scrophuloderma  of,  922 
syphilis  of,  918,  944 
teleangiectasis  of,  910 
tropical  phagedena,  936 
tuberculosis  of,  919 
tumors  of,  957 
ulceration  of,  948 
urticaria  of,  933 
verruca  of,  956 
xanthelasma  of,  961 
xeroderma  of,  907 
xerosis  of,  907 
:r-ray  burns  of,  915 
Smegma  bacilli,  307,  757 
Snake-tongue,  381 
Sneezing,  252 
Spermatic  cord,  808 
Spermatocystitis,  809 
Spina  bifida,  524 

ventosa,  1026 
Spinal  cord,  acute  anterior  poliomyelitis, 

576 

ascending  paralysis,  579 
suppurative  myelitis,  579 
transverse  myelitis,  577 
amyotrophic    lateral     sclerosis, 

596 
chronic    anterior    poliomyelitis, 

586 

circulatory  disturbances  of,  574 
compression  myelitis,  583 
disseminated  sclerosis  of,  585 
Friedreich's  ataxia,  594 
hematomyelopore,  574 
hemorrhage  into,  574 
heterotopia  of,  533 
lateral  sclerosis  of,  587 
myelitis  of,  575 
pernicious  anemia  of,  596 
posterior  sclerosis  of,  588 
posterolateral  sclerosis  of,  594 
syphilis  of,  581 
syringomyelia,  530 
syringomyelocele,  525 
tuberculosis  of,  580 
tumors  of,  599 
meningitis,  570 


INDEX 


1079 


Spinal  meningitis,  cerebro-,  544 
syphilitic,  573 
tuberculous,    572 
Spirochaeta  pallida,  318,  918,  946 
Splanchnoptosis,  406 
Spleen,  actinomycosis  of,  228 
anomalies  of,  222 
circulatory  disturbances  of,  223 
glanders  of,  228 
leprosy  of,  228 
retrogressive  changes  of,  228 
splenadenoma  of,  229 
splenitis,    225 
syphilis  of,  227 
tuberculosis  of,  227 
tumors  of,  230 
Splenadenoma,  229 
Splenitis,  225 

Spondylitis  deformans,  1059 
Staphyloma,  620,  626 
Status  lymphaticus,  209,  698 
Stenosis  of  aorta,  144,  168 
of  intestines,  424 
of  mitral  valve,  168 
of  ceosphagus,  397 
of  pulmonary  valves,  144,  168 
pyloric,  406 
of  tricuspid  valve,  168 
of  uterus,  825 
Stertor,  240 
Still's  disease,  1053 

Stomach,  circulatory  disturbances  of,  408 
congenital  anomalies  of,  404 
contraction  of,  407 
dilatation  of,  407 
displacement  of,  406 
diverticula  of,  404 
erosion  of,  416 

gastritis,  acute  catarrhal,  410 
atrophic,  412 
chronic  catarrhal,  411 
follicular,  411 
hematogenous,  410 
hypertrophic,  412 
membranous,  410 
per  extensionem,  410 
phlegmonous,  411 
specific,  412 
hair-balls  of,  413 
hypertrophy  of,  416 
pyloric  stenosis,  406 
retrograde  metamorphoses,  414 
thrush  of,  412 
tumors  of,  417 
volvulus  of,  408 
Stomatitis,  382 
Strauss'  phenomenon,  802 
Struma,  691,  688 

lipomatosa  suprarenalis,  703 
Succus  entericus,  363 
Sudamina,  974 
Suggillations,  78 
Suprarenal  glands,  679,  700 
accessory,  701 
hemorrhage  into,  701 
syphilis  of,  703 


Suprarenal  glands,  thrombosis  of,  702 
tuberculosis  of,  702 
tumors  of,  703 
Sweat-glands,  anidrosis,  973 
bromidrosis,  973 
chrornidrosis,  974 
hematidrosis,  974 
hydradenitis,  974 
hydrocystoma,  974 
hyperidrosis,  973 
hyphidrosis,  973 
sudamina,  974 
uridrosis,  974 
Symblepharon,  617 
Syndesmosis,  1043 
Synechia  of  iris,  629 
Synophthalmia,  526,  607 
Synorchidism,  799 
Synostosis,  1043 

Synovia!  sacs,  floating  cartilage,  1062 
free  bodies  of,  1063 
hemorrhage  of,  1051 
lipoma  arborescens,  1064,  1058 
synovitis,  1051 

fibrinosa,  1051 
pannosa,  1056 
purulenta,  1052 
serosa,  1051 
serofibrinosa,  1051 
syphilitica,  1056 
tuberculosa,  1055 
urica,  1053 

Syphilis  of  arteries,  192,  193,  196 
of  auricle,  660 
of  bladder  (urinary),  777 
of  bone,  1028 
of  bone-marrow,  232 
of  bronchi,  279 
of  buccal  cavity,  384 
of  cerebrum,  559 
of  choroid,  636 
of  conjunctiva,  614 
of  cornea,  624 
of  dura  mater,  538,  571 
dwarfism  and,  1005 
of  endocardium,  167 
of  Eustachian  tube,  665 
of  Fallopian  tubes,  841 
of  heart,  149 
of  intestines,  443 
of  iris,  630 
of  joints,  1056 
of  kidney,  758 
of  labyrinth,  672 
of  larynx,  273 
of  liver,  475 
of  lung,  305,  318 
of  lymphatics,  214 
of  lymph-nodes,  214 
of  mammary  gland,  887 
of  mediastinum,  335 
of  membrana  tvmpani,  663 
of  middle  ear,  670 
of  muscles,  986 
of  myocardium,  149 
of  nasal  cavity,  265 


1080 


INDEX 


Syphilis  of  oesophagus,  401 

of  optic  nerve,  64<S 

of  ovaries,  852 

of  pancreas,  499 

of  penis,  789,  918 

of  pericardium,  140 

of  peripheral  nerves,  602 

of  peritoneum,  511 

of  pharynx,  395 

of  pia-arachnoid,  547,  572 

of  pituitary,  709 

of  placenta,  876 

of  pleura,  331 

of  skin,  918,  944 

of  spinal  cord,  581 
meninges,  573 

of  spleen,  227 

of  stomach,  412 

of  suprarenal  glands,  703 

tabes  dorsalis  and,  592 

of  testis,  801 

of  thymus  gland,  691 

of  thyroid  gland,  691 

of  tonsil,  395 

of  tunica  vaginalis  testis,  807 

of  uterus,  832 

of  vagina,  818 

of  veins,  203 

of  vulva,  813 
Syringomyelia,  530 
Syringomyelocele,  525 


TALIPES,  1018 
Teeth,  390 

Teleangiectasis,  201,  910 
Tendinitis,  998 
Tenosynovitis,  90S 
Teratoma  of  mouth,  338 

of  ovary,  862 
Terminal  arteries,  34,  38 
Testes,  congenital  anomalies  of,  798 

glanders  of,  803 

orchitis,  799 

periorchitis,  805 

X1  ilis  of,  801 
rculosis  of,  800 
tumors  of,  803 
Thelitis,  885 
Thrombo-arteritis,  191 
Thrombosinusitis  of  brain,  536 

of  uterus,  870 
Thrombosis,  58 
portal,  462 

of  suprarenal  vein,  702 
Thrush,  384,  412 
Thymic  asthma,  686 
Thymus  gland,  anatomy  of,  697 

congenital  anomalies  of,  698 
cysts  of,  698 
embryology  of,  685 
thymic  asthma,  686 
tumors  of,  699 
Thyroid  gland,  anatomy  of,  676,  687 


Thyroid   gland,  congenital  anomalies  of, 

688 

embryology  of,  676,  688 
function  of,  676 
goitre,  692 

inflammations  of,  690 
Tinea  circinata,  929 

favosa,  927 

imbricata,  929 

sycosis,  929 

tonsurans,  929 

versicolor,  929 
Tokelau  ringworm,  929 
Tongue,  382 
Tonsillitis,  393 
Tonsils,  391 
Tophi,  657 
Toxic  oedema,  112 
Tracheitis,  270 
Trachoma,  612 
Trauma  of  choroid,  637 

of  endocardium,  Ids 

of  heart,  149 

of  uterus,  826 

of  vagina,  816 
Trichiasis,  613 
Trichinosis,  987 
Trichorrhexis  nodosa,  972 
Tricuspid  valve,  insufficiency  of,  168 

stenosis  of,  168 
Tropical  abscess  of  liver,  -1  ( i  1 

phagedena,  936 
Tuberculosis  of  appendix,  -!")() 

of  arteries,  192 

of  auricle,  660 

of  bladder,  777 

of  bone,  1024 

of  bone-marrow,  232 

of  broad  ligaments,  867 

bronchitis,  278 

of  buccal  cavity,  309 

of  bursir,  1001 

of  cerebrum,  559 

of  choroid,  636 

of  conjunctiva,  614 

of  cornea,  624 

of  dura,  538,  571 

of  endocardium,  167 

of  epididymis,  800 

of  Eustachian  tube,  665 

of  Fallopian  tubes,  846 

of  heart,  149 

of  intestines,  439 

of  iris,  630 

of  joints,  1054 

of  kidney,  755 
pelvis.  772 

of  larynx,  271 

of  liver,  474 

of  lungs,  307 

of  lymphatics,  204 

of  lymph-nodes,  212 

of  mammary  gland,  886 

of  mediastinum,  335 

of  membrana  tympani,  663 

of  muscles,  985 


INDEX 


1081 


Tuberculosis  of  myocardium,  149 

of  nasal  cavity,  265 

of  oesophagus,  401 

of  optic  nerve,  648 

of  ovaries,  852 

of  pancreas,  498 

of  penis,  790 

of  pericardium,  140 

of  peripheral  nerves,  602 

of  peritoneum,  510 

of  pia-arachnoid,  545,  572 

of  pleura,  330 

of  prostate,  793 

of  seminal  vesicles,  809 

of  skin,  919 

of  spinal  cord,  580 

of  spleen,  227 

of  suprarenal  glands,  702 

of  tendons,  999 

of  testis,  802 

of  thymus  gland,  698 

of  thyroid  gland,  698 

of  tonsils,  395 

of  tunica  vaginalis  testis,  807 

of  ureter,  772 

of  urethra,  784 

of  uterus,  832 

of  vagina,  818 

of  vas  deferens,  809 

of  veins,  203 

of  vulva,  803 
Tuboovarian  abscess,  851 

cyst,  843 
Tumors  of  auditory  nerve,  651 

of  bladder,  780 

of  cerebrum,  563,  567 

of  conjunctiva,  616 

of  Fallopian  tubes,  847 

of  heart,  158 

of  intestines,  451 

of  joints,  1064 

of  kidney,  764 

of  liver,  483 

of  lung,  322 

of  lymphatic  glands,  218 

of  mediastinum,  336 

of  muscles,  996 

of  nerves,  603 

of  oasophagus,  402 

of  optic  nerve,  648 

of  orbit,  654 

of  ovaries,  854 

of  pancreas,  501 

of  pelvis  of  kidney,  773 

of  penis,  791 

of  pericardium,  141 

of  prostate,  797 

of  sebaceous  glands,  977 

of  skin,  957 

of  spinal  cord,  599 

of  spleen,  230 

of  stomach,  417 

of  suprarenal  glands,  703 

of  testes,  803 

of  thymus  gland,  699 

of  urethra,  784 


Tumors  of  uterus,  834 
of  vagina,  819 
of  vulva,  814 

Typhlitis,  444 

Typhoid  fever,  436 

Tyloma,  951 


ULCER,  peptic,  402 

perforating,  of  foot,  949 
of  nasal  septum,  266 
rodent,  970 
Umbilical  cord,  877 
Uratic  infarcts,  763 
Ureter,  770 
Urethra,   781 
injuries,  785 
tumors,  784 
Urethritis,  782 
Uridosis,  974 
Urinary  calculi,  779 
Uterus,  apoplexy  of,  827 
atresia  of,  822 
atrophy  of,  833 

circulatory  disturbances  of,  827 
congenital  anomalies  of,  821 
diverticula  of,  826 
endometritis,  828 

acute,  catarrhal,  828 
diphtheria!,  829 
hemorrhagic,  828 
interstitial,  829 
membranous,  829 
chronic  interstitial,  830 

proliferating,  829 
fistulae  of,  827 
flexions  of,  822,  825 
foreign  bodies,  833 
hematoma  of,  827 
hematometra  of,  827 
hydrometra  of,  826 
hypertrophy  of,  833 
inversion  of,  822 
malpositions  of,  822 
menorrhagia,  827 
metritis,  831 
metrorrhagia,  827 
ovula  Nabothi,  830 
parametritis,  866 
parasites  of,  832 
perimetritis,  866 
physometra,  826 
puerperal,  hemorrhage,  869 

nydrorrhoea  gravidarum,  871 
inflammations,  869 
injuries,  869 
lochiometra,    827 
placental  polyps,  871 
syncytioma  malignum,  872 
moles,  876 
pyometra,  826 
rupture  of,  826 
stenosis  of,  825 
syphilis  of,  832 


1082 


INDEX 


Uterus,  thrombosinusitis  of,  870 
trauma  of,  826 
tuberculosis  of,  832 
tumors  of,  834 


VACCINIA,  !H3 
Vagabond's  disease,  932 
Vagina,  atresia  of,  815 

colpohyperplasia  cystica,  818 
congenital  anomalies  of,  815 
cysts  of,  819 
fistulffl  of,  816 
foreign  bodies  in,  819 
hematokolpos,  816 
parasites  of,  819 
perivaginitis,  818 
prolapse  of,  816 
syphilis  of,  818 
trauma  of,  816 
tuberculosis  of,  818 
tumors  of,  819 
vaginitis,  adhesive,  817 
aphthous,  818 
catarrhal,  817 
emphysematous,  818 
exfoliative,  817 
granular,  817 
membranous,  817 
phlegmonous,  818 
senile,  817 
Vaginitis,  817 
Varicella,  944 
Varicocele,  808,  865 
Varicose  veins,  201 
Variola,  611,  665,  942 

of  bones,  1033 
Venous  occlusion,  46 
Verruca,  956 

tuberculosa,  919 
Vesicles,  914 
Vibices,  911 
Vicarious  menstruation,  884 


Yitiligo,  950 
Vitreous  humor,  653 
Volvulus,  424 
Vomiting,  349 
Vulva,  atrophy  of,  813 

condyloma  of,  812 

congenital  anomalies  of,  810 

chancroid  of,  813 

elephantiasis  of,  814 

gangrene  (noma)  of,  813 

kraurosis  of,  813 

lupus  of,  813 

phagedena  of,  813 

syphilis  of,  813 

t  iimors  of,  814 
Vulvitis,  811 


\\  AIMS,  956 

\\hcals,   914 

-  White  swelling,"  1056 

Witch's  milk,  883 


XANTHELASMA  (xanthoma),  961 
Xeroderma,  907,  964,  975 
Xerophthalmia,  612 
Xerosis,  614,  907 
X-ray  dermatitis,  915 


YAWS,  926 


/KNKKR'S  necrosis,  992 
"  Zuckergussherz,"  140,  335 
"  Zuckergussleber,"  335,  472 
Zuckerkandl's  organ,  685 


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